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Restless Leg Syndrome More Common in Temporal Lobe Epilepsy
Patients with temporal lobe epilepsy (TLE) are more likely to experience restless leg syndrome (RLS) than the general public, according to a recent outpatient clinic analysis, which compared 98 TLE patients to 50 controls who did not have a history of epilepsy or any family members with the disorder. The investigators also found that the odds of developing RLS were 4.6 times greater in patients with right-sided TLE, when compared to left-sided TLE. They also suggested that worsening RLS may serve as an early warning of an impending seizure in some patients.
Geyer JD, Geyer EE, Fetterman Z, Carney PR. Epilepsy and restless legs syndrome. Epilepsy Behav. 2017;68:41-44.
Patients with temporal lobe epilepsy (TLE) are more likely to experience restless leg syndrome (RLS) than the general public, according to a recent outpatient clinic analysis, which compared 98 TLE patients to 50 controls who did not have a history of epilepsy or any family members with the disorder. The investigators also found that the odds of developing RLS were 4.6 times greater in patients with right-sided TLE, when compared to left-sided TLE. They also suggested that worsening RLS may serve as an early warning of an impending seizure in some patients.
Geyer JD, Geyer EE, Fetterman Z, Carney PR. Epilepsy and restless legs syndrome. Epilepsy Behav. 2017;68:41-44.
Patients with temporal lobe epilepsy (TLE) are more likely to experience restless leg syndrome (RLS) than the general public, according to a recent outpatient clinic analysis, which compared 98 TLE patients to 50 controls who did not have a history of epilepsy or any family members with the disorder. The investigators also found that the odds of developing RLS were 4.6 times greater in patients with right-sided TLE, when compared to left-sided TLE. They also suggested that worsening RLS may serve as an early warning of an impending seizure in some patients.
Geyer JD, Geyer EE, Fetterman Z, Carney PR. Epilepsy and restless legs syndrome. Epilepsy Behav. 2017;68:41-44.
Half of Patients With Epilepsy Do Not Receive Medication Soon Enough
A recent analysis of Medicare records has found that among 3706 new cases of epilepsy, 79.6% had received 1 antiepilepsy drug within 1 year of follow-up. However, only 50% of patients had received prompt therapy, which was defined as receiving the first medication within 30 days of diagnosis. The delay in initiating monotherapy was detected when researchers performed retrospective analyses of 2008–2010 Medicare administrative claims that were obtained from a 5% random sample of patients. The investigators have called for additional research to determine the reasons for the delays and have urged the development of new paradigms to improve patient care.
Martin RC, Faught E, Szaflarski JP, et al. What does the U.S. Medicare administrative claims database tell us about initial antiepileptic drug treatment for older adults with new-onset epilepsy? Epilepsia. 2017[Epub ahead of print]
A recent analysis of Medicare records has found that among 3706 new cases of epilepsy, 79.6% had received 1 antiepilepsy drug within 1 year of follow-up. However, only 50% of patients had received prompt therapy, which was defined as receiving the first medication within 30 days of diagnosis. The delay in initiating monotherapy was detected when researchers performed retrospective analyses of 2008–2010 Medicare administrative claims that were obtained from a 5% random sample of patients. The investigators have called for additional research to determine the reasons for the delays and have urged the development of new paradigms to improve patient care.
Martin RC, Faught E, Szaflarski JP, et al. What does the U.S. Medicare administrative claims database tell us about initial antiepileptic drug treatment for older adults with new-onset epilepsy? Epilepsia. 2017[Epub ahead of print]
A recent analysis of Medicare records has found that among 3706 new cases of epilepsy, 79.6% had received 1 antiepilepsy drug within 1 year of follow-up. However, only 50% of patients had received prompt therapy, which was defined as receiving the first medication within 30 days of diagnosis. The delay in initiating monotherapy was detected when researchers performed retrospective analyses of 2008–2010 Medicare administrative claims that were obtained from a 5% random sample of patients. The investigators have called for additional research to determine the reasons for the delays and have urged the development of new paradigms to improve patient care.
Martin RC, Faught E, Szaflarski JP, et al. What does the U.S. Medicare administrative claims database tell us about initial antiepileptic drug treatment for older adults with new-onset epilepsy? Epilepsia. 2017[Epub ahead of print]
Changes in Pre-Seizure Interictal Spike Shape Repeated During Post-Seizure Sleep
Tapping Electronic Medical Records to Improve Quality of Care
Electronic medical records have the potential to improve quality of care among patients with epilepsy. With that goal in mind, Jaishree Narayanan et al have created a digital toolkit that allows providers to capture structured clinical data at the point of care. The software facilitates writing notes and inputting test scores in several domains, including Generalized Anxiety Disorder-7, Neurological Disorders Depression Inventory for Epilepsy, the Montreal Cognitive Assessment/Short Test of Mental Status, and the Medical Research Council Prognostic Index.
Narayanan J, Dobrin S, Choi J, et al. Structured clinical documentation in the electronic medical record to improve quality and to support practice-based research in epilepsy. Epilepsia. 2017;58(1):68-76.
Electronic medical records have the potential to improve quality of care among patients with epilepsy. With that goal in mind, Jaishree Narayanan et al have created a digital toolkit that allows providers to capture structured clinical data at the point of care. The software facilitates writing notes and inputting test scores in several domains, including Generalized Anxiety Disorder-7, Neurological Disorders Depression Inventory for Epilepsy, the Montreal Cognitive Assessment/Short Test of Mental Status, and the Medical Research Council Prognostic Index.
Narayanan J, Dobrin S, Choi J, et al. Structured clinical documentation in the electronic medical record to improve quality and to support practice-based research in epilepsy. Epilepsia. 2017;58(1):68-76.
Electronic medical records have the potential to improve quality of care among patients with epilepsy. With that goal in mind, Jaishree Narayanan et al have created a digital toolkit that allows providers to capture structured clinical data at the point of care. The software facilitates writing notes and inputting test scores in several domains, including Generalized Anxiety Disorder-7, Neurological Disorders Depression Inventory for Epilepsy, the Montreal Cognitive Assessment/Short Test of Mental Status, and the Medical Research Council Prognostic Index.
Narayanan J, Dobrin S, Choi J, et al. Structured clinical documentation in the electronic medical record to improve quality and to support practice-based research in epilepsy. Epilepsia. 2017;58(1):68-76.
Finding the Best Oxygen Saturation Setting for Seizure Monitoring
To determine the value of pulse oximetry in detecting seizures, Goldenholz et al evaluated 193 seizures among 45 patients who were being monitored with video EEG, SpO2, and EKG. Their analysis found that SpO2 thresholds of 80%-86% were able to detect 63%-73% of generalized convulsions and 20%-28% of focal seizures. While the researchers concluded that continuous SpO2 monitoring requires a tradeoff between false alarms and accurate detection of seizures, they believe an alarm setting of 86% is likely to be worthwhile in patients who experience fewer false alarms and a setting of 80% would be better for those who have more false alarms.
Goldenholz DM, Kuhn A, Austermuehle A, et al. Long-term monitoring of cardiorespiratory patterns in drug-resistant epilepsy. Epilepsia. 2017;58(1):77-84.
To determine the value of pulse oximetry in detecting seizures, Goldenholz et al evaluated 193 seizures among 45 patients who were being monitored with video EEG, SpO2, and EKG. Their analysis found that SpO2 thresholds of 80%-86% were able to detect 63%-73% of generalized convulsions and 20%-28% of focal seizures. While the researchers concluded that continuous SpO2 monitoring requires a tradeoff between false alarms and accurate detection of seizures, they believe an alarm setting of 86% is likely to be worthwhile in patients who experience fewer false alarms and a setting of 80% would be better for those who have more false alarms.
Goldenholz DM, Kuhn A, Austermuehle A, et al. Long-term monitoring of cardiorespiratory patterns in drug-resistant epilepsy. Epilepsia. 2017;58(1):77-84.
To determine the value of pulse oximetry in detecting seizures, Goldenholz et al evaluated 193 seizures among 45 patients who were being monitored with video EEG, SpO2, and EKG. Their analysis found that SpO2 thresholds of 80%-86% were able to detect 63%-73% of generalized convulsions and 20%-28% of focal seizures. While the researchers concluded that continuous SpO2 monitoring requires a tradeoff between false alarms and accurate detection of seizures, they believe an alarm setting of 86% is likely to be worthwhile in patients who experience fewer false alarms and a setting of 80% would be better for those who have more false alarms.
Goldenholz DM, Kuhn A, Austermuehle A, et al. Long-term monitoring of cardiorespiratory patterns in drug-resistant epilepsy. Epilepsia. 2017;58(1):77-84.
AAN Guideline Assesses fMRI for Presurgical Evaluation of Patients With Epilepsy
When preparing for epilepsy surgery, neurologists may consider using functional MRI (fMRI) instead of the intracarotid amobarbital procedure (IAP) to map language and memory functions in the brain, according to a practice guideline developed by the American Academy of Neurology (AAN). The current evidence is weak, however, and clinicians should advise patients carefully about the benefits and risks of IAP (also known as the Wada test) and fMRI, according to the guideline, which was published online ahead of print January 11 in Neurology.
IAP, an invasive technique, is the current standard for presurgical evaluation in epilepsy. fMRI is noninvasive and also considered safe. Neither of the two methods has been standardized. “Because fMRI is becoming more widely available, we wanted to see how it compares to the Wada test,” said lead author Jerzy Szaflarski, MD, PhD, Professor of Neurology at the University of Alabama at Birmingham. “While the risks associated with the Wada test are rare, they can be serious, including stroke and injury to the carotid artery.” 
The AAN formed an 11-member panel to draft the guidelines. Two panelists independently selected 37 possibly relevant articles. Studies with fewer than 15 cases, case reports, meta-analyses, and editorials were excluded. Two panelists rated each article according to the AAN’s diagnostic and prognostic classification of evidence, and the panel ultimately developed consensus recommendations.
Data Support fMRI for Certain Situations
Class II data indicated that fMRI possibly provides language lateralization information concordant with that of IAP in 87% of people with medial temporal lobe epilepsy and in 81% of patients with extratemporal epilepsy. Current comparative data for temporal tumors or lateral temporal cases are insufficient to draw conclusions. Thus, fMRI may be considered as an option for lateralizing language functions in place of IAP in patients with medial temporal lobe epilepsy (Level C), temporal epilepsy in general (Level C), or extratemporal epilepsy (Level C), said the authors. The evidence is unclear for patients with temporal neocortical epilepsy or temporal tumors (Level U).
One Class II study and one Class III study suggested that fMRI is possibly effective in aiding the prediction of postsurgical language deficits in patients undergoing presurgical evaluation for possible temporal lobectomy. The authors recommended that fMRI may be considered for predicting postsurgical language outcomes after anterior temporal lobe resection for the control of temporal lobe epilepsy (Level C).
Class II evidence suggests that in patients with medial temporal lobe epilepsy, fMRI is comparable with IAP in its ability to lateralize memory functions and may be used for this purpose. The authors recommended that fMRI may be considered as an option to lateralize memory functions in place of IAP in patients with medial temporal lobe epilepsy (Level C).
Nine Class II studies with different methods together suggested that fMRI leftward activation asymmetry during encoding of verbal material, regardless of whether measured in the medial temporal lobe or in the language network, probably predicts verbal memory decline after left medial temporal lobe surgery. The authors therefore recommended that presurgical fMRI of verbal memory or of language encoding should be considered as an option to predict verbal memory outcome in patients with epilepsy who are undergoing evaluation for left medial temporal lobe surgery (Level B).
A Class II study indicated that fMRI activation asymmetry during nonverbal (ie, scene and face recognition) memory tasks possibly predicts nonverbal memory decline after medial temporal lobe surgery. The authors recommended that presurgical fMRI using nonverbal memory encoding may be considered as a means to predict visuospatial memory outcomes in patients with epilepsy who are undergoing evaluation for temporal lobe surgery (Level C).
Based on data from one Class II study and one Class III study, the authors recommended that presurgical fMRI may be used instead of the IAP for language lateralization in patients with epilepsy who are undergoing evaluation for brain surgery (Level C). “However, when fMRI is used for this purpose, task design, data analysis methods, and epilepsy type should be considered,” they added.
In addition, based on nine Class II studies, the authors recommended that fMRI of language and verbal memory lateralization may be an alternative to IAP memory testing for prediction of verbal memory outcome in medial temporal lobe epilepsy (Level C). The authors note that fMRI is not yet established as an alternative to the IAP for prediction of global amnesia in patients who have undergone anterior temporal lobe surgery.
More and Larger Studies Are Needed
“The imperfect concordance between fMRI and IAP language lateralization leaves open the question of which test is more accurate in discordant cases,” said the authors. Although the IAP is the reference standard, it is subject to limitations resulting from individual variation in arterial anatomy, variable effects of anesthesia, the rate of amobarbital injection, variability in patient cooperation, and variation in testing methods.
Like the IAP, cognitive fMRI “is a complex diagnostic procedure that requires both advanced technical expertise in imaging and expert interaction with patients to elicit adequate levels of task performance, select a set of activation tasks appropriate to the patient’s ability and the clinical aims of the study, instruct the patient on the tasks, administer the tasks during scanning, and evaluate and provide corrective feedback on task performance during the scanning session,” said the authors.
Global amnesia may result from bilateral medial temporal lobe damage, and some neurologists depend on the IAP to evaluate a patient’s risk for this outcome. “Global amnesia is rare after unilateral temporal lobe surgery, however, and occurs mainly when there is preexisting contralateral medial temporal lobe dysfunction,” said the authors. “One possible approach, therefore, is to reserve use of the IAP memory test for those patients at greatest risk for global amnesia, that is, patients undergoing unilateral anterior temporal lobe resection who have structural or functional evidence of damage to the contralateral medial temporal lobe.”
“Larger studies need to be conducted to increase the quality of available evidence,” Dr. Szaflarski concluded.
—Erik Greb
Suggested Reading
Szaflarski JP, Gloss D, Binder JR, et al. Practice guideline summary: Use of fMRI in the presurgical evaluation of patients with epilepsy—Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2017 Jan 11 [Epub ahead of print].
When preparing for epilepsy surgery, neurologists may consider using functional MRI (fMRI) instead of the intracarotid amobarbital procedure (IAP) to map language and memory functions in the brain, according to a practice guideline developed by the American Academy of Neurology (AAN). The current evidence is weak, however, and clinicians should advise patients carefully about the benefits and risks of IAP (also known as the Wada test) and fMRI, according to the guideline, which was published online ahead of print January 11 in Neurology.
IAP, an invasive technique, is the current standard for presurgical evaluation in epilepsy. fMRI is noninvasive and also considered safe. Neither of the two methods has been standardized. “Because fMRI is becoming more widely available, we wanted to see how it compares to the Wada test,” said lead author Jerzy Szaflarski, MD, PhD, Professor of Neurology at the University of Alabama at Birmingham. “While the risks associated with the Wada test are rare, they can be serious, including stroke and injury to the carotid artery.”
The AAN formed an 11-member panel to draft the guidelines. Two panelists independently selected 37 possibly relevant articles. Studies with fewer than 15 cases, case reports, meta-analyses, and editorials were excluded. Two panelists rated each article according to the AAN’s diagnostic and prognostic classification of evidence, and the panel ultimately developed consensus recommendations.
Data Support fMRI for Certain Situations
Class II data indicated that fMRI possibly provides language lateralization information concordant with that of IAP in 87% of people with medial temporal lobe epilepsy and in 81% of patients with extratemporal epilepsy. Current comparative data for temporal tumors or lateral temporal cases are insufficient to draw conclusions. Thus, fMRI may be considered as an option for lateralizing language functions in place of IAP in patients with medial temporal lobe epilepsy (Level C), temporal epilepsy in general (Level C), or extratemporal epilepsy (Level C), said the authors. The evidence is unclear for patients with temporal neocortical epilepsy or temporal tumors (Level U).
One Class II study and one Class III study suggested that fMRI is possibly effective in aiding the prediction of postsurgical language deficits in patients undergoing presurgical evaluation for possible temporal lobectomy. The authors recommended that fMRI may be considered for predicting postsurgical language outcomes after anterior temporal lobe resection for the control of temporal lobe epilepsy (Level C).
Class II evidence suggests that in patients with medial temporal lobe epilepsy, fMRI is comparable with IAP in its ability to lateralize memory functions and may be used for this purpose. The authors recommended that fMRI may be considered as an option to lateralize memory functions in place of IAP in patients with medial temporal lobe epilepsy (Level C).
Nine Class II studies with different methods together suggested that fMRI leftward activation asymmetry during encoding of verbal material, regardless of whether measured in the medial temporal lobe or in the language network, probably predicts verbal memory decline after left medial temporal lobe surgery. The authors therefore recommended that presurgical fMRI of verbal memory or of language encoding should be considered as an option to predict verbal memory outcome in patients with epilepsy who are undergoing evaluation for left medial temporal lobe surgery (Level B).
A Class II study indicated that fMRI activation asymmetry during nonverbal (ie, scene and face recognition) memory tasks possibly predicts nonverbal memory decline after medial temporal lobe surgery. The authors recommended that presurgical fMRI using nonverbal memory encoding may be considered as a means to predict visuospatial memory outcomes in patients with epilepsy who are undergoing evaluation for temporal lobe surgery (Level C).
Based on data from one Class II study and one Class III study, the authors recommended that presurgical fMRI may be used instead of the IAP for language lateralization in patients with epilepsy who are undergoing evaluation for brain surgery (Level C). “However, when fMRI is used for this purpose, task design, data analysis methods, and epilepsy type should be considered,” they added.
In addition, based on nine Class II studies, the authors recommended that fMRI of language and verbal memory lateralization may be an alternative to IAP memory testing for prediction of verbal memory outcome in medial temporal lobe epilepsy (Level C). The authors note that fMRI is not yet established as an alternative to the IAP for prediction of global amnesia in patients who have undergone anterior temporal lobe surgery.
More and Larger Studies Are Needed
“The imperfect concordance between fMRI and IAP language lateralization leaves open the question of which test is more accurate in discordant cases,” said the authors. Although the IAP is the reference standard, it is subject to limitations resulting from individual variation in arterial anatomy, variable effects of anesthesia, the rate of amobarbital injection, variability in patient cooperation, and variation in testing methods.
Like the IAP, cognitive fMRI “is a complex diagnostic procedure that requires both advanced technical expertise in imaging and expert interaction with patients to elicit adequate levels of task performance, select a set of activation tasks appropriate to the patient’s ability and the clinical aims of the study, instruct the patient on the tasks, administer the tasks during scanning, and evaluate and provide corrective feedback on task performance during the scanning session,” said the authors.
Global amnesia may result from bilateral medial temporal lobe damage, and some neurologists depend on the IAP to evaluate a patient’s risk for this outcome. “Global amnesia is rare after unilateral temporal lobe surgery, however, and occurs mainly when there is preexisting contralateral medial temporal lobe dysfunction,” said the authors. “One possible approach, therefore, is to reserve use of the IAP memory test for those patients at greatest risk for global amnesia, that is, patients undergoing unilateral anterior temporal lobe resection who have structural or functional evidence of damage to the contralateral medial temporal lobe.”
“Larger studies need to be conducted to increase the quality of available evidence,” Dr. Szaflarski concluded.
—Erik Greb
Suggested Reading
Szaflarski JP, Gloss D, Binder JR, et al. Practice guideline summary: Use of fMRI in the presurgical evaluation of patients with epilepsy—Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2017 Jan 11 [Epub ahead of print].
When preparing for epilepsy surgery, neurologists may consider using functional MRI (fMRI) instead of the intracarotid amobarbital procedure (IAP) to map language and memory functions in the brain, according to a practice guideline developed by the American Academy of Neurology (AAN). The current evidence is weak, however, and clinicians should advise patients carefully about the benefits and risks of IAP (also known as the Wada test) and fMRI, according to the guideline, which was published online ahead of print January 11 in Neurology.
IAP, an invasive technique, is the current standard for presurgical evaluation in epilepsy. fMRI is noninvasive and also considered safe. Neither of the two methods has been standardized. “Because fMRI is becoming more widely available, we wanted to see how it compares to the Wada test,” said lead author Jerzy Szaflarski, MD, PhD, Professor of Neurology at the University of Alabama at Birmingham. “While the risks associated with the Wada test are rare, they can be serious, including stroke and injury to the carotid artery.”
The AAN formed an 11-member panel to draft the guidelines. Two panelists independently selected 37 possibly relevant articles. Studies with fewer than 15 cases, case reports, meta-analyses, and editorials were excluded. Two panelists rated each article according to the AAN’s diagnostic and prognostic classification of evidence, and the panel ultimately developed consensus recommendations.
Data Support fMRI for Certain Situations
Class II data indicated that fMRI possibly provides language lateralization information concordant with that of IAP in 87% of people with medial temporal lobe epilepsy and in 81% of patients with extratemporal epilepsy. Current comparative data for temporal tumors or lateral temporal cases are insufficient to draw conclusions. Thus, fMRI may be considered as an option for lateralizing language functions in place of IAP in patients with medial temporal lobe epilepsy (Level C), temporal epilepsy in general (Level C), or extratemporal epilepsy (Level C), said the authors. The evidence is unclear for patients with temporal neocortical epilepsy or temporal tumors (Level U).
One Class II study and one Class III study suggested that fMRI is possibly effective in aiding the prediction of postsurgical language deficits in patients undergoing presurgical evaluation for possible temporal lobectomy. The authors recommended that fMRI may be considered for predicting postsurgical language outcomes after anterior temporal lobe resection for the control of temporal lobe epilepsy (Level C).
Class II evidence suggests that in patients with medial temporal lobe epilepsy, fMRI is comparable with IAP in its ability to lateralize memory functions and may be used for this purpose. The authors recommended that fMRI may be considered as an option to lateralize memory functions in place of IAP in patients with medial temporal lobe epilepsy (Level C).
Nine Class II studies with different methods together suggested that fMRI leftward activation asymmetry during encoding of verbal material, regardless of whether measured in the medial temporal lobe or in the language network, probably predicts verbal memory decline after left medial temporal lobe surgery. The authors therefore recommended that presurgical fMRI of verbal memory or of language encoding should be considered as an option to predict verbal memory outcome in patients with epilepsy who are undergoing evaluation for left medial temporal lobe surgery (Level B).
A Class II study indicated that fMRI activation asymmetry during nonverbal (ie, scene and face recognition) memory tasks possibly predicts nonverbal memory decline after medial temporal lobe surgery. The authors recommended that presurgical fMRI using nonverbal memory encoding may be considered as a means to predict visuospatial memory outcomes in patients with epilepsy who are undergoing evaluation for temporal lobe surgery (Level C).
Based on data from one Class II study and one Class III study, the authors recommended that presurgical fMRI may be used instead of the IAP for language lateralization in patients with epilepsy who are undergoing evaluation for brain surgery (Level C). “However, when fMRI is used for this purpose, task design, data analysis methods, and epilepsy type should be considered,” they added.
In addition, based on nine Class II studies, the authors recommended that fMRI of language and verbal memory lateralization may be an alternative to IAP memory testing for prediction of verbal memory outcome in medial temporal lobe epilepsy (Level C). The authors note that fMRI is not yet established as an alternative to the IAP for prediction of global amnesia in patients who have undergone anterior temporal lobe surgery.
More and Larger Studies Are Needed
“The imperfect concordance between fMRI and IAP language lateralization leaves open the question of which test is more accurate in discordant cases,” said the authors. Although the IAP is the reference standard, it is subject to limitations resulting from individual variation in arterial anatomy, variable effects of anesthesia, the rate of amobarbital injection, variability in patient cooperation, and variation in testing methods.
Like the IAP, cognitive fMRI “is a complex diagnostic procedure that requires both advanced technical expertise in imaging and expert interaction with patients to elicit adequate levels of task performance, select a set of activation tasks appropriate to the patient’s ability and the clinical aims of the study, instruct the patient on the tasks, administer the tasks during scanning, and evaluate and provide corrective feedback on task performance during the scanning session,” said the authors.
Global amnesia may result from bilateral medial temporal lobe damage, and some neurologists depend on the IAP to evaluate a patient’s risk for this outcome. “Global amnesia is rare after unilateral temporal lobe surgery, however, and occurs mainly when there is preexisting contralateral medial temporal lobe dysfunction,” said the authors. “One possible approach, therefore, is to reserve use of the IAP memory test for those patients at greatest risk for global amnesia, that is, patients undergoing unilateral anterior temporal lobe resection who have structural or functional evidence of damage to the contralateral medial temporal lobe.”
“Larger studies need to be conducted to increase the quality of available evidence,” Dr. Szaflarski concluded.
—Erik Greb
Suggested Reading
Szaflarski JP, Gloss D, Binder JR, et al. Practice guideline summary: Use of fMRI in the presurgical evaluation of patients with epilepsy—Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology. Neurology. 2017 Jan 11 [Epub ahead of print].
Common Epilepsies Share Genetic Overlap With Rare Types
Several genes previously implicated only in rare, severe forms of pediatric epilepsy may also contribute to common forms of the disorder, according to a report published online ahead of print January 13 in Lancet Neurology. “Our findings raise hopes that the emerging paradigm for the treatment of rare epilepsies, where therapies are targeted to the precise genetic cause of disease, may also extend to a proportion of common epilepsy syndromes,” said study leader David B. Goldstein, PhD, Director of the Institute for Genomic Medicine and Professor in the Departments of Genetics and Development and Neurology at Columbia University Medical Center in New York City. 
In recent years, researchers have identified dozens of genes that, alone or in combination with other factors, cause rare pediatric epilepsies. These discoveries have led to the use of targeted therapies for some seizure disorders, such as the ketogenic diet for patients with Dravet syndrome or GLUT-1 deficiency syndrome. Other therapies such as quinidine, a medication to treat heart arrhythmias, and memantine, an Alzheimer’s disease treatment, have been tried in children with certain gene mutations. These attempts have not proved universally effective for all patients with these mutations, but suggest the potential to repurpose existing medicines to treat rare genetic forms of epilepsy.
“Unlike very rare types of epilepsies, previous studies had shed little light on the genetic underpinnings of common epilepsies, which suggested that this precision medicine paradigm may have a very narrow application,” said Dr. Goldstein.
To learn more about the genetics of epilepsy, Dr. Goldstein and his colleagues conducted a study to identify the genetic contributions to more common forms of epilepsy. Analyses were conducted at Columbia University Medical Center’s Institute for Genomic Medicine, in collaboration with NewYork-Presbyterian, as part of Epi4K, an international consortium of epilepsy clinicians and researchers. Most of the patients were recruited through the Epilepsy Phenome/Genome Project.
The researchers separately compared the sequence data from 640 individuals with familial genetic generalized epilepsy and 525 individuals with familial non-acquired focal epilepsy to the same group of 3,877 controls. The researchers found significantly higher rates of ultra-rare deleterious variation in genes established as causative for dominant epilepsy disorders (familial genetic generalized epilepsy, odd ratio 2.3; familial non-acquired focal epilepsy, odds ratio 3.6). Comparison of an additional cohort of 662 individuals with sporadic non-acquired focal epilepsy to controls did not identify study-wide significant signals.
Five Genes Implicated
For the individuals with familial non-acquired focal epilepsy, the researchers found that five known epilepsy genes—DEPDC5, LG11, PCDH19, SCN1A, and GRIN2A—ranked as the top five genes enriched for ultra-rare deleterious variation. “After accounting for the control carrier rate, we estimate that these five genes contribute to the risk of epilepsy in approximately 8% of individuals with familial non-acquired focal epilepsy,” said Erin Heinzen Cox, PhD, Assistant Professor in the Department of Pathology and Cell Biology and Deputy Director of the Institute for Genomic Medicine at Columbia University Medical Center. 
Treatment Targeted to Epilepsy Subtype
The findings have important implications for clinical practice and for research. “At present, all common epilepsies are treated the same way, with the same group of medications,” said Dr. Goldstein. “But as we identify more of these epilepsy genes that span a much wider range of types of epilepsy than previously thought, we can begin to try targeted therapies across these patient populations. As this genetically driven treatment paradigm becomes more established, our field, which is accustomed to undertaking large clinical trials in broad patient populations, will need to take a new approach to clinical research, focusing on patients based on their genetic subtype.”
“This is a very exciting breakthrough in the treatment of epilepsy, in which current treatment is based on whether a child has focal seizures … or generalized seizures,” said James J. Riviello, MD, the Sergievsky Family Professor of Neurology and Pediatrics and Chief of Child Neurology at NewYork-Presbyterian Morgan Stanley Children’s Hospital in New York City. “Genetic testing for epilepsy may allow us to identify the specific anticonvulsant medication that potentially works best for an individual patient. We have already identified children in whom knowing the underlying genetic basis of the epilepsy has guided our treatment choices.” 
Additional studies, which will analyze 10,000 to 12,000 samples, are planned for the coming year. “With a larger analysis, we expect to find additional genetic variations that contribute to common epilepsies,” said Dr. Goldstein.
Suggested Reading
Epi4K consortium, Epilepsy Phenome/Genome Project. Ultra-rare genetic variation in common epilepsies: a case-control sequencing study. Lancet Neurol. 2017;16(2):135-143.
Several genes previously implicated only in rare, severe forms of pediatric epilepsy may also contribute to common forms of the disorder, according to a report published online ahead of print January 13 in Lancet Neurology. “Our findings raise hopes that the emerging paradigm for the treatment of rare epilepsies, where therapies are targeted to the precise genetic cause of disease, may also extend to a proportion of common epilepsy syndromes,” said study leader David B. Goldstein, PhD, Director of the Institute for Genomic Medicine and Professor in the Departments of Genetics and Development and Neurology at Columbia University Medical Center in New York City.
In recent years, researchers have identified dozens of genes that, alone or in combination with other factors, cause rare pediatric epilepsies. These discoveries have led to the use of targeted therapies for some seizure disorders, such as the ketogenic diet for patients with Dravet syndrome or GLUT-1 deficiency syndrome. Other therapies such as quinidine, a medication to treat heart arrhythmias, and memantine, an Alzheimer’s disease treatment, have been tried in children with certain gene mutations. These attempts have not proved universally effective for all patients with these mutations, but suggest the potential to repurpose existing medicines to treat rare genetic forms of epilepsy.
“Unlike very rare types of epilepsies, previous studies had shed little light on the genetic underpinnings of common epilepsies, which suggested that this precision medicine paradigm may have a very narrow application,” said Dr. Goldstein.
To learn more about the genetics of epilepsy, Dr. Goldstein and his colleagues conducted a study to identify the genetic contributions to more common forms of epilepsy. Analyses were conducted at Columbia University Medical Center’s Institute for Genomic Medicine, in collaboration with NewYork-Presbyterian, as part of Epi4K, an international consortium of epilepsy clinicians and researchers. Most of the patients were recruited through the Epilepsy Phenome/Genome Project.
The researchers separately compared the sequence data from 640 individuals with familial genetic generalized epilepsy and 525 individuals with familial non-acquired focal epilepsy to the same group of 3,877 controls. The researchers found significantly higher rates of ultra-rare deleterious variation in genes established as causative for dominant epilepsy disorders (familial genetic generalized epilepsy, odd ratio 2.3; familial non-acquired focal epilepsy, odds ratio 3.6). Comparison of an additional cohort of 662 individuals with sporadic non-acquired focal epilepsy to controls did not identify study-wide significant signals.
Five Genes Implicated
For the individuals with familial non-acquired focal epilepsy, the researchers found that five known epilepsy genes—DEPDC5, LG11, PCDH19, SCN1A, and GRIN2A—ranked as the top five genes enriched for ultra-rare deleterious variation. “After accounting for the control carrier rate, we estimate that these five genes contribute to the risk of epilepsy in approximately 8% of individuals with familial non-acquired focal epilepsy,” said Erin Heinzen Cox, PhD, Assistant Professor in the Department of Pathology and Cell Biology and Deputy Director of the Institute for Genomic Medicine at Columbia University Medical Center.
Treatment Targeted to Epilepsy Subtype
The findings have important implications for clinical practice and for research. “At present, all common epilepsies are treated the same way, with the same group of medications,” said Dr. Goldstein. “But as we identify more of these epilepsy genes that span a much wider range of types of epilepsy than previously thought, we can begin to try targeted therapies across these patient populations. As this genetically driven treatment paradigm becomes more established, our field, which is accustomed to undertaking large clinical trials in broad patient populations, will need to take a new approach to clinical research, focusing on patients based on their genetic subtype.”
“This is a very exciting breakthrough in the treatment of epilepsy, in which current treatment is based on whether a child has focal seizures … or generalized seizures,” said James J. Riviello, MD, the Sergievsky Family Professor of Neurology and Pediatrics and Chief of Child Neurology at NewYork-Presbyterian Morgan Stanley Children’s Hospital in New York City. “Genetic testing for epilepsy may allow us to identify the specific anticonvulsant medication that potentially works best for an individual patient. We have already identified children in whom knowing the underlying genetic basis of the epilepsy has guided our treatment choices.”
Additional studies, which will analyze 10,000 to 12,000 samples, are planned for the coming year. “With a larger analysis, we expect to find additional genetic variations that contribute to common epilepsies,” said Dr. Goldstein.
Suggested Reading
Epi4K consortium, Epilepsy Phenome/Genome Project. Ultra-rare genetic variation in common epilepsies: a case-control sequencing study. Lancet Neurol. 2017;16(2):135-143.
Several genes previously implicated only in rare, severe forms of pediatric epilepsy may also contribute to common forms of the disorder, according to a report published online ahead of print January 13 in Lancet Neurology. “Our findings raise hopes that the emerging paradigm for the treatment of rare epilepsies, where therapies are targeted to the precise genetic cause of disease, may also extend to a proportion of common epilepsy syndromes,” said study leader David B. Goldstein, PhD, Director of the Institute for Genomic Medicine and Professor in the Departments of Genetics and Development and Neurology at Columbia University Medical Center in New York City.
In recent years, researchers have identified dozens of genes that, alone or in combination with other factors, cause rare pediatric epilepsies. These discoveries have led to the use of targeted therapies for some seizure disorders, such as the ketogenic diet for patients with Dravet syndrome or GLUT-1 deficiency syndrome. Other therapies such as quinidine, a medication to treat heart arrhythmias, and memantine, an Alzheimer’s disease treatment, have been tried in children with certain gene mutations. These attempts have not proved universally effective for all patients with these mutations, but suggest the potential to repurpose existing medicines to treat rare genetic forms of epilepsy.
“Unlike very rare types of epilepsies, previous studies had shed little light on the genetic underpinnings of common epilepsies, which suggested that this precision medicine paradigm may have a very narrow application,” said Dr. Goldstein.
To learn more about the genetics of epilepsy, Dr. Goldstein and his colleagues conducted a study to identify the genetic contributions to more common forms of epilepsy. Analyses were conducted at Columbia University Medical Center’s Institute for Genomic Medicine, in collaboration with NewYork-Presbyterian, as part of Epi4K, an international consortium of epilepsy clinicians and researchers. Most of the patients were recruited through the Epilepsy Phenome/Genome Project.
The researchers separately compared the sequence data from 640 individuals with familial genetic generalized epilepsy and 525 individuals with familial non-acquired focal epilepsy to the same group of 3,877 controls. The researchers found significantly higher rates of ultra-rare deleterious variation in genes established as causative for dominant epilepsy disorders (familial genetic generalized epilepsy, odd ratio 2.3; familial non-acquired focal epilepsy, odds ratio 3.6). Comparison of an additional cohort of 662 individuals with sporadic non-acquired focal epilepsy to controls did not identify study-wide significant signals.
Five Genes Implicated
For the individuals with familial non-acquired focal epilepsy, the researchers found that five known epilepsy genes—DEPDC5, LG11, PCDH19, SCN1A, and GRIN2A—ranked as the top five genes enriched for ultra-rare deleterious variation. “After accounting for the control carrier rate, we estimate that these five genes contribute to the risk of epilepsy in approximately 8% of individuals with familial non-acquired focal epilepsy,” said Erin Heinzen Cox, PhD, Assistant Professor in the Department of Pathology and Cell Biology and Deputy Director of the Institute for Genomic Medicine at Columbia University Medical Center.
Treatment Targeted to Epilepsy Subtype
The findings have important implications for clinical practice and for research. “At present, all common epilepsies are treated the same way, with the same group of medications,” said Dr. Goldstein. “But as we identify more of these epilepsy genes that span a much wider range of types of epilepsy than previously thought, we can begin to try targeted therapies across these patient populations. As this genetically driven treatment paradigm becomes more established, our field, which is accustomed to undertaking large clinical trials in broad patient populations, will need to take a new approach to clinical research, focusing on patients based on their genetic subtype.”
“This is a very exciting breakthrough in the treatment of epilepsy, in which current treatment is based on whether a child has focal seizures … or generalized seizures,” said James J. Riviello, MD, the Sergievsky Family Professor of Neurology and Pediatrics and Chief of Child Neurology at NewYork-Presbyterian Morgan Stanley Children’s Hospital in New York City. “Genetic testing for epilepsy may allow us to identify the specific anticonvulsant medication that potentially works best for an individual patient. We have already identified children in whom knowing the underlying genetic basis of the epilepsy has guided our treatment choices.”
Additional studies, which will analyze 10,000 to 12,000 samples, are planned for the coming year. “With a larger analysis, we expect to find additional genetic variations that contribute to common epilepsies,” said Dr. Goldstein.
Suggested Reading
Epi4K consortium, Epilepsy Phenome/Genome Project. Ultra-rare genetic variation in common epilepsies: a case-control sequencing study. Lancet Neurol. 2017;16(2):135-143.
Vagus Nerve Stimulation Reduces Seizures and Improves Quality of Life
Patients with epilepsy who do not respond to drug therapy and who are not candidates for surgery sometimes respond to vagus nerve stimulation (VNS). Research suggests that VNS reduces the frequency of seizures, but a recent study now suggests it may also improve patients’ quality of life. Englot et al found that the therapy improves alertness, post-ictal state, cluster seizures, mood, verbal communication, school and professional achievements, and memory. Several factors predicted patients’ improvement, including a shorter time to implant, female gender, generalized seizure type, and being Caucasian.
Englot DJ, Hassnain KH, Rolston JD, et al. Quality-of-life metrics with vagus nerve stimulation for epilepsy from provider survey data. Epilepsy Behav. 2017;66:4-9.
Patients with epilepsy who do not respond to drug therapy and who are not candidates for surgery sometimes respond to vagus nerve stimulation (VNS). Research suggests that VNS reduces the frequency of seizures, but a recent study now suggests it may also improve patients’ quality of life. Englot et al found that the therapy improves alertness, post-ictal state, cluster seizures, mood, verbal communication, school and professional achievements, and memory. Several factors predicted patients’ improvement, including a shorter time to implant, female gender, generalized seizure type, and being Caucasian.
Englot DJ, Hassnain KH, Rolston JD, et al. Quality-of-life metrics with vagus nerve stimulation for epilepsy from provider survey data. Epilepsy Behav. 2017;66:4-9.
Patients with epilepsy who do not respond to drug therapy and who are not candidates for surgery sometimes respond to vagus nerve stimulation (VNS). Research suggests that VNS reduces the frequency of seizures, but a recent study now suggests it may also improve patients’ quality of life. Englot et al found that the therapy improves alertness, post-ictal state, cluster seizures, mood, verbal communication, school and professional achievements, and memory. Several factors predicted patients’ improvement, including a shorter time to implant, female gender, generalized seizure type, and being Caucasian.
Englot DJ, Hassnain KH, Rolston JD, et al. Quality-of-life metrics with vagus nerve stimulation for epilepsy from provider survey data. Epilepsy Behav. 2017;66:4-9.
Auditory Auras May Signal Poor Surgical Outcomes
Patients with temporal lobe epilepsy are more likely to fare poorly after surgery if they experience auditory auras. When Ali A. Asadi-Pooya and associates performed a retrospective analysis of 1186 drug-resistant patients who had had a temporal resection, they found that those with auditory auras were more likely to relapse after surgery, when compared to those who did not experience the auras (P=.03). The side of the head in which the procedure was performed did not affect postoperative prognosis.
Asadi-Pooya AA, Wyeth D, Nei M, et al. Postsurgical outcome in patients with auditory auras and drug-resistant epilepsy. Epilepsy Behav. 2017;66:49-52.
Patients with temporal lobe epilepsy are more likely to fare poorly after surgery if they experience auditory auras. When Ali A. Asadi-Pooya and associates performed a retrospective analysis of 1186 drug-resistant patients who had had a temporal resection, they found that those with auditory auras were more likely to relapse after surgery, when compared to those who did not experience the auras (P=.03). The side of the head in which the procedure was performed did not affect postoperative prognosis.
Asadi-Pooya AA, Wyeth D, Nei M, et al. Postsurgical outcome in patients with auditory auras and drug-resistant epilepsy. Epilepsy Behav. 2017;66:49-52.
Patients with temporal lobe epilepsy are more likely to fare poorly after surgery if they experience auditory auras. When Ali A. Asadi-Pooya and associates performed a retrospective analysis of 1186 drug-resistant patients who had had a temporal resection, they found that those with auditory auras were more likely to relapse after surgery, when compared to those who did not experience the auras (P=.03). The side of the head in which the procedure was performed did not affect postoperative prognosis.
Asadi-Pooya AA, Wyeth D, Nei M, et al. Postsurgical outcome in patients with auditory auras and drug-resistant epilepsy. Epilepsy Behav. 2017;66:49-52.
Older, Poorer Patients With Epilepsy Less Likely to Take their Medication
Approximately one-third of older adults with epilepsy do not adhere very well to their antiepileptic drug regimen, with older minority patients even less compliant. That’s the conclusion reached by researchers who analyzed Medicare claims from 2008 to 2010, using a 5% random sample of beneficiaries and augmenting it with minority patients. Piper et al looked at 36,912 cases of epilepsy and found 31.8% were nonadherent; that included 24.1% of whites and 34.3% of African Americans. They also found that Medicare beneficiaries who lived in high poverty areas were more likely to be noncompliant.
Piper K, Richman J, Faught E, at al. Adherence to antiepileptic drugs among diverse older Americans on Part D Medicare. Epilepsy Behav. 2017;66:68-73.
Approximately one-third of older adults with epilepsy do not adhere very well to their antiepileptic drug regimen, with older minority patients even less compliant. That’s the conclusion reached by researchers who analyzed Medicare claims from 2008 to 2010, using a 5% random sample of beneficiaries and augmenting it with minority patients. Piper et al looked at 36,912 cases of epilepsy and found 31.8% were nonadherent; that included 24.1% of whites and 34.3% of African Americans. They also found that Medicare beneficiaries who lived in high poverty areas were more likely to be noncompliant.
Piper K, Richman J, Faught E, at al. Adherence to antiepileptic drugs among diverse older Americans on Part D Medicare. Epilepsy Behav. 2017;66:68-73.
Approximately one-third of older adults with epilepsy do not adhere very well to their antiepileptic drug regimen, with older minority patients even less compliant. That’s the conclusion reached by researchers who analyzed Medicare claims from 2008 to 2010, using a 5% random sample of beneficiaries and augmenting it with minority patients. Piper et al looked at 36,912 cases of epilepsy and found 31.8% were nonadherent; that included 24.1% of whites and 34.3% of African Americans. They also found that Medicare beneficiaries who lived in high poverty areas were more likely to be noncompliant.
Piper K, Richman J, Faught E, at al. Adherence to antiepileptic drugs among diverse older Americans on Part D Medicare. Epilepsy Behav. 2017;66:68-73.