Neurology

To the Editor: We read with great pleasure the article by Sharayah et al about acute gastro­paresis in a patient with diabetic ketoacidosis.¹ However, in the case description, the authors reached a diagnosis of gastroparesis secondary to diabetic ketoacidosis without aptly ruling out some of its most common causes such as hypokalemia and other electrolyte imbalances seen in diabetic patients (in the setting of recurrent vomiting).

The authors also did not include the patient’s duration of diabetes or hemoglobin A_1c level, both of which are linked with gastroparesis in diabetic patients.² Pertinent biochemical information that can help readers formulate a rational approach and journey to making a diagnosis appears elusive in their article.

To the Editor: We read with great pleasure the article by Sharayah et al about acute gastro­paresis in a patient with diabetic ketoacidosis.¹ However, in the case description, the authors reached a diagnosis of gastroparesis secondary to diabetic ketoacidosis without aptly ruling out some of its most common causes such as hypokalemia and other electrolyte imbalances seen in diabetic patients (in the setting of recurrent vomiting).

The authors also did not include the patient’s duration of diabetes or hemoglobin A_1c level, both of which are linked with gastroparesis in diabetic patients.² Pertinent biochemical information that can help readers formulate a rational approach and journey to making a diagnosis appears elusive in their article.

To the Editor: We read with great pleasure the article by Sharayah et al about acute gastro­paresis in a patient with diabetic ketoacidosis.¹ However, in the case description, the authors reached a diagnosis of gastroparesis secondary to diabetic ketoacidosis without aptly ruling out some of its most common causes such as hypokalemia and other electrolyte imbalances seen in diabetic patients (in the setting of recurrent vomiting).

The authors also did not include the patient’s duration of diabetes or hemoglobin A_1c level, both of which are linked with gastroparesis in diabetic patients.² Pertinent biochemical information that can help readers formulate a rational approach and journey to making a diagnosis appears elusive in their article.

In Reply: We thank the readers for their letter. Our patient’s laboratory values at the time of presentation were as follows:

• Corrected sodium 142 mmol/L
• Potassium 5.5 mmol/L
• Phosphorus 6.6 mmol/L.

The rest of the electrolyte levels were within normal limits.

These reported electrolyte levels were unlikely to cause such gastroparesis. The patient’s hemoglobin A_1c was 8.7% at the time of presentation, with no previous values available. However, since abdominal computed tomography done 1 year before this presentation did not show stomach dilation and the patient was asymptomatic, his gastroparesis was presumed to be acute.

In Reply: We thank the readers for their letter. Our patient’s laboratory values at the time of presentation were as follows:

• Corrected sodium 142 mmol/L
• Potassium 5.5 mmol/L
• Phosphorus 6.6 mmol/L.

The rest of the electrolyte levels were within normal limits.

These reported electrolyte levels were unlikely to cause such gastroparesis. The patient’s hemoglobin A_1c was 8.7% at the time of presentation, with no previous values available. However, since abdominal computed tomography done 1 year before this presentation did not show stomach dilation and the patient was asymptomatic, his gastroparesis was presumed to be acute.

In Reply: We thank the readers for their letter. Our patient’s laboratory values at the time of presentation were as follows:

• Corrected sodium 142 mmol/L
• Potassium 5.5 mmol/L
• Phosphorus 6.6 mmol/L.

The rest of the electrolyte levels were within normal limits.

These reported electrolyte levels were unlikely to cause such gastroparesis. The patient’s hemoglobin A_1c was 8.7% at the time of presentation, with no previous values available. However, since abdominal computed tomography done 1 year before this presentation did not show stomach dilation and the patient was asymptomatic, his gastroparesis was presumed to be acute.

CASE Increasingly frequent paroxysmal episodes

Ms. N, age 12, comes to the hospital for evaluation of paroxysmal episodes of pain, weakness, and muscle spasms. A neurologist who evaluated her as an outpatient had recommended a routine electroencephalogram (EEG); after those results were inconclusive, Ms. N’s mother brought her to the hospital for a 24-hour video EEG.

Ms. N has a history of asthma. She has no history of seizures or headache, but her mother has an unspecified seizure disorder that has been stable with antiepileptic medication for many years. Ms. N has no other family history of autoimmune or neurologic disorders.

Ms. N’s episodes began 6 months ago and have progressively increased in frequency from 5 to 12 episodes a day. She says that before she has an episode, she “ feels tingling in her fingers and mouth, and butterflies in her belly,” and then her “whole body clenches up.” She denies experiencing tongue biting, facial or extremity weakness, incontinence, or loss of consciousness during these episodes.

Shortly before her hospitalization, Ms. N had won a scholarship to attend an overnight art camp. Because her episodes were becoming more frequent and their etiology remained unclear, Ms. N and her mother decided it would be unsafe for her to attend, and that she should go to the hospital for evaluation instead.

EVALUATION Tough questions reveal answers

The pediatric team evaluates Ms. N. Her physical exam, laboratory values, and imaging are all within normal limits. Her neurologic exam demonstrates full strength, tone, and sensation in all extremities. All cranial nerves and reflexes are intact. No dysmorphic features or gait abnormalities are noted. All laboratory and imaging tests are normal, including complete blood cell count, electrolytes, calcium, magnesium, phosphorus, glucose, creatine kinase, liver enzymes, urine drug screen, human chorionic gonadotropin (hCG) urine test, and head CT.

After the initial workup, the pediatric team consults the child and adolescent psychiatry team for a complete assessment of Ms. N due to concerns that a psychological component is contributing to her episodes. According to the psychosocial history obtained from Ms. N and her mother, Ms. N had experienced disrupted attachment, trauma, and loss. At age 5, Ms. N was temporarily removed from her mother’s custody after a fight between her mother and brother. At age 9, Ms. N’s stepfather, her primary father figure, died of a brain tumor.

Ms. N also has significant trauma stemming from her relationship with her biological father. Ms. N’s mother reports that her daughter was conceived during nonconsensual sexual intercourse. Ms. N did not have much contact with her biological father until 6 months ago, when he started picking her up at school and taking her to his home for several hours without permission or supervision. Afterwards, Ms. N confided to her mother and a teacher that her father sexually assaulted her during those visits.

Continue to: Ms. N and her mother...

Ms. N and her mother reported the assault to the police and were awaiting legal action.

During the interview with the psychiatry team, Ms. N denies that any thoughts or actions trigger the episodes and reports that she cannot control when they happen. Because she cannot anticipate the episodes, she says she is afraid to leave her house. She does not know why the episodes are happening and feels frustrated that they are getting worse. Ms. N says, “I have been feeling down lately,” but she denies hopelessness, worthlessness, suicidal ideation, homicidal ideation, delusions, or hallucinations.

In the hospital, when the psychiatry team asks Ms. N about her visits with her father, she says that they are “too painful to talk about,” and fears that discussing them will trigger an episode. However, her mother suggests that her daughter’s sexual trauma, as well as ongoing frustrations with the legal system, are influencing her mood; she has had low energy, poor appetite, and is spending more time in bed. Her mother also reports that Ms. N “avoids going out in the sun and spending time with her friends outside. She doesn’t seem to enjoy shopping and art like she used to.” Ms. N told her mother that she was having nightmares about the trauma and “could not stop thinking about some of the bad stuff that happened during the day.”

Ten minutes into the interview, while being questioned about her father, Ms. N experiences a spastic episode. She curls up in bed on her left side, clenches her entire body, and shuts her eyes. Her mother quickly runs to her bedside and counts the seconds until the end of the episode. After 25 seconds, Ms. N awakes with full recollection of the episode. On review of the video EEG during the episode, no ictal patterns are seen.

[polldaddy:10375873]

The authors’ observations

Paroxysmal episodes of weakness, numbness, and muscle spasms in a young female are suggestive of either epilepsy or nonepileptic seizure (NES).^1,2 The negative EEG and physical features are inconsistent with epileptiform seizure, and Ms. N’s history and evaluation are suggestive of NES. Nonepileptic seizures are a type of a conversion disorder, or functional neurologic symptom disorder, in which a patient experiences weakness, abnormal movements, or seizure-like episodes that are inconsistent with organic neurologic disease.³ When a diagnosis of conversion disorder is suspected, a clinician must always consider other pathology that can explain the symptoms, such as migraine, vasovagal syncope, or intracranial mass. If a patient has focal neurologic deficits, head imaging should be pursued. Additionally, the clinician must screen for malingering and factitious disorder before establishing a definitive diagnosis. However, conversion disorder is not a diagnosis of exclusion. For example, a negative EEG does not rule out epilepsy, and patients can have both epilepsy and concomitant NES.

Continue to: Although NES is a common...

Although NES is a common type of conversion disorder, it is often difficult to diagnose, manage, and treat. Patients often receive antiepileptic medications but continue to have worsening events that are refractory to treatment. Various clinical features can suggest NES instead of epilepsy. Forced eye closure on video recording is a specific finding suggestive of NES, yet this feature is not sufficient to make the diagnosis.⁴ A video EEG must be performed to assess for epilepsy. The diagnosis of NES does not exclude the possibility that a patient has epilepsy, as NES can occur in up to 40% of patients with epilepsy.⁵ A video EEG without ictal patterns before, during, and after an observed episode is diagnostic of NES.⁶

[polldaddy:10375874]

The authors’ observations

Conversion disorders such as NES are a presentation of neurologic symptoms that cannot be readily accounted for by other conditions and are often associated with antecedent trauma. Multiple factors in Ms. N’s history increase her risk of NES, including loss of multiple loved ones, ongoing legal involvement, and alleged sexual abuse by her father.

Victims of sexual abuse are more likely than the general population to demonstrate symptoms of conversion disorder, especially NES.^7,8 The onset of paroxysmal episodes after incestuous abuse in a teenage girl is characteristic of NES. Compared with patients with complex partial epilepsy (CPE), patients with NES are 3 times more likely to report sexual trauma.^9,10 Children who report sexual abuse that precedes NES are more likely to have been victimized by a first-degree relative than patients with CPE who report sexual abuse.¹¹ Risk factors for victims developing NES may be related to the severity of adversity, stress sensitivity, and decreased hippocampal volume.^12,13

Ms. N endorsed many psychiatric symptoms that accompany her paroxysmal episodes; this is similar to findings in other patients with NES._¹⁴ One study found that depression is 3 times more prevalent and PTSD is 8 times more prevalent in patients with NES.¹² During the evaluation, Ms. N’s mother said her daughter had low energy, poor appetite, lethargy, and anhedonia for the preceding 5 months, which is consistent with adjustment disorder._³ Her flashbacks, nightmares, difficulty sleeping, and agoraphobia, along with her trouble engaging with the people and activities that used to bring her joy, are symptoms of PTSD. Nonepileptic seizure is often associated with PTSD and can be viewed as an expression of a dissociated subtype.¹⁵

In a literature review, Durrant et al¹⁶ isolated prognostic indicators for NES (Table¹⁶). This study found that 70% of children and 40% of adults achieve remission from NES. Ms. N’s case has multiple concerning features, such as her comorbid psychiatric conditions, ongoing involvement in a legal case, and sexual trauma; this last factor is associated with the most severe symptoms and worse outcomes.^16,17 Despite this somber reality, Ms. N has the support of her mother and is relatively young, which play a vital role in recovery.

Continue to: TREATMENT A strategy for minimizing the episodes

 

 

TREATMENT A strategy for minimizing the episodes

Ms. N’s medical workup remains unremarkable throughout the rest of her hospital stay. The psychiatry and pediatric teams discuss their assessments and agree that NES is the most likely diagnosis. The psychiatry team counsels Ms. N and her mother on the diagnosis and etiology of NES.

[polldaddy:10375876]

The authors’ observations

Cognitive-behavioral therapy is currently the treatment of choice for reducing seizure frequency in patients with NES.^18,19 The use of CBT was suggested due to the theory that NES represents a dissociative response to trauma. Therapy focuses on changing a patient’s beliefs and perceptions associated with attacks.⁵ A randomized study of 66 patients with NES compared the use of CBT plus standard medical care with standard medical care alone.¹⁸ The standard medical care consisted of supportive treatment, an explanation of NES from a neuropsychiatrist, and supervised withdrawal of antiepileptic drugs. The CBT treatment group was offered weekly hour-long sessions for 12 weeks, accompanied by CBT homework and journaling the frequency and nature of seizure episodes (the CBT techniques are outlined in the Figure¹⁸). After 4 months, the CBT treatment group had fewer seizures, and after a 6-month follow-up, they were more likely to be seizure-free. However, in this study, CBT treatment did not improve mood or employment status.

A later investigation looked at using selective serotonin reuptake inhibitors to treat NES in adults.¹⁹ This study divided participants into 4 treatment groups: CBT with informed psychotherapy (CBT-ip), CBT-ip plus sertraline, sertraline alone, and treatment as usual. Sertraline was titrated up to a dose of 200 mg/d as tolerated. After 16 weeks of sertraline alone, seizure frequency did not decrease. Although both CBT groups showed a reduction in symptoms of up to 60%, the CBT-ip group reported fewer psychiatric symptoms with better social interactions, quality of life, and global functioning compared with patients treated with CBT-ip plus sertraline. The authors suggested that this may be due to the somatic adverse effects associated with sertraline. This study suggests that CBT without medication is the treatment of choice.

In addition to CBT, studies of psychodynamic psychotherapy for NES have had promising findings.²⁰ Psychodynamic psychotherapy focuses on addressing conscious and unconscious anger, loss, feelings of isolation, and trauma. Through improving emotional processing, insight, coping skills and self-regulation, patients often benefit from an improvement in seizures, psychosocial functioning and health care utilization.

Metin et al²¹ found that group therapy alongside a family-centered approach elicited a strong and durable reduction in seizures in patients with NES. At enrollment, investigators distributed information on NES to patients and families. Psychoeducation and psychoanalysis with behavior modification techniques were provided in 90-minute weekly group sessions over 3 months. Participants also underwent monthly individualized sessions for standard psychiatric care for 9 months. During the group sessions, operant conditioning techniques were used to prevent secondary gain from seizure-like activity. Families met 4 times for 1 hour each to discuss seizures, receive psychoeducation on a subconscious etiology of NES, and learn behavior modification techniques. All 9 participants who completed group and individual therapy reported a significant and sustained reduction in seizure frequency by at least 50% at 12-month follow-up. Patients also demonstrated improvements in mood, anxiety, and quality of life.

Continue to: A meta-analysis...

A meta-analysis by Carlson and Perry²² that included 13 studies and 228 participants, examined different treatment modalities and their effectiveness for NES. They found that patients who received psychological intervention had a 47% remission rate and 82% improvement in seizure frequency compared with only 14% to 23% of those who did not receive therapy. They postulated that therapy for this illness must be flexible to properly address the socially, psychologically, and functionally heterogenous patient population. Although there are few randomized controlled trials for NES to determine the best evidence-based intervention, there is now consensus that NES has a favorable prognosis when barriers to psychological care are eliminated.

OUTCOME Referral for CBT

The treatment team advises Ms. N to engage in outpatient therapy after discharge from the hospital. Ms. N and her mother agree to the treatment plan, and leave the hospital with a referral for CBT the next day.

Bottom Line

Nonepileptic seizure (NES) is a type of conversion disorder characterized by seizure-like episodes without ictal qualities. Risk factors for NES include concomitant epilepsy, psychiatric disorders, unstable psychosocial situations, and antecedent trauma. Patients with a history of incestuous sexual abuse are most at risk for developing NES. A normal EEG that fully captures a seizure-like episode is diagnostic of NES. Cognitive-behavioral therapy can minimize seizure frequency and intensity.

Related Resources

• Marsh P, Benbadis S, Fernandez F. Psychogenic nonepileptic seizures: ways to win over skeptical patients. Current Psychiatry. 2008;7(1):21-24, 32-35.
• LaFrance WC Jr. Eye-opening behaviors help diagnose nonepileptic seizures. Current Psychiatry. 2006;5(11):121-122, 124, 130.
• LaFrance WC Jr, Kanner AM, Barry JJ. Treating patients with psychological nonepileptic seizures. In: Ettinger AB, Kanner AM, eds. Psychiatric issues in epilepsy: a practical guide to diagnosis and treatment. 2nd ed. Philadelphia, PA: Wolters Kluwer Health/ Lippincott Williams & Wilkins; 2007:461-488.

Drug Brand Name

Sertraline • Zoloft

CASE Increasingly frequent paroxysmal episodes

Ms. N, age 12, comes to the hospital for evaluation of paroxysmal episodes of pain, weakness, and muscle spasms. A neurologist who evaluated her as an outpatient had recommended a routine electroencephalogram (EEG); after those results were inconclusive, Ms. N’s mother brought her to the hospital for a 24-hour video EEG.

Ms. N has a history of asthma. She has no history of seizures or headache, but her mother has an unspecified seizure disorder that has been stable with antiepileptic medication for many years. Ms. N has no other family history of autoimmune or neurologic disorders.

Ms. N’s episodes began 6 months ago and have progressively increased in frequency from 5 to 12 episodes a day. She says that before she has an episode, she “ feels tingling in her fingers and mouth, and butterflies in her belly,” and then her “whole body clenches up.” She denies experiencing tongue biting, facial or extremity weakness, incontinence, or loss of consciousness during these episodes.

Shortly before her hospitalization, Ms. N had won a scholarship to attend an overnight art camp. Because her episodes were becoming more frequent and their etiology remained unclear, Ms. N and her mother decided it would be unsafe for her to attend, and that she should go to the hospital for evaluation instead.

EVALUATION Tough questions reveal answers

The pediatric team evaluates Ms. N. Her physical exam, laboratory values, and imaging are all within normal limits. Her neurologic exam demonstrates full strength, tone, and sensation in all extremities. All cranial nerves and reflexes are intact. No dysmorphic features or gait abnormalities are noted. All laboratory and imaging tests are normal, including complete blood cell count, electrolytes, calcium, magnesium, phosphorus, glucose, creatine kinase, liver enzymes, urine drug screen, human chorionic gonadotropin (hCG) urine test, and head CT.

After the initial workup, the pediatric team consults the child and adolescent psychiatry team for a complete assessment of Ms. N due to concerns that a psychological component is contributing to her episodes. According to the psychosocial history obtained from Ms. N and her mother, Ms. N had experienced disrupted attachment, trauma, and loss. At age 5, Ms. N was temporarily removed from her mother’s custody after a fight between her mother and brother. At age 9, Ms. N’s stepfather, her primary father figure, died of a brain tumor.

Ms. N also has significant trauma stemming from her relationship with her biological father. Ms. N’s mother reports that her daughter was conceived during nonconsensual sexual intercourse. Ms. N did not have much contact with her biological father until 6 months ago, when he started picking her up at school and taking her to his home for several hours without permission or supervision. Afterwards, Ms. N confided to her mother and a teacher that her father sexually assaulted her during those visits.

Continue to: Ms. N and her mother...

Ms. N and her mother reported the assault to the police and were awaiting legal action.

During the interview with the psychiatry team, Ms. N denies that any thoughts or actions trigger the episodes and reports that she cannot control when they happen. Because she cannot anticipate the episodes, she says she is afraid to leave her house. She does not know why the episodes are happening and feels frustrated that they are getting worse. Ms. N says, “I have been feeling down lately,” but she denies hopelessness, worthlessness, suicidal ideation, homicidal ideation, delusions, or hallucinations.

In the hospital, when the psychiatry team asks Ms. N about her visits with her father, she says that they are “too painful to talk about,” and fears that discussing them will trigger an episode. However, her mother suggests that her daughter’s sexual trauma, as well as ongoing frustrations with the legal system, are influencing her mood; she has had low energy, poor appetite, and is spending more time in bed. Her mother also reports that Ms. N “avoids going out in the sun and spending time with her friends outside. She doesn’t seem to enjoy shopping and art like she used to.” Ms. N told her mother that she was having nightmares about the trauma and “could not stop thinking about some of the bad stuff that happened during the day.”

Ten minutes into the interview, while being questioned about her father, Ms. N experiences a spastic episode. She curls up in bed on her left side, clenches her entire body, and shuts her eyes. Her mother quickly runs to her bedside and counts the seconds until the end of the episode. After 25 seconds, Ms. N awakes with full recollection of the episode. On review of the video EEG during the episode, no ictal patterns are seen.

[polldaddy:10375873]

The authors’ observations

Paroxysmal episodes of weakness, numbness, and muscle spasms in a young female are suggestive of either epilepsy or nonepileptic seizure (NES).^1,2 The negative EEG and physical features are inconsistent with epileptiform seizure, and Ms. N’s history and evaluation are suggestive of NES. Nonepileptic seizures are a type of a conversion disorder, or functional neurologic symptom disorder, in which a patient experiences weakness, abnormal movements, or seizure-like episodes that are inconsistent with organic neurologic disease.³ When a diagnosis of conversion disorder is suspected, a clinician must always consider other pathology that can explain the symptoms, such as migraine, vasovagal syncope, or intracranial mass. If a patient has focal neurologic deficits, head imaging should be pursued. Additionally, the clinician must screen for malingering and factitious disorder before establishing a definitive diagnosis. However, conversion disorder is not a diagnosis of exclusion. For example, a negative EEG does not rule out epilepsy, and patients can have both epilepsy and concomitant NES.

Continue to: Although NES is a common...

Although NES is a common type of conversion disorder, it is often difficult to diagnose, manage, and treat. Patients often receive antiepileptic medications but continue to have worsening events that are refractory to treatment. Various clinical features can suggest NES instead of epilepsy. Forced eye closure on video recording is a specific finding suggestive of NES, yet this feature is not sufficient to make the diagnosis.⁴ A video EEG must be performed to assess for epilepsy. The diagnosis of NES does not exclude the possibility that a patient has epilepsy, as NES can occur in up to 40% of patients with epilepsy.⁵ A video EEG without ictal patterns before, during, and after an observed episode is diagnostic of NES.⁶

[polldaddy:10375874]

The authors’ observations

Conversion disorders such as NES are a presentation of neurologic symptoms that cannot be readily accounted for by other conditions and are often associated with antecedent trauma. Multiple factors in Ms. N’s history increase her risk of NES, including loss of multiple loved ones, ongoing legal involvement, and alleged sexual abuse by her father.

Victims of sexual abuse are more likely than the general population to demonstrate symptoms of conversion disorder, especially NES.^7,8 The onset of paroxysmal episodes after incestuous abuse in a teenage girl is characteristic of NES. Compared with patients with complex partial epilepsy (CPE), patients with NES are 3 times more likely to report sexual trauma.^9,10 Children who report sexual abuse that precedes NES are more likely to have been victimized by a first-degree relative than patients with CPE who report sexual abuse.¹¹ Risk factors for victims developing NES may be related to the severity of adversity, stress sensitivity, and decreased hippocampal volume.^12,13

Ms. N endorsed many psychiatric symptoms that accompany her paroxysmal episodes; this is similar to findings in other patients with NES._¹⁴ One study found that depression is 3 times more prevalent and PTSD is 8 times more prevalent in patients with NES.¹² During the evaluation, Ms. N’s mother said her daughter had low energy, poor appetite, lethargy, and anhedonia for the preceding 5 months, which is consistent with adjustment disorder._³ Her flashbacks, nightmares, difficulty sleeping, and agoraphobia, along with her trouble engaging with the people and activities that used to bring her joy, are symptoms of PTSD. Nonepileptic seizure is often associated with PTSD and can be viewed as an expression of a dissociated subtype.¹⁵

In a literature review, Durrant et al¹⁶ isolated prognostic indicators for NES (Table¹⁶). This study found that 70% of children and 40% of adults achieve remission from NES. Ms. N’s case has multiple concerning features, such as her comorbid psychiatric conditions, ongoing involvement in a legal case, and sexual trauma; this last factor is associated with the most severe symptoms and worse outcomes.^16,17 Despite this somber reality, Ms. N has the support of her mother and is relatively young, which play a vital role in recovery.

Continue to: TREATMENT A strategy for minimizing the episodes

 

 

TREATMENT A strategy for minimizing the episodes

Ms. N’s medical workup remains unremarkable throughout the rest of her hospital stay. The psychiatry and pediatric teams discuss their assessments and agree that NES is the most likely diagnosis. The psychiatry team counsels Ms. N and her mother on the diagnosis and etiology of NES.

[polldaddy:10375876]

The authors’ observations

Cognitive-behavioral therapy is currently the treatment of choice for reducing seizure frequency in patients with NES.^18,19 The use of CBT was suggested due to the theory that NES represents a dissociative response to trauma. Therapy focuses on changing a patient’s beliefs and perceptions associated with attacks.⁵ A randomized study of 66 patients with NES compared the use of CBT plus standard medical care with standard medical care alone.¹⁸ The standard medical care consisted of supportive treatment, an explanation of NES from a neuropsychiatrist, and supervised withdrawal of antiepileptic drugs. The CBT treatment group was offered weekly hour-long sessions for 12 weeks, accompanied by CBT homework and journaling the frequency and nature of seizure episodes (the CBT techniques are outlined in the Figure¹⁸). After 4 months, the CBT treatment group had fewer seizures, and after a 6-month follow-up, they were more likely to be seizure-free. However, in this study, CBT treatment did not improve mood or employment status.

A later investigation looked at using selective serotonin reuptake inhibitors to treat NES in adults.¹⁹ This study divided participants into 4 treatment groups: CBT with informed psychotherapy (CBT-ip), CBT-ip plus sertraline, sertraline alone, and treatment as usual. Sertraline was titrated up to a dose of 200 mg/d as tolerated. After 16 weeks of sertraline alone, seizure frequency did not decrease. Although both CBT groups showed a reduction in symptoms of up to 60%, the CBT-ip group reported fewer psychiatric symptoms with better social interactions, quality of life, and global functioning compared with patients treated with CBT-ip plus sertraline. The authors suggested that this may be due to the somatic adverse effects associated with sertraline. This study suggests that CBT without medication is the treatment of choice.

In addition to CBT, studies of psychodynamic psychotherapy for NES have had promising findings.²⁰ Psychodynamic psychotherapy focuses on addressing conscious and unconscious anger, loss, feelings of isolation, and trauma. Through improving emotional processing, insight, coping skills and self-regulation, patients often benefit from an improvement in seizures, psychosocial functioning and health care utilization.

Metin et al²¹ found that group therapy alongside a family-centered approach elicited a strong and durable reduction in seizures in patients with NES. At enrollment, investigators distributed information on NES to patients and families. Psychoeducation and psychoanalysis with behavior modification techniques were provided in 90-minute weekly group sessions over 3 months. Participants also underwent monthly individualized sessions for standard psychiatric care for 9 months. During the group sessions, operant conditioning techniques were used to prevent secondary gain from seizure-like activity. Families met 4 times for 1 hour each to discuss seizures, receive psychoeducation on a subconscious etiology of NES, and learn behavior modification techniques. All 9 participants who completed group and individual therapy reported a significant and sustained reduction in seizure frequency by at least 50% at 12-month follow-up. Patients also demonstrated improvements in mood, anxiety, and quality of life.

Continue to: A meta-analysis...

A meta-analysis by Carlson and Perry²² that included 13 studies and 228 participants, examined different treatment modalities and their effectiveness for NES. They found that patients who received psychological intervention had a 47% remission rate and 82% improvement in seizure frequency compared with only 14% to 23% of those who did not receive therapy. They postulated that therapy for this illness must be flexible to properly address the socially, psychologically, and functionally heterogenous patient population. Although there are few randomized controlled trials for NES to determine the best evidence-based intervention, there is now consensus that NES has a favorable prognosis when barriers to psychological care are eliminated.

OUTCOME Referral for CBT

The treatment team advises Ms. N to engage in outpatient therapy after discharge from the hospital. Ms. N and her mother agree to the treatment plan, and leave the hospital with a referral for CBT the next day.

Bottom Line

Nonepileptic seizure (NES) is a type of conversion disorder characterized by seizure-like episodes without ictal qualities. Risk factors for NES include concomitant epilepsy, psychiatric disorders, unstable psychosocial situations, and antecedent trauma. Patients with a history of incestuous sexual abuse are most at risk for developing NES. A normal EEG that fully captures a seizure-like episode is diagnostic of NES. Cognitive-behavioral therapy can minimize seizure frequency and intensity.

Related Resources

• Marsh P, Benbadis S, Fernandez F. Psychogenic nonepileptic seizures: ways to win over skeptical patients. Current Psychiatry. 2008;7(1):21-24, 32-35.
• LaFrance WC Jr. Eye-opening behaviors help diagnose nonepileptic seizures. Current Psychiatry. 2006;5(11):121-122, 124, 130.
• LaFrance WC Jr, Kanner AM, Barry JJ. Treating patients with psychological nonepileptic seizures. In: Ettinger AB, Kanner AM, eds. Psychiatric issues in epilepsy: a practical guide to diagnosis and treatment. 2nd ed. Philadelphia, PA: Wolters Kluwer Health/ Lippincott Williams & Wilkins; 2007:461-488.

Drug Brand Name

Sertraline • Zoloft

CASE Increasingly frequent paroxysmal episodes

Ms. N, age 12, comes to the hospital for evaluation of paroxysmal episodes of pain, weakness, and muscle spasms. A neurologist who evaluated her as an outpatient had recommended a routine electroencephalogram (EEG); after those results were inconclusive, Ms. N’s mother brought her to the hospital for a 24-hour video EEG.

Ms. N has a history of asthma. She has no history of seizures or headache, but her mother has an unspecified seizure disorder that has been stable with antiepileptic medication for many years. Ms. N has no other family history of autoimmune or neurologic disorders.

Ms. N’s episodes began 6 months ago and have progressively increased in frequency from 5 to 12 episodes a day. She says that before she has an episode, she “ feels tingling in her fingers and mouth, and butterflies in her belly,” and then her “whole body clenches up.” She denies experiencing tongue biting, facial or extremity weakness, incontinence, or loss of consciousness during these episodes.

Shortly before her hospitalization, Ms. N had won a scholarship to attend an overnight art camp. Because her episodes were becoming more frequent and their etiology remained unclear, Ms. N and her mother decided it would be unsafe for her to attend, and that she should go to the hospital for evaluation instead.

EVALUATION Tough questions reveal answers

The pediatric team evaluates Ms. N. Her physical exam, laboratory values, and imaging are all within normal limits. Her neurologic exam demonstrates full strength, tone, and sensation in all extremities. All cranial nerves and reflexes are intact. No dysmorphic features or gait abnormalities are noted. All laboratory and imaging tests are normal, including complete blood cell count, electrolytes, calcium, magnesium, phosphorus, glucose, creatine kinase, liver enzymes, urine drug screen, human chorionic gonadotropin (hCG) urine test, and head CT.

After the initial workup, the pediatric team consults the child and adolescent psychiatry team for a complete assessment of Ms. N due to concerns that a psychological component is contributing to her episodes. According to the psychosocial history obtained from Ms. N and her mother, Ms. N had experienced disrupted attachment, trauma, and loss. At age 5, Ms. N was temporarily removed from her mother’s custody after a fight between her mother and brother. At age 9, Ms. N’s stepfather, her primary father figure, died of a brain tumor.

Ms. N also has significant trauma stemming from her relationship with her biological father. Ms. N’s mother reports that her daughter was conceived during nonconsensual sexual intercourse. Ms. N did not have much contact with her biological father until 6 months ago, when he started picking her up at school and taking her to his home for several hours without permission or supervision. Afterwards, Ms. N confided to her mother and a teacher that her father sexually assaulted her during those visits.

Continue to: Ms. N and her mother...

Ms. N and her mother reported the assault to the police and were awaiting legal action.

During the interview with the psychiatry team, Ms. N denies that any thoughts or actions trigger the episodes and reports that she cannot control when they happen. Because she cannot anticipate the episodes, she says she is afraid to leave her house. She does not know why the episodes are happening and feels frustrated that they are getting worse. Ms. N says, “I have been feeling down lately,” but she denies hopelessness, worthlessness, suicidal ideation, homicidal ideation, delusions, or hallucinations.

In the hospital, when the psychiatry team asks Ms. N about her visits with her father, she says that they are “too painful to talk about,” and fears that discussing them will trigger an episode. However, her mother suggests that her daughter’s sexual trauma, as well as ongoing frustrations with the legal system, are influencing her mood; she has had low energy, poor appetite, and is spending more time in bed. Her mother also reports that Ms. N “avoids going out in the sun and spending time with her friends outside. She doesn’t seem to enjoy shopping and art like she used to.” Ms. N told her mother that she was having nightmares about the trauma and “could not stop thinking about some of the bad stuff that happened during the day.”

Ten minutes into the interview, while being questioned about her father, Ms. N experiences a spastic episode. She curls up in bed on her left side, clenches her entire body, and shuts her eyes. Her mother quickly runs to her bedside and counts the seconds until the end of the episode. After 25 seconds, Ms. N awakes with full recollection of the episode. On review of the video EEG during the episode, no ictal patterns are seen.

[polldaddy:10375873]

The authors’ observations

Paroxysmal episodes of weakness, numbness, and muscle spasms in a young female are suggestive of either epilepsy or nonepileptic seizure (NES).^1,2 The negative EEG and physical features are inconsistent with epileptiform seizure, and Ms. N’s history and evaluation are suggestive of NES. Nonepileptic seizures are a type of a conversion disorder, or functional neurologic symptom disorder, in which a patient experiences weakness, abnormal movements, or seizure-like episodes that are inconsistent with organic neurologic disease.³ When a diagnosis of conversion disorder is suspected, a clinician must always consider other pathology that can explain the symptoms, such as migraine, vasovagal syncope, or intracranial mass. If a patient has focal neurologic deficits, head imaging should be pursued. Additionally, the clinician must screen for malingering and factitious disorder before establishing a definitive diagnosis. However, conversion disorder is not a diagnosis of exclusion. For example, a negative EEG does not rule out epilepsy, and patients can have both epilepsy and concomitant NES.

Continue to: Although NES is a common...

Although NES is a common type of conversion disorder, it is often difficult to diagnose, manage, and treat. Patients often receive antiepileptic medications but continue to have worsening events that are refractory to treatment. Various clinical features can suggest NES instead of epilepsy. Forced eye closure on video recording is a specific finding suggestive of NES, yet this feature is not sufficient to make the diagnosis.⁴ A video EEG must be performed to assess for epilepsy. The diagnosis of NES does not exclude the possibility that a patient has epilepsy, as NES can occur in up to 40% of patients with epilepsy.⁵ A video EEG without ictal patterns before, during, and after an observed episode is diagnostic of NES.⁶

[polldaddy:10375874]

The authors’ observations

Conversion disorders such as NES are a presentation of neurologic symptoms that cannot be readily accounted for by other conditions and are often associated with antecedent trauma. Multiple factors in Ms. N’s history increase her risk of NES, including loss of multiple loved ones, ongoing legal involvement, and alleged sexual abuse by her father.

Victims of sexual abuse are more likely than the general population to demonstrate symptoms of conversion disorder, especially NES.^7,8 The onset of paroxysmal episodes after incestuous abuse in a teenage girl is characteristic of NES. Compared with patients with complex partial epilepsy (CPE), patients with NES are 3 times more likely to report sexual trauma.^9,10 Children who report sexual abuse that precedes NES are more likely to have been victimized by a first-degree relative than patients with CPE who report sexual abuse.¹¹ Risk factors for victims developing NES may be related to the severity of adversity, stress sensitivity, and decreased hippocampal volume.^12,13

Ms. N endorsed many psychiatric symptoms that accompany her paroxysmal episodes; this is similar to findings in other patients with NES._¹⁴ One study found that depression is 3 times more prevalent and PTSD is 8 times more prevalent in patients with NES.¹² During the evaluation, Ms. N’s mother said her daughter had low energy, poor appetite, lethargy, and anhedonia for the preceding 5 months, which is consistent with adjustment disorder._³ Her flashbacks, nightmares, difficulty sleeping, and agoraphobia, along with her trouble engaging with the people and activities that used to bring her joy, are symptoms of PTSD. Nonepileptic seizure is often associated with PTSD and can be viewed as an expression of a dissociated subtype.¹⁵

In a literature review, Durrant et al¹⁶ isolated prognostic indicators for NES (Table¹⁶). This study found that 70% of children and 40% of adults achieve remission from NES. Ms. N’s case has multiple concerning features, such as her comorbid psychiatric conditions, ongoing involvement in a legal case, and sexual trauma; this last factor is associated with the most severe symptoms and worse outcomes.^16,17 Despite this somber reality, Ms. N has the support of her mother and is relatively young, which play a vital role in recovery.

Continue to: TREATMENT A strategy for minimizing the episodes

 

 

TREATMENT A strategy for minimizing the episodes

Ms. N’s medical workup remains unremarkable throughout the rest of her hospital stay. The psychiatry and pediatric teams discuss their assessments and agree that NES is the most likely diagnosis. The psychiatry team counsels Ms. N and her mother on the diagnosis and etiology of NES.

[polldaddy:10375876]

The authors’ observations

Cognitive-behavioral therapy is currently the treatment of choice for reducing seizure frequency in patients with NES.^18,19 The use of CBT was suggested due to the theory that NES represents a dissociative response to trauma. Therapy focuses on changing a patient’s beliefs and perceptions associated with attacks.⁵ A randomized study of 66 patients with NES compared the use of CBT plus standard medical care with standard medical care alone.¹⁸ The standard medical care consisted of supportive treatment, an explanation of NES from a neuropsychiatrist, and supervised withdrawal of antiepileptic drugs. The CBT treatment group was offered weekly hour-long sessions for 12 weeks, accompanied by CBT homework and journaling the frequency and nature of seizure episodes (the CBT techniques are outlined in the Figure¹⁸). After 4 months, the CBT treatment group had fewer seizures, and after a 6-month follow-up, they were more likely to be seizure-free. However, in this study, CBT treatment did not improve mood or employment status.

A later investigation looked at using selective serotonin reuptake inhibitors to treat NES in adults.¹⁹ This study divided participants into 4 treatment groups: CBT with informed psychotherapy (CBT-ip), CBT-ip plus sertraline, sertraline alone, and treatment as usual. Sertraline was titrated up to a dose of 200 mg/d as tolerated. After 16 weeks of sertraline alone, seizure frequency did not decrease. Although both CBT groups showed a reduction in symptoms of up to 60%, the CBT-ip group reported fewer psychiatric symptoms with better social interactions, quality of life, and global functioning compared with patients treated with CBT-ip plus sertraline. The authors suggested that this may be due to the somatic adverse effects associated with sertraline. This study suggests that CBT without medication is the treatment of choice.

In addition to CBT, studies of psychodynamic psychotherapy for NES have had promising findings.²⁰ Psychodynamic psychotherapy focuses on addressing conscious and unconscious anger, loss, feelings of isolation, and trauma. Through improving emotional processing, insight, coping skills and self-regulation, patients often benefit from an improvement in seizures, psychosocial functioning and health care utilization.

Metin et al²¹ found that group therapy alongside a family-centered approach elicited a strong and durable reduction in seizures in patients with NES. At enrollment, investigators distributed information on NES to patients and families. Psychoeducation and psychoanalysis with behavior modification techniques were provided in 90-minute weekly group sessions over 3 months. Participants also underwent monthly individualized sessions for standard psychiatric care for 9 months. During the group sessions, operant conditioning techniques were used to prevent secondary gain from seizure-like activity. Families met 4 times for 1 hour each to discuss seizures, receive psychoeducation on a subconscious etiology of NES, and learn behavior modification techniques. All 9 participants who completed group and individual therapy reported a significant and sustained reduction in seizure frequency by at least 50% at 12-month follow-up. Patients also demonstrated improvements in mood, anxiety, and quality of life.

Continue to: A meta-analysis...

A meta-analysis by Carlson and Perry²² that included 13 studies and 228 participants, examined different treatment modalities and their effectiveness for NES. They found that patients who received psychological intervention had a 47% remission rate and 82% improvement in seizure frequency compared with only 14% to 23% of those who did not receive therapy. They postulated that therapy for this illness must be flexible to properly address the socially, psychologically, and functionally heterogenous patient population. Although there are few randomized controlled trials for NES to determine the best evidence-based intervention, there is now consensus that NES has a favorable prognosis when barriers to psychological care are eliminated.

OUTCOME Referral for CBT

The treatment team advises Ms. N to engage in outpatient therapy after discharge from the hospital. Ms. N and her mother agree to the treatment plan, and leave the hospital with a referral for CBT the next day.

Bottom Line

Nonepileptic seizure (NES) is a type of conversion disorder characterized by seizure-like episodes without ictal qualities. Risk factors for NES include concomitant epilepsy, psychiatric disorders, unstable psychosocial situations, and antecedent trauma. Patients with a history of incestuous sexual abuse are most at risk for developing NES. A normal EEG that fully captures a seizure-like episode is diagnostic of NES. Cognitive-behavioral therapy can minimize seizure frequency and intensity.

Related Resources

• Marsh P, Benbadis S, Fernandez F. Psychogenic nonepileptic seizures: ways to win over skeptical patients. Current Psychiatry. 2008;7(1):21-24, 32-35.
• LaFrance WC Jr. Eye-opening behaviors help diagnose nonepileptic seizures. Current Psychiatry. 2006;5(11):121-122, 124, 130.
• LaFrance WC Jr, Kanner AM, Barry JJ. Treating patients with psychological nonepileptic seizures. In: Ettinger AB, Kanner AM, eds. Psychiatric issues in epilepsy: a practical guide to diagnosis and treatment. 2nd ed. Philadelphia, PA: Wolters Kluwer Health/ Lippincott Williams & Wilkins; 2007:461-488.

Drug Brand Name

Sertraline • Zoloft

I agree with Dr. Hickner’s editorial “To refer—or not?” (J Fam Pract. 2019;68:8) that family physicians could manage about 30% of the patients they refer to specialists. Still, it’s worth noting that many referrals are motivated by the threat of unmerited malpractice suits. Until the medical liability system becomes less adversarial and unmerited suits are eliminated, all primary care doctors—not just family physicians—will continue to send patients to specialists—even when these physicians are themselves capable of treating such patients.

What might help mitigate malpractice suits? There could be benefit from oversight of health courts, which would be presided over by judges with special training in medical malpractice. Being nonadversarial, health courts would cut down on legal wrangling, settle suits, and get awards to patients quicker. They would also cut down on attorney and court fees, which account for almost half of the total amount spent on litigation. These courts wouldn’t completely eliminate unnecessary referrals to specialists, but they could help make a difference.

Edward Volpintesta, MD
Bethel, Conn

I agree with Dr. Hickner’s editorial “To refer—or not?” (J Fam Pract. 2019;68:8) that family physicians could manage about 30% of the patients they refer to specialists. Still, it’s worth noting that many referrals are motivated by the threat of unmerited malpractice suits. Until the medical liability system becomes less adversarial and unmerited suits are eliminated, all primary care doctors—not just family physicians—will continue to send patients to specialists—even when these physicians are themselves capable of treating such patients.

What might help mitigate malpractice suits? There could be benefit from oversight of health courts, which would be presided over by judges with special training in medical malpractice. Being nonadversarial, health courts would cut down on legal wrangling, settle suits, and get awards to patients quicker. They would also cut down on attorney and court fees, which account for almost half of the total amount spent on litigation. These courts wouldn’t completely eliminate unnecessary referrals to specialists, but they could help make a difference.

Edward Volpintesta, MD
Bethel, Conn

I agree with Dr. Hickner’s editorial “To refer—or not?” (J Fam Pract. 2019;68:8) that family physicians could manage about 30% of the patients they refer to specialists. Still, it’s worth noting that many referrals are motivated by the threat of unmerited malpractice suits. Until the medical liability system becomes less adversarial and unmerited suits are eliminated, all primary care doctors—not just family physicians—will continue to send patients to specialists—even when these physicians are themselves capable of treating such patients.

What might help mitigate malpractice suits? There could be benefit from oversight of health courts, which would be presided over by judges with special training in medical malpractice. Being nonadversarial, health courts would cut down on legal wrangling, settle suits, and get awards to patients quicker. They would also cut down on attorney and court fees, which account for almost half of the total amount spent on litigation. These courts wouldn’t completely eliminate unnecessary referrals to specialists, but they could help make a difference.

Edward Volpintesta, MD
Bethel, Conn

THE CASE

A healthy 48-year-old man presented to our otolaryngology clinic with a 2-hour history of hearing loss, tinnitus, and fullness in the left ear. He denied any vertigo, nausea, vomiting, otalgia, or otorrhea. He had noticed signs of a possible upper respiratory infection, including a sore throat and headache, the day before his symptoms started. His medical history was unremarkable. He denied any history of otologic surgery, trauma, or vision problems, and he was not taking any medications.

The patient was afebrile on physical examination with a heart rate of 48 beats/min and blood pressure of 117/68 mm Hg. A Weber test performed using a 512-Hz tuning fork lateralized to the right ear. A Rinne test showed air conduction was louder than bone conduction in the affected left ear—a normal finding. Tympanometry and otoscopic examination showed the bilateral tympanic membranes were normal.

THE DIAGNOSIS

Pure tone audiometry showed severe sensorineural hearing loss in the left ear and a poor speech discrimination score. The Weber test confirmed the hearing loss was sensorineural and not conductive, ruling out a middle ear effusion. Additionally, the normal tympanogram made conductive hearing loss from a middle ear effusion or tympanic membrane perforation unlikely. The positive Rinne test was consistent with a diagnosis of idiopathic sudden sensorineural hearing loss (SSNHL).

DISCUSSION

SSNHL is defined by hearing loss of more than 30 dB in at least 3 consecutive frequencies with acute onset of less than 72 hours.^1,2 The most common symptoms include acute hearing loss, tinnitus, and fullness in the affected ear.¹ The majority of cases of SSNHL are unilateral. The typical age of onset is in the fourth and fifth decades, occurring with equal distribution in both sexes. The annual incidence of SSNHL is 5 to 20 cases per 100,000 individuals worldwide.^1,2

Etiology. Identifiable causes of SSNHL include viral infections, vascular events, cochlear hydrops, head trauma, tumors (eg, vestibular schwannoma), and demyelinating disorders. Bilateral SSNHL can be seen in autoimmune diseases and rarely can be caused by medications, such as aminoglycosides or certain chemotherapy medications. However, 90% of cases of SSNHL are considered idiopathic because the etiology cannot be determined.¹

The most common symptoms of sudden sensorineural hearing loss include not only acute hearing loss, but also tinnitus and fullness in the affected ear.

Diagnosis. The initial evaluation should include an otoscopic examination, tuning fork tests, and pure tone audiometry.^1-3 Weber and Rinne tests are essential when evaluating patients for unilateral hearing loss and determining the type of loss (ie, sensorineural vs conductive). The Weber test (ideally using a 512-Hz tuning fork) can detect either conductive or sensorineural hearing loss. In a normal Weber test, the patient should hear the vibration of the tuning fork equally in both ears. The tuning fork will be heard in both ears in conductive hearing loss but will only be heard in the unaffected hear if sensorineural hearing loss is present. So, for instance, if a patient has a perforation in the right tympanic membrane causing conductive hearing loss in the right hear, the tuning fork would be heard in both ears. If the patient has sensorineural hearing loss in the right ear, the tuning fork would only be heard in the left ear.

The Rinne test compares the perception of sound waves transmitted by air conduction vs bone conduction and serves as a rapid screen for conductive hearing loss. In a positive Rinne test, the patient should be able to hear the vibrating tuning fork next to the pinna louder than when placed against the mastoid bone (ie, air conduction greater than bone conduction). In a negative Rinne test, bone conduction is greater than air conduction, and a conductive hearing loss is present. In our patient, pure tone audiometry, tympanometry, and speech audiometry results were consistent with SSNHL.

Continue to: Magnetic resonance imaging...

Magnetic resonance imaging (MRI) of the brain and brainstem with gadolinium contrast can reveal vascular events (thrombotic or hemorrhagic), demyelinating disorders, or retrocochlear lesions such as vestibular schwannoma and is indicated in all cases of suspected SSNHL.^4,5An MRI should be obtained within 6 weeks of the initial presentation—even if the patient’s hearing returns to normal after treatment.⁴

Treatment and management. The current standard of care for treatment of idiopathic SSNHL is systemic steroids.^1,2 Although the gold standard currently is oral prednisolone or methylprednisolone (1 mg/kg/d for 10 to 14 days with a taper,^1,2 the evidence for this regimen stems from a single placebo-controlled trial (N = 67) that demonstrated greater improvement in the steroid group compared with the placebo group (61% vs 32%).⁶ A Cochrane review and other systematic analyses have not demonstrated clear efficacy of corticosteroid treatment for the management of idiopathic SSNHL.^7,8

Because of the potential systemic adverse effects associated with oral corticosteroids, intratympanic (IT) corticosteroids have been advocated as an alternative treatment option. A prospective, randomized, noninferiority trial comparing the efficacy of oral vs IT corticosteroids for idiopathic SSNHL found IT corticosteroids to be noninferior to systemic treatment.⁹ IT treatment also has been advocated as a rescue therapy for patients who do not respond to systemic treatment.¹⁰

A combination of oral and IT corticosteroids was investigated in a retrospective study analyzing multiple treatment modalities.¹⁰ Researchers first compared 122 patients receiving one of 3 treatments: (1) IT corticosteroids, (2) oral corticosteroids, and (3) combination treatment (IT + oral corticosteroids). There was no difference in hearing recovery among any of the treatments. Fifty-eight patients who were refractory to initial treatment were then included in a second analysis in which they were divided into those who received additional IT corticosteroids (salvage treatment) vs no treatment (control). There was no difference in hearing recovery between the 2 groups. The authors concluded that IT corticosteroids were as effective as oral treatment and that salvage IT treatment did not add any benefit.¹⁰

The American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) recently published guidelines on the diagnosis and management of SSNHL.¹¹ The guidelines state that IT steroids should be considered in patients who cannot tolerate oral steroids, such as patients with diabetes. It is important to note, however, that the high cost of IT treatment (~$2000 for dexamethasone or methylprednisolone vs < $10 for oral prednisolone) is an issue that needs to be considered as health care costs continue to rise.

Continue to: Antivirals

Antivirals. Because an underlying viral etiology has been speculated as a potential cause of idiopathic SSNHL, antiviral agents such as valacyclovir or famciclovir also are potential treatment agents.¹² Antiviral medications have minimal adverse effects and are relatively inexpensive, but the benefits have not yet been proven in randomized controlled trials,and they currently are not endorsed by the AAO-HNS in their guidelines for the management of SSNHL.¹¹

Spontaneous recovery occurs in up to 40% of patients with idiopathic SSNHL. As many as 65% of those who experience recovery do so within 2 weeks of the onset of symptoms, regardless of treatment.^1,2 Treatment beyond 2 weeks after onset of symptoms is unlikely to be of any benefit, although some otolaryngologists will treat for up to 6 weeks after the onset of hearing loss.

A substantial number of patients with SSNHL may not recover. Management of these patients begins with referral to an appropriate specialist to initiate counseling and lifestyle changes. Depending on the degree of hearing loss, audiologic rehabilitation may include use of a traditional or bone-anchored hearing aid or a frequency-modulation system.^1,2,11 Tinnitus retraining therapy might be of benefit for patients with persistent tinnitus.¹¹

Our patient. After a discussion of his treatment options, our patient decided on a combination of oral prednisolone (60 mg once daily for 9 days followed by a taper for 5 days) and intratympanic dexamethasone injections (1 mL [10 mg/mL] once weekly for 3 weeks). Additionally, antiviral treatment with oral valacyclovir (2 g every 8 hours for 7 days) was initiated per the patient’s request (but is not currently recommended by AAO-HNS).

Ninety percent of sudden sensorineural hearing loss cases are considered idiopathic.

The rationale for this approach was the minimal adverse effects associated with short-term (ie, days to 1–2 weeks) use of high-dose (ie, > 30 mg/d) corticosteroids. Although steroid therapy has been associated with adverse effects such as aseptic necrosis of the hip, these complications usually arise after longer periods (ie, months to years) of high-dose steroid therapy with a mean cumulative dose much higher than what was used in our patient.¹³

Continue to: Our patient...

Our patient noticed slight improvement within 48 hours of the initial onset of symptoms that continued for the next several weeks until full recovery was attained. An MRI performed 5 days after the onset of symptoms was negative for retrocochlear pathology.

THE TAKEAWAY

SSNHL is a medical emergency that requires prompt recognition and diagnosis. The steps in evaluating sudden hearing loss include: (1) appropriate history and physical examination (eg, otoscopic examination, tuning fork tests), (2) urgent audiometry to confirm hearing loss, (3) immediate referral to an otolaryngologist for further testing (eg, tympanometry, blood tests, MRI), and (4) initiation of treatment.

If a specific etiology is identified (eg, vestibular schwannoma), the patient should be referred to a specialist for appropriate treatment. If there is no identifiable cause (idiopathic SSNHL), the patient should be treated with oral and/or intratympanic steroids. Patients who do not recover following treatment should be offered audiologic rehabilitation.

CORRESPONDENCE
Sergio Huerta, MD, UT Southwestern Medical Center, 4500 S Lancaster Road #112L, Dallas, TX 75216; Sergio.Huerta@UTSouthwestern.edu

THE CASE

A healthy 48-year-old man presented to our otolaryngology clinic with a 2-hour history of hearing loss, tinnitus, and fullness in the left ear. He denied any vertigo, nausea, vomiting, otalgia, or otorrhea. He had noticed signs of a possible upper respiratory infection, including a sore throat and headache, the day before his symptoms started. His medical history was unremarkable. He denied any history of otologic surgery, trauma, or vision problems, and he was not taking any medications.

The patient was afebrile on physical examination with a heart rate of 48 beats/min and blood pressure of 117/68 mm Hg. A Weber test performed using a 512-Hz tuning fork lateralized to the right ear. A Rinne test showed air conduction was louder than bone conduction in the affected left ear—a normal finding. Tympanometry and otoscopic examination showed the bilateral tympanic membranes were normal.

THE DIAGNOSIS

Pure tone audiometry showed severe sensorineural hearing loss in the left ear and a poor speech discrimination score. The Weber test confirmed the hearing loss was sensorineural and not conductive, ruling out a middle ear effusion. Additionally, the normal tympanogram made conductive hearing loss from a middle ear effusion or tympanic membrane perforation unlikely. The positive Rinne test was consistent with a diagnosis of idiopathic sudden sensorineural hearing loss (SSNHL).

DISCUSSION

SSNHL is defined by hearing loss of more than 30 dB in at least 3 consecutive frequencies with acute onset of less than 72 hours.^1,2 The most common symptoms include acute hearing loss, tinnitus, and fullness in the affected ear.¹ The majority of cases of SSNHL are unilateral. The typical age of onset is in the fourth and fifth decades, occurring with equal distribution in both sexes. The annual incidence of SSNHL is 5 to 20 cases per 100,000 individuals worldwide.^1,2

Etiology. Identifiable causes of SSNHL include viral infections, vascular events, cochlear hydrops, head trauma, tumors (eg, vestibular schwannoma), and demyelinating disorders. Bilateral SSNHL can be seen in autoimmune diseases and rarely can be caused by medications, such as aminoglycosides or certain chemotherapy medications. However, 90% of cases of SSNHL are considered idiopathic because the etiology cannot be determined.¹

The most common symptoms of sudden sensorineural hearing loss include not only acute hearing loss, but also tinnitus and fullness in the affected ear.

Diagnosis. The initial evaluation should include an otoscopic examination, tuning fork tests, and pure tone audiometry.^1-3 Weber and Rinne tests are essential when evaluating patients for unilateral hearing loss and determining the type of loss (ie, sensorineural vs conductive). The Weber test (ideally using a 512-Hz tuning fork) can detect either conductive or sensorineural hearing loss. In a normal Weber test, the patient should hear the vibration of the tuning fork equally in both ears. The tuning fork will be heard in both ears in conductive hearing loss but will only be heard in the unaffected hear if sensorineural hearing loss is present. So, for instance, if a patient has a perforation in the right tympanic membrane causing conductive hearing loss in the right hear, the tuning fork would be heard in both ears. If the patient has sensorineural hearing loss in the right ear, the tuning fork would only be heard in the left ear.

The Rinne test compares the perception of sound waves transmitted by air conduction vs bone conduction and serves as a rapid screen for conductive hearing loss. In a positive Rinne test, the patient should be able to hear the vibrating tuning fork next to the pinna louder than when placed against the mastoid bone (ie, air conduction greater than bone conduction). In a negative Rinne test, bone conduction is greater than air conduction, and a conductive hearing loss is present. In our patient, pure tone audiometry, tympanometry, and speech audiometry results were consistent with SSNHL.

Continue to: Magnetic resonance imaging...

Magnetic resonance imaging (MRI) of the brain and brainstem with gadolinium contrast can reveal vascular events (thrombotic or hemorrhagic), demyelinating disorders, or retrocochlear lesions such as vestibular schwannoma and is indicated in all cases of suspected SSNHL.^4,5An MRI should be obtained within 6 weeks of the initial presentation—even if the patient’s hearing returns to normal after treatment.⁴

Treatment and management. The current standard of care for treatment of idiopathic SSNHL is systemic steroids.^1,2 Although the gold standard currently is oral prednisolone or methylprednisolone (1 mg/kg/d for 10 to 14 days with a taper,^1,2 the evidence for this regimen stems from a single placebo-controlled trial (N = 67) that demonstrated greater improvement in the steroid group compared with the placebo group (61% vs 32%).⁶ A Cochrane review and other systematic analyses have not demonstrated clear efficacy of corticosteroid treatment for the management of idiopathic SSNHL.^7,8

Because of the potential systemic adverse effects associated with oral corticosteroids, intratympanic (IT) corticosteroids have been advocated as an alternative treatment option. A prospective, randomized, noninferiority trial comparing the efficacy of oral vs IT corticosteroids for idiopathic SSNHL found IT corticosteroids to be noninferior to systemic treatment.⁹ IT treatment also has been advocated as a rescue therapy for patients who do not respond to systemic treatment.¹⁰

A combination of oral and IT corticosteroids was investigated in a retrospective study analyzing multiple treatment modalities.¹⁰ Researchers first compared 122 patients receiving one of 3 treatments: (1) IT corticosteroids, (2) oral corticosteroids, and (3) combination treatment (IT + oral corticosteroids). There was no difference in hearing recovery among any of the treatments. Fifty-eight patients who were refractory to initial treatment were then included in a second analysis in which they were divided into those who received additional IT corticosteroids (salvage treatment) vs no treatment (control). There was no difference in hearing recovery between the 2 groups. The authors concluded that IT corticosteroids were as effective as oral treatment and that salvage IT treatment did not add any benefit.¹⁰

The American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) recently published guidelines on the diagnosis and management of SSNHL.¹¹ The guidelines state that IT steroids should be considered in patients who cannot tolerate oral steroids, such as patients with diabetes. It is important to note, however, that the high cost of IT treatment (~$2000 for dexamethasone or methylprednisolone vs < $10 for oral prednisolone) is an issue that needs to be considered as health care costs continue to rise.

Continue to: Antivirals

Antivirals. Because an underlying viral etiology has been speculated as a potential cause of idiopathic SSNHL, antiviral agents such as valacyclovir or famciclovir also are potential treatment agents.¹² Antiviral medications have minimal adverse effects and are relatively inexpensive, but the benefits have not yet been proven in randomized controlled trials,and they currently are not endorsed by the AAO-HNS in their guidelines for the management of SSNHL.¹¹

Spontaneous recovery occurs in up to 40% of patients with idiopathic SSNHL. As many as 65% of those who experience recovery do so within 2 weeks of the onset of symptoms, regardless of treatment.^1,2 Treatment beyond 2 weeks after onset of symptoms is unlikely to be of any benefit, although some otolaryngologists will treat for up to 6 weeks after the onset of hearing loss.

A substantial number of patients with SSNHL may not recover. Management of these patients begins with referral to an appropriate specialist to initiate counseling and lifestyle changes. Depending on the degree of hearing loss, audiologic rehabilitation may include use of a traditional or bone-anchored hearing aid or a frequency-modulation system.^1,2,11 Tinnitus retraining therapy might be of benefit for patients with persistent tinnitus.¹¹

Our patient. After a discussion of his treatment options, our patient decided on a combination of oral prednisolone (60 mg once daily for 9 days followed by a taper for 5 days) and intratympanic dexamethasone injections (1 mL [10 mg/mL] once weekly for 3 weeks). Additionally, antiviral treatment with oral valacyclovir (2 g every 8 hours for 7 days) was initiated per the patient’s request (but is not currently recommended by AAO-HNS).

Ninety percent of sudden sensorineural hearing loss cases are considered idiopathic.

The rationale for this approach was the minimal adverse effects associated with short-term (ie, days to 1–2 weeks) use of high-dose (ie, > 30 mg/d) corticosteroids. Although steroid therapy has been associated with adverse effects such as aseptic necrosis of the hip, these complications usually arise after longer periods (ie, months to years) of high-dose steroid therapy with a mean cumulative dose much higher than what was used in our patient.¹³

Continue to: Our patient...

Our patient noticed slight improvement within 48 hours of the initial onset of symptoms that continued for the next several weeks until full recovery was attained. An MRI performed 5 days after the onset of symptoms was negative for retrocochlear pathology.

THE TAKEAWAY

SSNHL is a medical emergency that requires prompt recognition and diagnosis. The steps in evaluating sudden hearing loss include: (1) appropriate history and physical examination (eg, otoscopic examination, tuning fork tests), (2) urgent audiometry to confirm hearing loss, (3) immediate referral to an otolaryngologist for further testing (eg, tympanometry, blood tests, MRI), and (4) initiation of treatment.

If a specific etiology is identified (eg, vestibular schwannoma), the patient should be referred to a specialist for appropriate treatment. If there is no identifiable cause (idiopathic SSNHL), the patient should be treated with oral and/or intratympanic steroids. Patients who do not recover following treatment should be offered audiologic rehabilitation.

CORRESPONDENCE
Sergio Huerta, MD, UT Southwestern Medical Center, 4500 S Lancaster Road #112L, Dallas, TX 75216; Sergio.Huerta@UTSouthwestern.edu

THE CASE

A healthy 48-year-old man presented to our otolaryngology clinic with a 2-hour history of hearing loss, tinnitus, and fullness in the left ear. He denied any vertigo, nausea, vomiting, otalgia, or otorrhea. He had noticed signs of a possible upper respiratory infection, including a sore throat and headache, the day before his symptoms started. His medical history was unremarkable. He denied any history of otologic surgery, trauma, or vision problems, and he was not taking any medications.

The patient was afebrile on physical examination with a heart rate of 48 beats/min and blood pressure of 117/68 mm Hg. A Weber test performed using a 512-Hz tuning fork lateralized to the right ear. A Rinne test showed air conduction was louder than bone conduction in the affected left ear—a normal finding. Tympanometry and otoscopic examination showed the bilateral tympanic membranes were normal.

THE DIAGNOSIS

Pure tone audiometry showed severe sensorineural hearing loss in the left ear and a poor speech discrimination score. The Weber test confirmed the hearing loss was sensorineural and not conductive, ruling out a middle ear effusion. Additionally, the normal tympanogram made conductive hearing loss from a middle ear effusion or tympanic membrane perforation unlikely. The positive Rinne test was consistent with a diagnosis of idiopathic sudden sensorineural hearing loss (SSNHL).

DISCUSSION

SSNHL is defined by hearing loss of more than 30 dB in at least 3 consecutive frequencies with acute onset of less than 72 hours.^1,2 The most common symptoms include acute hearing loss, tinnitus, and fullness in the affected ear.¹ The majority of cases of SSNHL are unilateral. The typical age of onset is in the fourth and fifth decades, occurring with equal distribution in both sexes. The annual incidence of SSNHL is 5 to 20 cases per 100,000 individuals worldwide.^1,2

Etiology. Identifiable causes of SSNHL include viral infections, vascular events, cochlear hydrops, head trauma, tumors (eg, vestibular schwannoma), and demyelinating disorders. Bilateral SSNHL can be seen in autoimmune diseases and rarely can be caused by medications, such as aminoglycosides or certain chemotherapy medications. However, 90% of cases of SSNHL are considered idiopathic because the etiology cannot be determined.¹

The most common symptoms of sudden sensorineural hearing loss include not only acute hearing loss, but also tinnitus and fullness in the affected ear.

Diagnosis. The initial evaluation should include an otoscopic examination, tuning fork tests, and pure tone audiometry.^1-3 Weber and Rinne tests are essential when evaluating patients for unilateral hearing loss and determining the type of loss (ie, sensorineural vs conductive). The Weber test (ideally using a 512-Hz tuning fork) can detect either conductive or sensorineural hearing loss. In a normal Weber test, the patient should hear the vibration of the tuning fork equally in both ears. The tuning fork will be heard in both ears in conductive hearing loss but will only be heard in the unaffected hear if sensorineural hearing loss is present. So, for instance, if a patient has a perforation in the right tympanic membrane causing conductive hearing loss in the right hear, the tuning fork would be heard in both ears. If the patient has sensorineural hearing loss in the right ear, the tuning fork would only be heard in the left ear.

The Rinne test compares the perception of sound waves transmitted by air conduction vs bone conduction and serves as a rapid screen for conductive hearing loss. In a positive Rinne test, the patient should be able to hear the vibrating tuning fork next to the pinna louder than when placed against the mastoid bone (ie, air conduction greater than bone conduction). In a negative Rinne test, bone conduction is greater than air conduction, and a conductive hearing loss is present. In our patient, pure tone audiometry, tympanometry, and speech audiometry results were consistent with SSNHL.

Continue to: Magnetic resonance imaging...

Magnetic resonance imaging (MRI) of the brain and brainstem with gadolinium contrast can reveal vascular events (thrombotic or hemorrhagic), demyelinating disorders, or retrocochlear lesions such as vestibular schwannoma and is indicated in all cases of suspected SSNHL.^4,5An MRI should be obtained within 6 weeks of the initial presentation—even if the patient’s hearing returns to normal after treatment.⁴

Treatment and management. The current standard of care for treatment of idiopathic SSNHL is systemic steroids.^1,2 Although the gold standard currently is oral prednisolone or methylprednisolone (1 mg/kg/d for 10 to 14 days with a taper,^1,2 the evidence for this regimen stems from a single placebo-controlled trial (N = 67) that demonstrated greater improvement in the steroid group compared with the placebo group (61% vs 32%).⁶ A Cochrane review and other systematic analyses have not demonstrated clear efficacy of corticosteroid treatment for the management of idiopathic SSNHL.^7,8

Because of the potential systemic adverse effects associated with oral corticosteroids, intratympanic (IT) corticosteroids have been advocated as an alternative treatment option. A prospective, randomized, noninferiority trial comparing the efficacy of oral vs IT corticosteroids for idiopathic SSNHL found IT corticosteroids to be noninferior to systemic treatment.⁹ IT treatment also has been advocated as a rescue therapy for patients who do not respond to systemic treatment.¹⁰

A combination of oral and IT corticosteroids was investigated in a retrospective study analyzing multiple treatment modalities.¹⁰ Researchers first compared 122 patients receiving one of 3 treatments: (1) IT corticosteroids, (2) oral corticosteroids, and (3) combination treatment (IT + oral corticosteroids). There was no difference in hearing recovery among any of the treatments. Fifty-eight patients who were refractory to initial treatment were then included in a second analysis in which they were divided into those who received additional IT corticosteroids (salvage treatment) vs no treatment (control). There was no difference in hearing recovery between the 2 groups. The authors concluded that IT corticosteroids were as effective as oral treatment and that salvage IT treatment did not add any benefit.¹⁰

The American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS) recently published guidelines on the diagnosis and management of SSNHL.¹¹ The guidelines state that IT steroids should be considered in patients who cannot tolerate oral steroids, such as patients with diabetes. It is important to note, however, that the high cost of IT treatment (~$2000 for dexamethasone or methylprednisolone vs < $10 for oral prednisolone) is an issue that needs to be considered as health care costs continue to rise.

Continue to: Antivirals

Antivirals. Because an underlying viral etiology has been speculated as a potential cause of idiopathic SSNHL, antiviral agents such as valacyclovir or famciclovir also are potential treatment agents.¹² Antiviral medications have minimal adverse effects and are relatively inexpensive, but the benefits have not yet been proven in randomized controlled trials,and they currently are not endorsed by the AAO-HNS in their guidelines for the management of SSNHL.¹¹

Spontaneous recovery occurs in up to 40% of patients with idiopathic SSNHL. As many as 65% of those who experience recovery do so within 2 weeks of the onset of symptoms, regardless of treatment.^1,2 Treatment beyond 2 weeks after onset of symptoms is unlikely to be of any benefit, although some otolaryngologists will treat for up to 6 weeks after the onset of hearing loss.

A substantial number of patients with SSNHL may not recover. Management of these patients begins with referral to an appropriate specialist to initiate counseling and lifestyle changes. Depending on the degree of hearing loss, audiologic rehabilitation may include use of a traditional or bone-anchored hearing aid or a frequency-modulation system.^1,2,11 Tinnitus retraining therapy might be of benefit for patients with persistent tinnitus.¹¹

Our patient. After a discussion of his treatment options, our patient decided on a combination of oral prednisolone (60 mg once daily for 9 days followed by a taper for 5 days) and intratympanic dexamethasone injections (1 mL [10 mg/mL] once weekly for 3 weeks). Additionally, antiviral treatment with oral valacyclovir (2 g every 8 hours for 7 days) was initiated per the patient’s request (but is not currently recommended by AAO-HNS).

Ninety percent of sudden sensorineural hearing loss cases are considered idiopathic.

The rationale for this approach was the minimal adverse effects associated with short-term (ie, days to 1–2 weeks) use of high-dose (ie, > 30 mg/d) corticosteroids. Although steroid therapy has been associated with adverse effects such as aseptic necrosis of the hip, these complications usually arise after longer periods (ie, months to years) of high-dose steroid therapy with a mean cumulative dose much higher than what was used in our patient.¹³

Continue to: Our patient...

Our patient noticed slight improvement within 48 hours of the initial onset of symptoms that continued for the next several weeks until full recovery was attained. An MRI performed 5 days after the onset of symptoms was negative for retrocochlear pathology.

THE TAKEAWAY

SSNHL is a medical emergency that requires prompt recognition and diagnosis. The steps in evaluating sudden hearing loss include: (1) appropriate history and physical examination (eg, otoscopic examination, tuning fork tests), (2) urgent audiometry to confirm hearing loss, (3) immediate referral to an otolaryngologist for further testing (eg, tympanometry, blood tests, MRI), and (4) initiation of treatment.

If a specific etiology is identified (eg, vestibular schwannoma), the patient should be referred to a specialist for appropriate treatment. If there is no identifiable cause (idiopathic SSNHL), the patient should be treated with oral and/or intratympanic steroids. Patients who do not recover following treatment should be offered audiologic rehabilitation.

CORRESPONDENCE
Sergio Huerta, MD, UT Southwestern Medical Center, 4500 S Lancaster Road #112L, Dallas, TX 75216; Sergio.Huerta@UTSouthwestern.edu

Adults with low levels of hemoglobin and adults with high levels of hemoglobin have an increased risk of developing dementia over 12 years of follow-up, compared with adults with midrange levels, according to a population-based study in the Netherlands.

This U-shaped association “may relate to differences in white matter integrity and cerebral perfusion,” the researchers wrote in Neurology.

Ton Everaers, Erasmus Medical Center

“With around 10% of people over age 65 having anemia in the Americas and Europe and up to 45% in African and southeast Asian countries, these results could have important implications for the burden of dementia,” said study author M. Arfan Ikram, MD, PhD, in a news release. Dr. Ikram is a professor of epidemiology at Erasmus Medical Center in Rotterdam, the Netherlands.

Prior studies have found that low hemoglobin levels are associated with adverse health outcomes, such as coronary heart disease, stroke, and mortality, but data about the relationship between hemoglobin levels and dementia risk have been limited.

A population-based cohort study

To examine the long-term association of hemoglobin levels and anemia with risk of dementia, Dr. Ikram and coauthors analyzed data from the Rotterdam Study, an ongoing population-based cohort study in the Netherlands that started in 1990. Their analysis included data from 12,305 participants without dementia who had serum hemoglobin measured at baseline (mean age, 64.6 years; 57.7% women).

During a mean follow-up of 12.1 years, 1,520 participants developed dementia, 1,194 of whom had Alzheimer’s disease.

“Both low and high hemoglobin levels were associated with increased dementia risk,” the authors wrote. Compared with participants in the middle quintile of hemoglobin levels (8.57-8.99 mmol/L), participants in the lowest quintile (less than 8.11 mmol/L) had a hazard ratio of dementia of 1.29, and participants in the highest quintile (greater than 9.40 mmol/L) had an HR of 1.20.

About 6% of the participants had anemia – that is, a hemoglobin level of less than 8.1 mmol/L for men and less than 7.5 mmol/L for women. Anemia was associated with a 34% increased risk of dementia and a 41% increased risk of Alzheimer’s disease.

Of the 745 people with anemia, 128 developed dementia, compared with 1,392 of the 11,560 people who did not have anemia (17% vs. 12%).

A U-shaped association

The researchers also examined hemoglobin in relation to vascular brain disease, structural connectivity, and global cerebral perfusion among 5,267 participants without dementia who had brain MRI. White matter hyperintensity volume and hemoglobin had a U-shaped association, similar to that for dementia and hemoglobin. In addition, hemoglobin inversely correlated to cerebral perfusion.

The results remained consistent after adjustment for factors such as smoking, high blood pressure, high cholesterol, and alcohol use.

A limitation of the study is that the participants lived in the Netherlands and were primarily of European descent, so the results may not apply to other populations, the authors wrote.

Dr. Ikram noted that the study does not prove that low or high hemoglobin levels cause dementia. “More research is needed to determine whether hemoglobin levels play a direct role in this increased risk or whether these associations can be explained by underlying issues or other vascular or metabolic changes.”

The study was supported by the Netherlands Cardiovascular Research Initiative; Erasmus Medical Centre; Erasmus University Rotterdam; Netherlands Organization for Scientific Research; Netherlands Organization for Health Research and Development; Research Institute for Diseases in the Elderly; Netherlands Genomic Initiative; Dutch Ministry of Education, Culture, and Science; Dutch Ministry of Health, Welfare, and Sports; European Commission; Municipality of Rotterdam; Netherlands Consortium for Healthy Aging; and Dutch Heart Foundation. The authors reported no relevant disclosures.

jremaly@mdedge.com

SOURCE: Ikram MA et al. Neurology. 2019 Jul 31. doi: 10.1212/WNL.0000000000008003.

Adults with low levels of hemoglobin and adults with high levels of hemoglobin have an increased risk of developing dementia over 12 years of follow-up, compared with adults with midrange levels, according to a population-based study in the Netherlands.

This U-shaped association “may relate to differences in white matter integrity and cerebral perfusion,” the researchers wrote in Neurology.

Ton Everaers, Erasmus Medical Center

“With around 10% of people over age 65 having anemia in the Americas and Europe and up to 45% in African and southeast Asian countries, these results could have important implications for the burden of dementia,” said study author M. Arfan Ikram, MD, PhD, in a news release. Dr. Ikram is a professor of epidemiology at Erasmus Medical Center in Rotterdam, the Netherlands.

Prior studies have found that low hemoglobin levels are associated with adverse health outcomes, such as coronary heart disease, stroke, and mortality, but data about the relationship between hemoglobin levels and dementia risk have been limited.

A population-based cohort study

To examine the long-term association of hemoglobin levels and anemia with risk of dementia, Dr. Ikram and coauthors analyzed data from the Rotterdam Study, an ongoing population-based cohort study in the Netherlands that started in 1990. Their analysis included data from 12,305 participants without dementia who had serum hemoglobin measured at baseline (mean age, 64.6 years; 57.7% women).

During a mean follow-up of 12.1 years, 1,520 participants developed dementia, 1,194 of whom had Alzheimer’s disease.

“Both low and high hemoglobin levels were associated with increased dementia risk,” the authors wrote. Compared with participants in the middle quintile of hemoglobin levels (8.57-8.99 mmol/L), participants in the lowest quintile (less than 8.11 mmol/L) had a hazard ratio of dementia of 1.29, and participants in the highest quintile (greater than 9.40 mmol/L) had an HR of 1.20.

About 6% of the participants had anemia – that is, a hemoglobin level of less than 8.1 mmol/L for men and less than 7.5 mmol/L for women. Anemia was associated with a 34% increased risk of dementia and a 41% increased risk of Alzheimer’s disease.

Of the 745 people with anemia, 128 developed dementia, compared with 1,392 of the 11,560 people who did not have anemia (17% vs. 12%).

A U-shaped association

The researchers also examined hemoglobin in relation to vascular brain disease, structural connectivity, and global cerebral perfusion among 5,267 participants without dementia who had brain MRI. White matter hyperintensity volume and hemoglobin had a U-shaped association, similar to that for dementia and hemoglobin. In addition, hemoglobin inversely correlated to cerebral perfusion.

The results remained consistent after adjustment for factors such as smoking, high blood pressure, high cholesterol, and alcohol use.

A limitation of the study is that the participants lived in the Netherlands and were primarily of European descent, so the results may not apply to other populations, the authors wrote.

Dr. Ikram noted that the study does not prove that low or high hemoglobin levels cause dementia. “More research is needed to determine whether hemoglobin levels play a direct role in this increased risk or whether these associations can be explained by underlying issues or other vascular or metabolic changes.”

The study was supported by the Netherlands Cardiovascular Research Initiative; Erasmus Medical Centre; Erasmus University Rotterdam; Netherlands Organization for Scientific Research; Netherlands Organization for Health Research and Development; Research Institute for Diseases in the Elderly; Netherlands Genomic Initiative; Dutch Ministry of Education, Culture, and Science; Dutch Ministry of Health, Welfare, and Sports; European Commission; Municipality of Rotterdam; Netherlands Consortium for Healthy Aging; and Dutch Heart Foundation. The authors reported no relevant disclosures.

jremaly@mdedge.com

SOURCE: Ikram MA et al. Neurology. 2019 Jul 31. doi: 10.1212/WNL.0000000000008003.

Adults with low levels of hemoglobin and adults with high levels of hemoglobin have an increased risk of developing dementia over 12 years of follow-up, compared with adults with midrange levels, according to a population-based study in the Netherlands.

This U-shaped association “may relate to differences in white matter integrity and cerebral perfusion,” the researchers wrote in Neurology.

Ton Everaers, Erasmus Medical Center

“With around 10% of people over age 65 having anemia in the Americas and Europe and up to 45% in African and southeast Asian countries, these results could have important implications for the burden of dementia,” said study author M. Arfan Ikram, MD, PhD, in a news release. Dr. Ikram is a professor of epidemiology at Erasmus Medical Center in Rotterdam, the Netherlands.

Prior studies have found that low hemoglobin levels are associated with adverse health outcomes, such as coronary heart disease, stroke, and mortality, but data about the relationship between hemoglobin levels and dementia risk have been limited.

A population-based cohort study

To examine the long-term association of hemoglobin levels and anemia with risk of dementia, Dr. Ikram and coauthors analyzed data from the Rotterdam Study, an ongoing population-based cohort study in the Netherlands that started in 1990. Their analysis included data from 12,305 participants without dementia who had serum hemoglobin measured at baseline (mean age, 64.6 years; 57.7% women).

During a mean follow-up of 12.1 years, 1,520 participants developed dementia, 1,194 of whom had Alzheimer’s disease.

“Both low and high hemoglobin levels were associated with increased dementia risk,” the authors wrote. Compared with participants in the middle quintile of hemoglobin levels (8.57-8.99 mmol/L), participants in the lowest quintile (less than 8.11 mmol/L) had a hazard ratio of dementia of 1.29, and participants in the highest quintile (greater than 9.40 mmol/L) had an HR of 1.20.

About 6% of the participants had anemia – that is, a hemoglobin level of less than 8.1 mmol/L for men and less than 7.5 mmol/L for women. Anemia was associated with a 34% increased risk of dementia and a 41% increased risk of Alzheimer’s disease.

Of the 745 people with anemia, 128 developed dementia, compared with 1,392 of the 11,560 people who did not have anemia (17% vs. 12%).

A U-shaped association

The researchers also examined hemoglobin in relation to vascular brain disease, structural connectivity, and global cerebral perfusion among 5,267 participants without dementia who had brain MRI. White matter hyperintensity volume and hemoglobin had a U-shaped association, similar to that for dementia and hemoglobin. In addition, hemoglobin inversely correlated to cerebral perfusion.

The results remained consistent after adjustment for factors such as smoking, high blood pressure, high cholesterol, and alcohol use.

A limitation of the study is that the participants lived in the Netherlands and were primarily of European descent, so the results may not apply to other populations, the authors wrote.

Dr. Ikram noted that the study does not prove that low or high hemoglobin levels cause dementia. “More research is needed to determine whether hemoglobin levels play a direct role in this increased risk or whether these associations can be explained by underlying issues or other vascular or metabolic changes.”

The study was supported by the Netherlands Cardiovascular Research Initiative; Erasmus Medical Centre; Erasmus University Rotterdam; Netherlands Organization for Scientific Research; Netherlands Organization for Health Research and Development; Research Institute for Diseases in the Elderly; Netherlands Genomic Initiative; Dutch Ministry of Education, Culture, and Science; Dutch Ministry of Health, Welfare, and Sports; European Commission; Municipality of Rotterdam; Netherlands Consortium for Healthy Aging; and Dutch Heart Foundation. The authors reported no relevant disclosures.

jremaly@mdedge.com

SOURCE: Ikram MA et al. Neurology. 2019 Jul 31. doi: 10.1212/WNL.0000000000008003.

Researchers at the University of Alabama at Birmingham will lead a 5-year, federally-funded study of acute flaccid myelitis (AFM) – a rare pediatric neurologic disease.

CDC

The National Institute of Allergy and Infectious Diseases (NIAID) awarded the $10 million grant to primary investigator David Kimberlin, MD, a UAB professor of pediatrics. Carlos Pardo-Villamizar, MD, professor of neurology and pathology at Johns Hopkins University, Baltimore, is the co-principal investigator.

The university will organize and implement the international, multisite study. Its primary goal is to examine the incidence and distribution of AFM, and its pathogenesis and progression. Enrollment is expected to commence next fall. Investigators will enroll children with symptoms of AFM and follow them for 1 year. Household contacts of the subjects will serve as comparators.

In addition to collecting data about risk factors and disease progression, the researchers will collect clinical specimens, including blood and cerebrospinal fluid. More details about the design and study sites will be released then, according to a press statement issued by NIAID.

AFM targets spinal nerves and often develops after a mild respiratory illness. The disease mounted a global epidemic comeback in 2014, primarily affecting children; it has occurred concurrently with enterovirus outbreaks.

“Growing epidemiological evidence suggests that enterovirus-D68 [EV-D68] could play a role,” the statement noted. “Most people who become infected with EV-D68 are asymptomatic or experience mild, cold-like symptoms. Researchers and physicians are working to understand if there is a connection between these viral outbreaks and AFM, and if so, why some children but not others experience this sudden muscle weakness and paralysis.”

The study will draw on the expertise of the AFM Task Force, established last fall. The group comprises physicians, scientists, and public health experts from diverse disciplines and institutions who will assist in the ongoing investigation.

The AFM natural history study is funded under contract HHSN272201600018C.

msullivan@mdedge.com

Researchers at the University of Alabama at Birmingham will lead a 5-year, federally-funded study of acute flaccid myelitis (AFM) – a rare pediatric neurologic disease.

CDC

The National Institute of Allergy and Infectious Diseases (NIAID) awarded the $10 million grant to primary investigator David Kimberlin, MD, a UAB professor of pediatrics. Carlos Pardo-Villamizar, MD, professor of neurology and pathology at Johns Hopkins University, Baltimore, is the co-principal investigator.

The university will organize and implement the international, multisite study. Its primary goal is to examine the incidence and distribution of AFM, and its pathogenesis and progression. Enrollment is expected to commence next fall. Investigators will enroll children with symptoms of AFM and follow them for 1 year. Household contacts of the subjects will serve as comparators.

In addition to collecting data about risk factors and disease progression, the researchers will collect clinical specimens, including blood and cerebrospinal fluid. More details about the design and study sites will be released then, according to a press statement issued by NIAID.

AFM targets spinal nerves and often develops after a mild respiratory illness. The disease mounted a global epidemic comeback in 2014, primarily affecting children; it has occurred concurrently with enterovirus outbreaks.

“Growing epidemiological evidence suggests that enterovirus-D68 [EV-D68] could play a role,” the statement noted. “Most people who become infected with EV-D68 are asymptomatic or experience mild, cold-like symptoms. Researchers and physicians are working to understand if there is a connection between these viral outbreaks and AFM, and if so, why some children but not others experience this sudden muscle weakness and paralysis.”

The study will draw on the expertise of the AFM Task Force, established last fall. The group comprises physicians, scientists, and public health experts from diverse disciplines and institutions who will assist in the ongoing investigation.

The AFM natural history study is funded under contract HHSN272201600018C.

msullivan@mdedge.com

Researchers at the University of Alabama at Birmingham will lead a 5-year, federally-funded study of acute flaccid myelitis (AFM) – a rare pediatric neurologic disease.

CDC

The National Institute of Allergy and Infectious Diseases (NIAID) awarded the $10 million grant to primary investigator David Kimberlin, MD, a UAB professor of pediatrics. Carlos Pardo-Villamizar, MD, professor of neurology and pathology at Johns Hopkins University, Baltimore, is the co-principal investigator.

The university will organize and implement the international, multisite study. Its primary goal is to examine the incidence and distribution of AFM, and its pathogenesis and progression. Enrollment is expected to commence next fall. Investigators will enroll children with symptoms of AFM and follow them for 1 year. Household contacts of the subjects will serve as comparators.

In addition to collecting data about risk factors and disease progression, the researchers will collect clinical specimens, including blood and cerebrospinal fluid. More details about the design and study sites will be released then, according to a press statement issued by NIAID.

AFM targets spinal nerves and often develops after a mild respiratory illness. The disease mounted a global epidemic comeback in 2014, primarily affecting children; it has occurred concurrently with enterovirus outbreaks.

“Growing epidemiological evidence suggests that enterovirus-D68 [EV-D68] could play a role,” the statement noted. “Most people who become infected with EV-D68 are asymptomatic or experience mild, cold-like symptoms. Researchers and physicians are working to understand if there is a connection between these viral outbreaks and AFM, and if so, why some children but not others experience this sudden muscle weakness and paralysis.”

The study will draw on the expertise of the AFM Task Force, established last fall. The group comprises physicians, scientists, and public health experts from diverse disciplines and institutions who will assist in the ongoing investigation.

The AFM natural history study is funded under contract HHSN272201600018C.

msullivan@mdedge.com

An estimated 1-1.8 million sport-related concussions (SRC) occur per year in patients younger than 18 years of age. Concussion is defined as “a traumatically induced transient disturbance of brain function.” More than 50% of concussions among high school youth are not related to organized sports and between 2% and 15% of athletes in organized sports will sustain a concussion during a season of play.

©s-c-s/Thinkstock

In its position statement on concussion in sports, the American Medical Society for Sports Medicine recommends that student athletes receive specific evaluations, which will be described in this article. The guidelines include recommendations for imaging, treatment, and decision making regarding when as well as whether to return to play. Here is a brief summary of those recommendations.


Preseason: Preseason evaluation includes a preparticipation physical evaluation and discussion of concussion history as well as risk factors associated with prolonged concussion recovery. Neurocognitive tests are available for baseline evaluation. While these may assist with diagnosis and return-to-play decisions, there can be considerable variation in an individual’s baseline score as well as the possibility of changes in that baseline over time. Because of this potential for variability, these tests are not required or accepted as the standard of care.

Sideline assessment: Familiarity with the athlete is the best way to detect subtle changes in personality or performance. Looking at symptoms is still the most sensitive way to diagnose a concussion. Loss of consciousness, seizure, tonic posturing, lack of motor coordination, confusion, amnesia, difficulty with balance, or any cognitive difficulty should prompt removal from play for possible concussion. Once a potential injury is identified, how the athlete responds to the elements of orientation, memory, concentration, speech pattern, and balance should be evaluated. If an athlete has a probable or definite concussion, the athlete needs to be removed from play and cannot return to same-day play, and a more detailed evaluation needs to be done.

Office assessment: It is not unusual for symptoms and testing to normalize by the time an office visit occurs. If this is the case, the visit should focus on recommendations for safe return to school and sport. A standard office evaluation should include taking a history with details of the mechanism of injury and preexisting conditions – such as depression and prior concussion – that can affect concussion recovery. The history should focus on detecting symptoms that typically cause impairment from concussion: headache, ocular-vestibular issues leading to problems with balance, and cognitive issues with difficulty concentrating and remembering, as well as fatigue and mood issues such as anxiety, irritability, and depression. The physical exam should include assessment of ocular and vestibular function, gait, and balance in addition to a neurological exam.

Imaging: Head CT or MRI are rarely indicated. Intracranial bleeds are rare in the context of SRC but can occur. If there is concern for a bleed, then CT scan is the imaging test of choice. MRI may have value for evaluation for atypical or prolonged recovery.

Recovery time: The large majority (80%-90%) of concussed older adolescents and adults return to preinjury levels of function within 2 weeks; in younger athletes, clinical recovery may take up to 4 weeks. The best predictor of recovery from SRC is the number and severity of symptoms.

Treatment: For decades, cognitive and physical rest has been the standard of treatment. However, this is no longer the “gold standard” as it has been shown that strict rest (“cocoon therapy”) after SRC slows recovery and leads to an increased chance of prolonged symptoms. Current consensus guidelines support 24-48 hours of symptom-limited rest, both cognitive and physical, followed by a gradual increase in activity, staying below symptom-exacerbation thresholds. Activity, along with good sleep hygiene, appears to be helpful in facilitating recovery from SRC. In athletes with persistent post concussive symptoms that continue beyond the expected recovery time frame, activities of daily living, school, and exercise that do not significantly exacerbate symptoms are recommended.

Return to learning/play: A concussion can cause temporary deficits in attention, cognitive processing, short-term memory, and executive functioning. School personnel should be informed of the injury and assist in employing an individualized return to learn plan, including academic accommodations. Ultimately, return to sports activities should follow a successful return to the classroom. Return to play involves a stepwise increase in physical demands/activity without symptoms before a student is allowed to participate in full contact play.

Concussion-related risks: Continuing to participate in sports before resolution of concussion can worsen and prolong symptoms of SRC. Returning too early after concussion, before full recovery, increases the risk of recurrent SRC. During the initial post-injury period, returning to sports too early increases the risk for a rare but devastating possibility of second impact syndrome that can be a life-threatening repeat head injury. Studies of long-term mental health diagnoses are conflicting and inconsistent. Chronic traumatic encephalopathy has been described in athletes with a long history of concussions and repetitive sub-symptom head impacts. The degree of exposure needed appears to be variable and dependent on the individual.

Disqualification from play: Because each athlete is individually assessed after SRC, there are no evidence-based studies indicating how many concussions are “safe” for an athlete to have in a lifetime. The decision to stop playing sports is both serious and difficult for most athletes and requires shared decision making between clinician, the athlete, and the athlete’s parents. Factors to consider when determining if disqualification from play is warranted include:

• The total number of concussions experienced by a patient.
• Whether a patient has sustained subsequent concussions with progressively less forceful blows to the head.
• If a patient has sustained multiple concussions,whether the time to complete a full recovery after each concussion event increased.

The bottom line: “Cocoon therapy” is no longer recommended. Consensus guidelines endorse 24-48 hours of symptom-limited cognitive and physical rest followed by a gradual increase in activity, including noncontact physical activity that does not provoke symptoms.

Dr. Belogorodsky is a second-year resident and Dr. Fidler is an associate director in the Family Medicine Residency Program at Abington (Pa.) Jefferson Health. Dr. Skolnik is professor of family and community medicine at Jefferson Medical College, Philadelphia, and an associate director of the family medicine residency program at Abington Jefferson Health.

Reference

Harmon KG et al. American Medical Society for Sports Medicine position statement on concussion in sport. Br J Sports Med. 2019;53:213-25.

An estimated 1-1.8 million sport-related concussions (SRC) occur per year in patients younger than 18 years of age. Concussion is defined as “a traumatically induced transient disturbance of brain function.” More than 50% of concussions among high school youth are not related to organized sports and between 2% and 15% of athletes in organized sports will sustain a concussion during a season of play.

©s-c-s/Thinkstock

In its position statement on concussion in sports, the American Medical Society for Sports Medicine recommends that student athletes receive specific evaluations, which will be described in this article. The guidelines include recommendations for imaging, treatment, and decision making regarding when as well as whether to return to play. Here is a brief summary of those recommendations.


Preseason: Preseason evaluation includes a preparticipation physical evaluation and discussion of concussion history as well as risk factors associated with prolonged concussion recovery. Neurocognitive tests are available for baseline evaluation. While these may assist with diagnosis and return-to-play decisions, there can be considerable variation in an individual’s baseline score as well as the possibility of changes in that baseline over time. Because of this potential for variability, these tests are not required or accepted as the standard of care.

Sideline assessment: Familiarity with the athlete is the best way to detect subtle changes in personality or performance. Looking at symptoms is still the most sensitive way to diagnose a concussion. Loss of consciousness, seizure, tonic posturing, lack of motor coordination, confusion, amnesia, difficulty with balance, or any cognitive difficulty should prompt removal from play for possible concussion. Once a potential injury is identified, how the athlete responds to the elements of orientation, memory, concentration, speech pattern, and balance should be evaluated. If an athlete has a probable or definite concussion, the athlete needs to be removed from play and cannot return to same-day play, and a more detailed evaluation needs to be done.

Office assessment: It is not unusual for symptoms and testing to normalize by the time an office visit occurs. If this is the case, the visit should focus on recommendations for safe return to school and sport. A standard office evaluation should include taking a history with details of the mechanism of injury and preexisting conditions – such as depression and prior concussion – that can affect concussion recovery. The history should focus on detecting symptoms that typically cause impairment from concussion: headache, ocular-vestibular issues leading to problems with balance, and cognitive issues with difficulty concentrating and remembering, as well as fatigue and mood issues such as anxiety, irritability, and depression. The physical exam should include assessment of ocular and vestibular function, gait, and balance in addition to a neurological exam.

Imaging: Head CT or MRI are rarely indicated. Intracranial bleeds are rare in the context of SRC but can occur. If there is concern for a bleed, then CT scan is the imaging test of choice. MRI may have value for evaluation for atypical or prolonged recovery.

Recovery time: The large majority (80%-90%) of concussed older adolescents and adults return to preinjury levels of function within 2 weeks; in younger athletes, clinical recovery may take up to 4 weeks. The best predictor of recovery from SRC is the number and severity of symptoms.

Treatment: For decades, cognitive and physical rest has been the standard of treatment. However, this is no longer the “gold standard” as it has been shown that strict rest (“cocoon therapy”) after SRC slows recovery and leads to an increased chance of prolonged symptoms. Current consensus guidelines support 24-48 hours of symptom-limited rest, both cognitive and physical, followed by a gradual increase in activity, staying below symptom-exacerbation thresholds. Activity, along with good sleep hygiene, appears to be helpful in facilitating recovery from SRC. In athletes with persistent post concussive symptoms that continue beyond the expected recovery time frame, activities of daily living, school, and exercise that do not significantly exacerbate symptoms are recommended.

Return to learning/play: A concussion can cause temporary deficits in attention, cognitive processing, short-term memory, and executive functioning. School personnel should be informed of the injury and assist in employing an individualized return to learn plan, including academic accommodations. Ultimately, return to sports activities should follow a successful return to the classroom. Return to play involves a stepwise increase in physical demands/activity without symptoms before a student is allowed to participate in full contact play.

Concussion-related risks: Continuing to participate in sports before resolution of concussion can worsen and prolong symptoms of SRC. Returning too early after concussion, before full recovery, increases the risk of recurrent SRC. During the initial post-injury period, returning to sports too early increases the risk for a rare but devastating possibility of second impact syndrome that can be a life-threatening repeat head injury. Studies of long-term mental health diagnoses are conflicting and inconsistent. Chronic traumatic encephalopathy has been described in athletes with a long history of concussions and repetitive sub-symptom head impacts. The degree of exposure needed appears to be variable and dependent on the individual.

Disqualification from play: Because each athlete is individually assessed after SRC, there are no evidence-based studies indicating how many concussions are “safe” for an athlete to have in a lifetime. The decision to stop playing sports is both serious and difficult for most athletes and requires shared decision making between clinician, the athlete, and the athlete’s parents. Factors to consider when determining if disqualification from play is warranted include:

• The total number of concussions experienced by a patient.
• Whether a patient has sustained subsequent concussions with progressively less forceful blows to the head.
• If a patient has sustained multiple concussions,whether the time to complete a full recovery after each concussion event increased.

The bottom line: “Cocoon therapy” is no longer recommended. Consensus guidelines endorse 24-48 hours of symptom-limited cognitive and physical rest followed by a gradual increase in activity, including noncontact physical activity that does not provoke symptoms.

Dr. Belogorodsky is a second-year resident and Dr. Fidler is an associate director in the Family Medicine Residency Program at Abington (Pa.) Jefferson Health. Dr. Skolnik is professor of family and community medicine at Jefferson Medical College, Philadelphia, and an associate director of the family medicine residency program at Abington Jefferson Health.

Reference

Harmon KG et al. American Medical Society for Sports Medicine position statement on concussion in sport. Br J Sports Med. 2019;53:213-25.

An estimated 1-1.8 million sport-related concussions (SRC) occur per year in patients younger than 18 years of age. Concussion is defined as “a traumatically induced transient disturbance of brain function.” More than 50% of concussions among high school youth are not related to organized sports and between 2% and 15% of athletes in organized sports will sustain a concussion during a season of play.

©s-c-s/Thinkstock

In its position statement on concussion in sports, the American Medical Society for Sports Medicine recommends that student athletes receive specific evaluations, which will be described in this article. The guidelines include recommendations for imaging, treatment, and decision making regarding when as well as whether to return to play. Here is a brief summary of those recommendations.


Preseason: Preseason evaluation includes a preparticipation physical evaluation and discussion of concussion history as well as risk factors associated with prolonged concussion recovery. Neurocognitive tests are available for baseline evaluation. While these may assist with diagnosis and return-to-play decisions, there can be considerable variation in an individual’s baseline score as well as the possibility of changes in that baseline over time. Because of this potential for variability, these tests are not required or accepted as the standard of care.

Sideline assessment: Familiarity with the athlete is the best way to detect subtle changes in personality or performance. Looking at symptoms is still the most sensitive way to diagnose a concussion. Loss of consciousness, seizure, tonic posturing, lack of motor coordination, confusion, amnesia, difficulty with balance, or any cognitive difficulty should prompt removal from play for possible concussion. Once a potential injury is identified, how the athlete responds to the elements of orientation, memory, concentration, speech pattern, and balance should be evaluated. If an athlete has a probable or definite concussion, the athlete needs to be removed from play and cannot return to same-day play, and a more detailed evaluation needs to be done.

Office assessment: It is not unusual for symptoms and testing to normalize by the time an office visit occurs. If this is the case, the visit should focus on recommendations for safe return to school and sport. A standard office evaluation should include taking a history with details of the mechanism of injury and preexisting conditions – such as depression and prior concussion – that can affect concussion recovery. The history should focus on detecting symptoms that typically cause impairment from concussion: headache, ocular-vestibular issues leading to problems with balance, and cognitive issues with difficulty concentrating and remembering, as well as fatigue and mood issues such as anxiety, irritability, and depression. The physical exam should include assessment of ocular and vestibular function, gait, and balance in addition to a neurological exam.

Imaging: Head CT or MRI are rarely indicated. Intracranial bleeds are rare in the context of SRC but can occur. If there is concern for a bleed, then CT scan is the imaging test of choice. MRI may have value for evaluation for atypical or prolonged recovery.

Recovery time: The large majority (80%-90%) of concussed older adolescents and adults return to preinjury levels of function within 2 weeks; in younger athletes, clinical recovery may take up to 4 weeks. The best predictor of recovery from SRC is the number and severity of symptoms.

Treatment: For decades, cognitive and physical rest has been the standard of treatment. However, this is no longer the “gold standard” as it has been shown that strict rest (“cocoon therapy”) after SRC slows recovery and leads to an increased chance of prolonged symptoms. Current consensus guidelines support 24-48 hours of symptom-limited rest, both cognitive and physical, followed by a gradual increase in activity, staying below symptom-exacerbation thresholds. Activity, along with good sleep hygiene, appears to be helpful in facilitating recovery from SRC. In athletes with persistent post concussive symptoms that continue beyond the expected recovery time frame, activities of daily living, school, and exercise that do not significantly exacerbate symptoms are recommended.

Return to learning/play: A concussion can cause temporary deficits in attention, cognitive processing, short-term memory, and executive functioning. School personnel should be informed of the injury and assist in employing an individualized return to learn plan, including academic accommodations. Ultimately, return to sports activities should follow a successful return to the classroom. Return to play involves a stepwise increase in physical demands/activity without symptoms before a student is allowed to participate in full contact play.

Concussion-related risks: Continuing to participate in sports before resolution of concussion can worsen and prolong symptoms of SRC. Returning too early after concussion, before full recovery, increases the risk of recurrent SRC. During the initial post-injury period, returning to sports too early increases the risk for a rare but devastating possibility of second impact syndrome that can be a life-threatening repeat head injury. Studies of long-term mental health diagnoses are conflicting and inconsistent. Chronic traumatic encephalopathy has been described in athletes with a long history of concussions and repetitive sub-symptom head impacts. The degree of exposure needed appears to be variable and dependent on the individual.

Disqualification from play: Because each athlete is individually assessed after SRC, there are no evidence-based studies indicating how many concussions are “safe” for an athlete to have in a lifetime. The decision to stop playing sports is both serious and difficult for most athletes and requires shared decision making between clinician, the athlete, and the athlete’s parents. Factors to consider when determining if disqualification from play is warranted include:

• The total number of concussions experienced by a patient.
• Whether a patient has sustained subsequent concussions with progressively less forceful blows to the head.
• If a patient has sustained multiple concussions,whether the time to complete a full recovery after each concussion event increased.

The bottom line: “Cocoon therapy” is no longer recommended. Consensus guidelines endorse 24-48 hours of symptom-limited cognitive and physical rest followed by a gradual increase in activity, including noncontact physical activity that does not provoke symptoms.

Dr. Belogorodsky is a second-year resident and Dr. Fidler is an associate director in the Family Medicine Residency Program at Abington (Pa.) Jefferson Health. Dr. Skolnik is professor of family and community medicine at Jefferson Medical College, Philadelphia, and an associate director of the family medicine residency program at Abington Jefferson Health.

Reference

Harmon KG et al. American Medical Society for Sports Medicine position statement on concussion in sport. Br J Sports Med. 2019;53:213-25.

PHILADELPHIA – People with moderately high levels of riboflavin consumption from food – two to three times the recommended dietary allowance – had a significantly lower prevalence of a recent severe or migraine headache in a study of more than 3,600 younger U.S. adults.

Adults 20-50 years old who consumed 2.07-2.87 mg riboflavin (vitamin B₂) in food a day based on a 24-hour recall questionnaire had an adjusted, statistically significant 27% reduced prevalence of a recent severe or migraine headache, compared with people in the lowest quartile of dietary riboflavin intake, 1.45 mg/day or less, Margaret Slavin, Ph.D., said at the annual meeting of the American Headache Society. Foods particularly high in riboflavin include eggs, milk, and meat.

Dietary riboflavin intakes greater than 2.87 mg/day were not linked to a difference in the prevalence of a recent history of severe or migraine headache, compared with lowest-quartile consumption. Additionally, riboflavin intake from supplements alone at any level of consumption also showed no statistically significant link with the prevalence of a recent, severe headache, said Dr. Slavin, a nutrition and food studies researcher at George Mason University in Fairfax, Va.

The “vast majority” of people in the study had a riboflavin intake that at least matched the U.S. recommended dietary allowance (RDA),1.3 mg/ day for men and 1.1 mg/day for women), “but it’s possible that people with migraine headaches need more riboflavin,” Dr. Slavin suggested. Professional societies in the United States (Neurology. 2012 Apr;78[17]: 1346-53) and Canada (Can J Neurol Sci. 2012 Mar;39[Suppl 2]S8-S28) have gone on record with some level of recommendation for a daily riboflavin supplement of 400 mg to prevent migraine headaches, she said.

A U.S. guideline that included riboflavin has been “retired” because of an issue unrelated to riboflavin, according to the Neurology website.

The new study ran data collected in the biennial National Health and Nutrition Examination Survey (NHANES), specifically the surveys from 2001-2002 and 2003-2004. The combined data included 5,528 adults 20-50 years old, and 3,634 with complete data and without an excluding condition such as pregnancy, diabetes, or menopause. Among the study participants 884 reported having “severe headaches or migraines,” during the 3 months preceding the survey and the remaining 2,750 people served as controls. People who reported recent severe headache or migraine overall had a significantly lower average amount of vitamin B2 in their diet than did the controls, but the two subgroups showed no significant differences in their levels of riboflavin intake from supplements, or from both diet and supplements combined.

The researchers calculated odds ratios for people having severe headaches or migraines relative to their riboflavin-intake quartile, and they adjusted the findings for age, sex, body mass index, and alcohol intake.

Further analysis that looked at total riboflavin intake, from both food and supplements, showed that the two middle quartiles for this metric, with a combined riboflavin intake of 1.6-3.8 mg/day, had a significantly reduced prevalence of recent severe or migraine headaches, compared with the lowest-intake quartile, with an odds ratio that roughly matched the dietary riboflavin analysis.

Dr. Slavin has received research funding from the Egg Nutrition Center, the Maryland Soybean Board, the McCormick Science Institute, and PepsiCo.

mzoler@mdedge.com

SOURCE: Slavin M. Headache. 2019 June;59[S1]:1-208, Abstract LBOR04.

PHILADELPHIA – People with moderately high levels of riboflavin consumption from food – two to three times the recommended dietary allowance – had a significantly lower prevalence of a recent severe or migraine headache in a study of more than 3,600 younger U.S. adults.

Adults 20-50 years old who consumed 2.07-2.87 mg riboflavin (vitamin B₂) in food a day based on a 24-hour recall questionnaire had an adjusted, statistically significant 27% reduced prevalence of a recent severe or migraine headache, compared with people in the lowest quartile of dietary riboflavin intake, 1.45 mg/day or less, Margaret Slavin, Ph.D., said at the annual meeting of the American Headache Society. Foods particularly high in riboflavin include eggs, milk, and meat.

Dietary riboflavin intakes greater than 2.87 mg/day were not linked to a difference in the prevalence of a recent history of severe or migraine headache, compared with lowest-quartile consumption. Additionally, riboflavin intake from supplements alone at any level of consumption also showed no statistically significant link with the prevalence of a recent, severe headache, said Dr. Slavin, a nutrition and food studies researcher at George Mason University in Fairfax, Va.

The “vast majority” of people in the study had a riboflavin intake that at least matched the U.S. recommended dietary allowance (RDA),1.3 mg/ day for men and 1.1 mg/day for women), “but it’s possible that people with migraine headaches need more riboflavin,” Dr. Slavin suggested. Professional societies in the United States (Neurology. 2012 Apr;78[17]: 1346-53) and Canada (Can J Neurol Sci. 2012 Mar;39[Suppl 2]S8-S28) have gone on record with some level of recommendation for a daily riboflavin supplement of 400 mg to prevent migraine headaches, she said.

A U.S. guideline that included riboflavin has been “retired” because of an issue unrelated to riboflavin, according to the Neurology website.

The new study ran data collected in the biennial National Health and Nutrition Examination Survey (NHANES), specifically the surveys from 2001-2002 and 2003-2004. The combined data included 5,528 adults 20-50 years old, and 3,634 with complete data and without an excluding condition such as pregnancy, diabetes, or menopause. Among the study participants 884 reported having “severe headaches or migraines,” during the 3 months preceding the survey and the remaining 2,750 people served as controls. People who reported recent severe headache or migraine overall had a significantly lower average amount of vitamin B2 in their diet than did the controls, but the two subgroups showed no significant differences in their levels of riboflavin intake from supplements, or from both diet and supplements combined.

The researchers calculated odds ratios for people having severe headaches or migraines relative to their riboflavin-intake quartile, and they adjusted the findings for age, sex, body mass index, and alcohol intake.

Further analysis that looked at total riboflavin intake, from both food and supplements, showed that the two middle quartiles for this metric, with a combined riboflavin intake of 1.6-3.8 mg/day, had a significantly reduced prevalence of recent severe or migraine headaches, compared with the lowest-intake quartile, with an odds ratio that roughly matched the dietary riboflavin analysis.

Dr. Slavin has received research funding from the Egg Nutrition Center, the Maryland Soybean Board, the McCormick Science Institute, and PepsiCo.

mzoler@mdedge.com

SOURCE: Slavin M. Headache. 2019 June;59[S1]:1-208, Abstract LBOR04.

PHILADELPHIA – People with moderately high levels of riboflavin consumption from food – two to three times the recommended dietary allowance – had a significantly lower prevalence of a recent severe or migraine headache in a study of more than 3,600 younger U.S. adults.

Adults 20-50 years old who consumed 2.07-2.87 mg riboflavin (vitamin B₂) in food a day based on a 24-hour recall questionnaire had an adjusted, statistically significant 27% reduced prevalence of a recent severe or migraine headache, compared with people in the lowest quartile of dietary riboflavin intake, 1.45 mg/day or less, Margaret Slavin, Ph.D., said at the annual meeting of the American Headache Society. Foods particularly high in riboflavin include eggs, milk, and meat.

Dietary riboflavin intakes greater than 2.87 mg/day were not linked to a difference in the prevalence of a recent history of severe or migraine headache, compared with lowest-quartile consumption. Additionally, riboflavin intake from supplements alone at any level of consumption also showed no statistically significant link with the prevalence of a recent, severe headache, said Dr. Slavin, a nutrition and food studies researcher at George Mason University in Fairfax, Va.

The “vast majority” of people in the study had a riboflavin intake that at least matched the U.S. recommended dietary allowance (RDA),1.3 mg/ day for men and 1.1 mg/day for women), “but it’s possible that people with migraine headaches need more riboflavin,” Dr. Slavin suggested. Professional societies in the United States (Neurology. 2012 Apr;78[17]: 1346-53) and Canada (Can J Neurol Sci. 2012 Mar;39[Suppl 2]S8-S28) have gone on record with some level of recommendation for a daily riboflavin supplement of 400 mg to prevent migraine headaches, she said.

A U.S. guideline that included riboflavin has been “retired” because of an issue unrelated to riboflavin, according to the Neurology website.

The new study ran data collected in the biennial National Health and Nutrition Examination Survey (NHANES), specifically the surveys from 2001-2002 and 2003-2004. The combined data included 5,528 adults 20-50 years old, and 3,634 with complete data and without an excluding condition such as pregnancy, diabetes, or menopause. Among the study participants 884 reported having “severe headaches or migraines,” during the 3 months preceding the survey and the remaining 2,750 people served as controls. People who reported recent severe headache or migraine overall had a significantly lower average amount of vitamin B2 in their diet than did the controls, but the two subgroups showed no significant differences in their levels of riboflavin intake from supplements, or from both diet and supplements combined.

The researchers calculated odds ratios for people having severe headaches or migraines relative to their riboflavin-intake quartile, and they adjusted the findings for age, sex, body mass index, and alcohol intake.

Further analysis that looked at total riboflavin intake, from both food and supplements, showed that the two middle quartiles for this metric, with a combined riboflavin intake of 1.6-3.8 mg/day, had a significantly reduced prevalence of recent severe or migraine headaches, compared with the lowest-intake quartile, with an odds ratio that roughly matched the dietary riboflavin analysis.

Dr. Slavin has received research funding from the Egg Nutrition Center, the Maryland Soybean Board, the McCormick Science Institute, and PepsiCo.

mzoler@mdedge.com

SOURCE: Slavin M. Headache. 2019 June;59[S1]:1-208, Abstract LBOR04.

A 37-year-old woman presents to her primary care clinic with a chief complaint of depression. She was diagnosed with relapsing multiple sclerosis (MS) at age 29 and is currently taking an injectable preventive therapy. Over the past 6 months, she has had increased marital strain secondary to losing her job because “I couldn’t mentally keep up with the work anymore.” This has caused financial difficulties for her family. In addition, she tires easily and has been napping in the afternoon. She and her husband are experiencing intimacy difficulties, and she confirms problems with vaginal dryness and a general loss of her sexual drive.

Sexual dysfunction in MS is common, affecting 40% to 80% of women and 50% to 90% of men with MS. It is an “invisible” symptom, similar to fatigue, cognitive dysfunction, and pain.^1-3

There are three ways that MS patients can be affected by sexual dysfunction, and they are categorized as primary, secondary, and tertiary. Primary sexual dysfunction results from demyelination/axonal destruction of the central nervous system, which potentially leads to altered genital sensation or paresthesia. Secondary sexual dysfunction stems from nonsexual MS symptoms, such as fatigue, spasticity, tremor, impairments in concentration/attention, and iatrogenic causes (eg, adverse effects of medication). Tertiary sexual dysfunction involves the psychosocial/cultural aspects of the disease that can impact a patient’s sexual drive.

SYMPTOMS

Like many other symptoms associated with MS, the symptoms of sexual dysfunction are highly variable. In women, the most common complaints are fatigue, decrease in genital sensation (27%-47%), decrease in libido (31%-74%) and vaginal lubrication (36%-48%), and difficulty with orgasm.⁴ In men with MS, in addition to erectile problems, surveys have identified decreased genital sensation, fatigue (75%), difficulty with ejaculation (18%-50%), decreased interest or arousal (39%), and anorgasmia (37%) as fairly common complaints.²

TREATMENT

Managing sexual dysfunction in a patient with MS is dependent on the underlying problem. Some examples include

• For many patients, their disease causes significant anxiety and worry about current and potentially future disability—which can make intimacy more difficult. Sometimes, referral to a mental health professional may be required to help the patient with individual and/or couples counseling to further elucidate underlying intimacy issues.
• For patients experiencing MS-associated fatigue, suggest planning for sexual activity in the morning, since fatigue is known to worsen throughout the day.
• For those who qualify for antidepressant medications, remember that some (eg, selective serotonin reuptake inhibitors) can further decrease libido and therefore should be avoided if possible.
• For women who have difficulty with lubrication, a nonpetroleum-based lubricant may reduce vaginal dryness, while use of a vibrator may assist with genital stimulation.
• For men who cannot maintain erection, phosphodiesterase inhibitor drugs (eg, sildenafil) can be helpful; other options include alprostadil urethral suppositories and intracavernous injections.

The patient is screened for depression using the Patient Health Questionnaire, which yields a score of 17 (moderately severe). You discuss the need for active treatment with her, and she agrees to start an antidepressant medication. Bupropion is chosen, given its effectiveness and lack of adverse effects (including sexual dysfunction). The patient also is encouraged to use nonpetroleum-based lubricants. Finally, a referral is made for couples counseling, and a 6-week follow-up appointment is scheduled.

CONCLUSION

Sexual dysfunction in MS is quite common in both women and men, and the related symptoms are often multifactorial. Strategies to address sexual dysfunction in MS require a tailored approach. Fortunately, any treatments for sexual dysfunction initiated by the patient’s primary care provider will not have an adverse effect on the patient’s outcome with MS. For more complicated cases of MS-associated sexual dysfunction, urology referral is recommended.

A 37-year-old woman presents to her primary care clinic with a chief complaint of depression. She was diagnosed with relapsing multiple sclerosis (MS) at age 29 and is currently taking an injectable preventive therapy. Over the past 6 months, she has had increased marital strain secondary to losing her job because “I couldn’t mentally keep up with the work anymore.” This has caused financial difficulties for her family. In addition, she tires easily and has been napping in the afternoon. She and her husband are experiencing intimacy difficulties, and she confirms problems with vaginal dryness and a general loss of her sexual drive.

Sexual dysfunction in MS is common, affecting 40% to 80% of women and 50% to 90% of men with MS. It is an “invisible” symptom, similar to fatigue, cognitive dysfunction, and pain.^1-3

There are three ways that MS patients can be affected by sexual dysfunction, and they are categorized as primary, secondary, and tertiary. Primary sexual dysfunction results from demyelination/axonal destruction of the central nervous system, which potentially leads to altered genital sensation or paresthesia. Secondary sexual dysfunction stems from nonsexual MS symptoms, such as fatigue, spasticity, tremor, impairments in concentration/attention, and iatrogenic causes (eg, adverse effects of medication). Tertiary sexual dysfunction involves the psychosocial/cultural aspects of the disease that can impact a patient’s sexual drive.

SYMPTOMS

Like many other symptoms associated with MS, the symptoms of sexual dysfunction are highly variable. In women, the most common complaints are fatigue, decrease in genital sensation (27%-47%), decrease in libido (31%-74%) and vaginal lubrication (36%-48%), and difficulty with orgasm.⁴ In men with MS, in addition to erectile problems, surveys have identified decreased genital sensation, fatigue (75%), difficulty with ejaculation (18%-50%), decreased interest or arousal (39%), and anorgasmia (37%) as fairly common complaints.²

TREATMENT

Managing sexual dysfunction in a patient with MS is dependent on the underlying problem. Some examples include

• For many patients, their disease causes significant anxiety and worry about current and potentially future disability—which can make intimacy more difficult. Sometimes, referral to a mental health professional may be required to help the patient with individual and/or couples counseling to further elucidate underlying intimacy issues.
• For patients experiencing MS-associated fatigue, suggest planning for sexual activity in the morning, since fatigue is known to worsen throughout the day.
• For those who qualify for antidepressant medications, remember that some (eg, selective serotonin reuptake inhibitors) can further decrease libido and therefore should be avoided if possible.
• For women who have difficulty with lubrication, a nonpetroleum-based lubricant may reduce vaginal dryness, while use of a vibrator may assist with genital stimulation.
• For men who cannot maintain erection, phosphodiesterase inhibitor drugs (eg, sildenafil) can be helpful; other options include alprostadil urethral suppositories and intracavernous injections.

The patient is screened for depression using the Patient Health Questionnaire, which yields a score of 17 (moderately severe). You discuss the need for active treatment with her, and she agrees to start an antidepressant medication. Bupropion is chosen, given its effectiveness and lack of adverse effects (including sexual dysfunction). The patient also is encouraged to use nonpetroleum-based lubricants. Finally, a referral is made for couples counseling, and a 6-week follow-up appointment is scheduled.

CONCLUSION

Sexual dysfunction in MS is quite common in both women and men, and the related symptoms are often multifactorial. Strategies to address sexual dysfunction in MS require a tailored approach. Fortunately, any treatments for sexual dysfunction initiated by the patient’s primary care provider will not have an adverse effect on the patient’s outcome with MS. For more complicated cases of MS-associated sexual dysfunction, urology referral is recommended.

A 37-year-old woman presents to her primary care clinic with a chief complaint of depression. She was diagnosed with relapsing multiple sclerosis (MS) at age 29 and is currently taking an injectable preventive therapy. Over the past 6 months, she has had increased marital strain secondary to losing her job because “I couldn’t mentally keep up with the work anymore.” This has caused financial difficulties for her family. In addition, she tires easily and has been napping in the afternoon. She and her husband are experiencing intimacy difficulties, and she confirms problems with vaginal dryness and a general loss of her sexual drive.

Sexual dysfunction in MS is common, affecting 40% to 80% of women and 50% to 90% of men with MS. It is an “invisible” symptom, similar to fatigue, cognitive dysfunction, and pain.^1-3

There are three ways that MS patients can be affected by sexual dysfunction, and they are categorized as primary, secondary, and tertiary. Primary sexual dysfunction results from demyelination/axonal destruction of the central nervous system, which potentially leads to altered genital sensation or paresthesia. Secondary sexual dysfunction stems from nonsexual MS symptoms, such as fatigue, spasticity, tremor, impairments in concentration/attention, and iatrogenic causes (eg, adverse effects of medication). Tertiary sexual dysfunction involves the psychosocial/cultural aspects of the disease that can impact a patient’s sexual drive.

SYMPTOMS

Like many other symptoms associated with MS, the symptoms of sexual dysfunction are highly variable. In women, the most common complaints are fatigue, decrease in genital sensation (27%-47%), decrease in libido (31%-74%) and vaginal lubrication (36%-48%), and difficulty with orgasm.⁴ In men with MS, in addition to erectile problems, surveys have identified decreased genital sensation, fatigue (75%), difficulty with ejaculation (18%-50%), decreased interest or arousal (39%), and anorgasmia (37%) as fairly common complaints.²

TREATMENT

Managing sexual dysfunction in a patient with MS is dependent on the underlying problem. Some examples include

• For many patients, their disease causes significant anxiety and worry about current and potentially future disability—which can make intimacy more difficult. Sometimes, referral to a mental health professional may be required to help the patient with individual and/or couples counseling to further elucidate underlying intimacy issues.
• For patients experiencing MS-associated fatigue, suggest planning for sexual activity in the morning, since fatigue is known to worsen throughout the day.
• For those who qualify for antidepressant medications, remember that some (eg, selective serotonin reuptake inhibitors) can further decrease libido and therefore should be avoided if possible.
• For women who have difficulty with lubrication, a nonpetroleum-based lubricant may reduce vaginal dryness, while use of a vibrator may assist with genital stimulation.
• For men who cannot maintain erection, phosphodiesterase inhibitor drugs (eg, sildenafil) can be helpful; other options include alprostadil urethral suppositories and intracavernous injections.

The patient is screened for depression using the Patient Health Questionnaire, which yields a score of 17 (moderately severe). You discuss the need for active treatment with her, and she agrees to start an antidepressant medication. Bupropion is chosen, given its effectiveness and lack of adverse effects (including sexual dysfunction). The patient also is encouraged to use nonpetroleum-based lubricants. Finally, a referral is made for couples counseling, and a 6-week follow-up appointment is scheduled.

CONCLUSION

Sexual dysfunction in MS is quite common in both women and men, and the related symptoms are often multifactorial. Strategies to address sexual dysfunction in MS require a tailored approach. Fortunately, any treatments for sexual dysfunction initiated by the patient’s primary care provider will not have an adverse effect on the patient’s outcome with MS. For more complicated cases of MS-associated sexual dysfunction, urology referral is recommended.