Literature Monitor

Pediatric refractory status epilepticus (RSE) and super refractory status epilepticus (SRSE) remain life-threatening disorders whose etiology and effective management are elusive according to a review in Seizure.

• RSE is defined as a disorder that doesn’t respond to first and second line antiepileptic agents.
• SRSE refers to status epilepticus that continues for at least 24 hours after anesthetic agents have been given or which recurs once the drugs are stopped.
• The evidence to support current treatment options is not based on randomized clinical trials but instead relies on case series and expert opinions.
• The most common treatment for both disorders is continuous IV infusion of anesthetic drugs but the best dosing and the optimal administration rate remain controversial.
• Some clinicians have used non-drug approaches to RSE and SRSE but the evidence supporting these options is limited.

Vasquez A, Farias-Moeller R, Tatum W. Pediatric refractory and super refractory status epilepticus [Published online ahead of print May 19, 2018]. Seizure. DOI: https://doi.org/10.1016/j.seizure.2018.05.012.

Pediatric refractory status epilepticus (RSE) and super refractory status epilepticus (SRSE) remain life-threatening disorders whose etiology and effective management are elusive according to a review in Seizure.

• RSE is defined as a disorder that doesn’t respond to first and second line antiepileptic agents.
• SRSE refers to status epilepticus that continues for at least 24 hours after anesthetic agents have been given or which recurs once the drugs are stopped.
• The evidence to support current treatment options is not based on randomized clinical trials but instead relies on case series and expert opinions.
• The most common treatment for both disorders is continuous IV infusion of anesthetic drugs but the best dosing and the optimal administration rate remain controversial.
• Some clinicians have used non-drug approaches to RSE and SRSE but the evidence supporting these options is limited.

Vasquez A, Farias-Moeller R, Tatum W. Pediatric refractory and super refractory status epilepticus [Published online ahead of print May 19, 2018]. Seizure. DOI: https://doi.org/10.1016/j.seizure.2018.05.012.

Pediatric refractory status epilepticus (RSE) and super refractory status epilepticus (SRSE) remain life-threatening disorders whose etiology and effective management are elusive according to a review in Seizure.

• RSE is defined as a disorder that doesn’t respond to first and second line antiepileptic agents.
• SRSE refers to status epilepticus that continues for at least 24 hours after anesthetic agents have been given or which recurs once the drugs are stopped.
• The evidence to support current treatment options is not based on randomized clinical trials but instead relies on case series and expert opinions.
• The most common treatment for both disorders is continuous IV infusion of anesthetic drugs but the best dosing and the optimal administration rate remain controversial.
• Some clinicians have used non-drug approaches to RSE and SRSE but the evidence supporting these options is limited.

Vasquez A, Farias-Moeller R, Tatum W. Pediatric refractory and super refractory status epilepticus [Published online ahead of print May 19, 2018]. Seizure. DOI: https://doi.org/10.1016/j.seizure.2018.05.012.

Patients with epilepsy clusters can benefit from rescue medications, but these drugs are underutilized according to a recent review of the medical literature.

• Saba Jafarpour from Boston Children’s Hospital, and associates found that the definition of epilepsy clusters differs among experts, including 3 or more seizures in 24 hours, 2 or more in 24 hours, and 2 or more in 6 hours.
• Most of the research analyzed by the investigators was conducted at tertiary centers on patients with refractory epilepsy.
• Although rectal diazepam gel is the only medication approved by the FDA as a rescue drug for seizure clusters, the researchers found that non-rectal oral benzodiazepines are effective and safe for outpatient treatment of these clusters.
• Acute treatment of seizure clusters can help prevent status epilepticus and reduce emergency department visits.

Jafarpour S, Hirsch  LJ, Gaínza-Lein M, Kellinghaus C, Detyniecki K. Seizure cluster: definition, prevalence, consequences, and management [Published online ahead of print May 21,2018]. Seizure. DOI: https://doi.org/10.1016/j.seizure.2018.05.013.

Patients with epilepsy clusters can benefit from rescue medications, but these drugs are underutilized according to a recent review of the medical literature.

• Saba Jafarpour from Boston Children’s Hospital, and associates found that the definition of epilepsy clusters differs among experts, including 3 or more seizures in 24 hours, 2 or more in 24 hours, and 2 or more in 6 hours.
• Most of the research analyzed by the investigators was conducted at tertiary centers on patients with refractory epilepsy.
• Although rectal diazepam gel is the only medication approved by the FDA as a rescue drug for seizure clusters, the researchers found that non-rectal oral benzodiazepines are effective and safe for outpatient treatment of these clusters.
• Acute treatment of seizure clusters can help prevent status epilepticus and reduce emergency department visits.

Jafarpour S, Hirsch  LJ, Gaínza-Lein M, Kellinghaus C, Detyniecki K. Seizure cluster: definition, prevalence, consequences, and management [Published online ahead of print May 21,2018]. Seizure. DOI: https://doi.org/10.1016/j.seizure.2018.05.013.

Patients with epilepsy clusters can benefit from rescue medications, but these drugs are underutilized according to a recent review of the medical literature.

• Saba Jafarpour from Boston Children’s Hospital, and associates found that the definition of epilepsy clusters differs among experts, including 3 or more seizures in 24 hours, 2 or more in 24 hours, and 2 or more in 6 hours.
• Most of the research analyzed by the investigators was conducted at tertiary centers on patients with refractory epilepsy.
• Although rectal diazepam gel is the only medication approved by the FDA as a rescue drug for seizure clusters, the researchers found that non-rectal oral benzodiazepines are effective and safe for outpatient treatment of these clusters.
• Acute treatment of seizure clusters can help prevent status epilepticus and reduce emergency department visits.

Jafarpour S, Hirsch  LJ, Gaínza-Lein M, Kellinghaus C, Detyniecki K. Seizure cluster: definition, prevalence, consequences, and management [Published online ahead of print May 21,2018]. Seizure. DOI: https://doi.org/10.1016/j.seizure.2018.05.013.

Children with drug-resistant epilepsy seem to have a lobar pattern of glucose hypometabolism according to PET scans performed on 41 patients with the disorder.

• Baseline positron emission tomography (PET) scans were performed on children with drug resistant epilepsy with a median age of 4.7 years.
• A follow-up PET scan was done 4.3 years (median) later.
• Children with space-occupying lesion-related epilepsy were not included in the study, nor were patients with progressive neurological disorders.
• Twenty-four children had unilateral glucose hypometabolism and 13 had bilateral hypometabolism at baseline.
• Follow-up PET scans revealed that 63% had interval expansion of the hypometabolic region of their brain; said expansion was associated with persistent seizures.

Govil-Dalela T, Kumar A Behen ME, Chugani HT, Juhász C. Evolution of lobar abnormalities of cerebral glucose metabolism in 41 children with drug-resistant epilepsy [Published online ahead of print May 22, 2018]. Epilepsia. doi: 10.1111/epi.14404.

Children with drug-resistant epilepsy seem to have a lobar pattern of glucose hypometabolism according to PET scans performed on 41 patients with the disorder.

• Baseline positron emission tomography (PET) scans were performed on children with drug resistant epilepsy with a median age of 4.7 years.
• A follow-up PET scan was done 4.3 years (median) later.
• Children with space-occupying lesion-related epilepsy were not included in the study, nor were patients with progressive neurological disorders.
• Twenty-four children had unilateral glucose hypometabolism and 13 had bilateral hypometabolism at baseline.
• Follow-up PET scans revealed that 63% had interval expansion of the hypometabolic region of their brain; said expansion was associated with persistent seizures.

Govil-Dalela T, Kumar A Behen ME, Chugani HT, Juhász C. Evolution of lobar abnormalities of cerebral glucose metabolism in 41 children with drug-resistant epilepsy [Published online ahead of print May 22, 2018]. Epilepsia. doi: 10.1111/epi.14404.

Children with drug-resistant epilepsy seem to have a lobar pattern of glucose hypometabolism according to PET scans performed on 41 patients with the disorder.

• Baseline positron emission tomography (PET) scans were performed on children with drug resistant epilepsy with a median age of 4.7 years.
• A follow-up PET scan was done 4.3 years (median) later.
• Children with space-occupying lesion-related epilepsy were not included in the study, nor were patients with progressive neurological disorders.
• Twenty-four children had unilateral glucose hypometabolism and 13 had bilateral hypometabolism at baseline.
• Follow-up PET scans revealed that 63% had interval expansion of the hypometabolic region of their brain; said expansion was associated with persistent seizures.

Govil-Dalela T, Kumar A Behen ME, Chugani HT, Juhász C. Evolution of lobar abnormalities of cerebral glucose metabolism in 41 children with drug-resistant epilepsy [Published online ahead of print May 22, 2018]. Epilepsia. doi: 10.1111/epi.14404.

More attention needs to be paid to evaluating quality of life and cognitive/behavioral outcomes in children with status epilepticus, according to a review of the relevant medical literature.

• An analysis of MEDLINE derived studies looked at the short-term and long-term outcomes in pediatric patients with status epilepticus.
• The review found that mortality in this patient population was relatively low.
• Health-related quality of life, neuroimaging, the use of continuous infusions, and cognitive/behavioral outcomes were not adequately addressed in the studies that were evaluated.
• The researchers point out, however, that the full effects of status epilepticus on the outcomes being evaluated were difficult to accurately establish because there were so many differences in the way the studies were conducted.

Jafarpour S, Stredny CM, Piantino J, Chapman KE. Baseline and outcome assessment in pediatric status epilepticus. [Published online ahead of print April 26, 2018]. Seizure. DOI: https://doi.org/10.1016/j.seizure.2018.04.019

More attention needs to be paid to evaluating quality of life and cognitive/behavioral outcomes in children with status epilepticus, according to a review of the relevant medical literature.

• An analysis of MEDLINE derived studies looked at the short-term and long-term outcomes in pediatric patients with status epilepticus.
• The review found that mortality in this patient population was relatively low.
• Health-related quality of life, neuroimaging, the use of continuous infusions, and cognitive/behavioral outcomes were not adequately addressed in the studies that were evaluated.
• The researchers point out, however, that the full effects of status epilepticus on the outcomes being evaluated were difficult to accurately establish because there were so many differences in the way the studies were conducted.

Jafarpour S, Stredny CM, Piantino J, Chapman KE. Baseline and outcome assessment in pediatric status epilepticus. [Published online ahead of print April 26, 2018]. Seizure. DOI: https://doi.org/10.1016/j.seizure.2018.04.019

More attention needs to be paid to evaluating quality of life and cognitive/behavioral outcomes in children with status epilepticus, according to a review of the relevant medical literature.

• An analysis of MEDLINE derived studies looked at the short-term and long-term outcomes in pediatric patients with status epilepticus.
• The review found that mortality in this patient population was relatively low.
• Health-related quality of life, neuroimaging, the use of continuous infusions, and cognitive/behavioral outcomes were not adequately addressed in the studies that were evaluated.
• The researchers point out, however, that the full effects of status epilepticus on the outcomes being evaluated were difficult to accurately establish because there were so many differences in the way the studies were conducted.

Jafarpour S, Stredny CM, Piantino J, Chapman KE. Baseline and outcome assessment in pediatric status epilepticus. [Published online ahead of print April 26, 2018]. Seizure. DOI: https://doi.org/10.1016/j.seizure.2018.04.019

A cross-sectional analysis of epilepsy intervention trials suggests there may be bias in the reporting of these investigations according to a recent review of the research.

• Investigators analyzed 126 epilepsy intervention trials, comparing two reporting periods: 2008 to 2011 and 2012 to 2015.
• Twenty five percent of the trials were not reported (31/126).
• 72 of the 126 trials were conducted in at least one US center.
• 56 of 72 trials (78%) met the US Food and Drug Administration’s Amendments Act requirements.
• Researchers found that the time it took to report trial results had become shorter over time, when comparing 2008-2011 to 2012-2015.
• However, only a third of the trials (19/56) reported their results within the FDA’s mandated one-year time frame. 

Rayi A, Thompson S, Gloss D, Malhotra K. Reporting bias in completed epilepsy intervention trials: a cross-sectional analysis. Epilepsy Res. 2018;143:1-6

A cross-sectional analysis of epilepsy intervention trials suggests there may be bias in the reporting of these investigations according to a recent review of the research.

• Investigators analyzed 126 epilepsy intervention trials, comparing two reporting periods: 2008 to 2011 and 2012 to 2015.
• Twenty five percent of the trials were not reported (31/126).
• 72 of the 126 trials were conducted in at least one US center.
• 56 of 72 trials (78%) met the US Food and Drug Administration’s Amendments Act requirements.
• Researchers found that the time it took to report trial results had become shorter over time, when comparing 2008-2011 to 2012-2015.
• However, only a third of the trials (19/56) reported their results within the FDA’s mandated one-year time frame. 

Rayi A, Thompson S, Gloss D, Malhotra K. Reporting bias in completed epilepsy intervention trials: a cross-sectional analysis. Epilepsy Res. 2018;143:1-6

A cross-sectional analysis of epilepsy intervention trials suggests there may be bias in the reporting of these investigations according to a recent review of the research.

• Investigators analyzed 126 epilepsy intervention trials, comparing two reporting periods: 2008 to 2011 and 2012 to 2015.
• Twenty five percent of the trials were not reported (31/126).
• 72 of the 126 trials were conducted in at least one US center.
• 56 of 72 trials (78%) met the US Food and Drug Administration’s Amendments Act requirements.
• Researchers found that the time it took to report trial results had become shorter over time, when comparing 2008-2011 to 2012-2015.
• However, only a third of the trials (19/56) reported their results within the FDA’s mandated one-year time frame. 

Rayi A, Thompson S, Gloss D, Malhotra K. Reporting bias in completed epilepsy intervention trials: a cross-sectional analysis. Epilepsy Res. 2018;143:1-6

Performing continuous EEG (cEEG) monitoring in patients who have experienced a subarachnoid hemorrhage can help predict the occurrence of delayed cerebral ischemia (DCI) according to a prospective study of 103 patients who underwent cEEG.

• DCI is a common complication of subarachnoid hemorrhage.
• Retrospective studies have suggested a link between cEEG and DCI but the association needed to be confirmed with a prospective evaluation.
• Continuous EEG monitoring involved an average of 7.7 days duration, and a EEG alarm occurred in about 96% of patients with subsequent DCI but in only 19.6% of patients without the ischemic complication.
• Among patients who had a EEG alarm, late onset epileptiform abnormalities were most likely to predict DCI.

Rosenthal ES, Biswal S, Zafar SF, et al. Continuous electroencephalography predicts delayed cerebral ischemia after subarachnoid hemorrhage: a prospective study of diagnostic accuracy [published online ahead of print Apr 16, 2018]. Ann Neurol. doi: 10.1002/ana.25232

Performing continuous EEG (cEEG) monitoring in patients who have experienced a subarachnoid hemorrhage can help predict the occurrence of delayed cerebral ischemia (DCI) according to a prospective study of 103 patients who underwent cEEG.

• DCI is a common complication of subarachnoid hemorrhage.
• Retrospective studies have suggested a link between cEEG and DCI but the association needed to be confirmed with a prospective evaluation.
• Continuous EEG monitoring involved an average of 7.7 days duration, and a EEG alarm occurred in about 96% of patients with subsequent DCI but in only 19.6% of patients without the ischemic complication.
• Among patients who had a EEG alarm, late onset epileptiform abnormalities were most likely to predict DCI.

Rosenthal ES, Biswal S, Zafar SF, et al. Continuous electroencephalography predicts delayed cerebral ischemia after subarachnoid hemorrhage: a prospective study of diagnostic accuracy [published online ahead of print Apr 16, 2018]. Ann Neurol. doi: 10.1002/ana.25232

Performing continuous EEG (cEEG) monitoring in patients who have experienced a subarachnoid hemorrhage can help predict the occurrence of delayed cerebral ischemia (DCI) according to a prospective study of 103 patients who underwent cEEG.

• DCI is a common complication of subarachnoid hemorrhage.
• Retrospective studies have suggested a link between cEEG and DCI but the association needed to be confirmed with a prospective evaluation.
• Continuous EEG monitoring involved an average of 7.7 days duration, and a EEG alarm occurred in about 96% of patients with subsequent DCI but in only 19.6% of patients without the ischemic complication.
• Among patients who had a EEG alarm, late onset epileptiform abnormalities were most likely to predict DCI.

Rosenthal ES, Biswal S, Zafar SF, et al. Continuous electroencephalography predicts delayed cerebral ischemia after subarachnoid hemorrhage: a prospective study of diagnostic accuracy [published online ahead of print Apr 16, 2018]. Ann Neurol. doi: 10.1002/ana.25232

The commonly accepted cutoff scores for reliable digit span (RDS), a way to measure attention and working memory and assess suboptimal effort, are not appropriate for adults with epilepsy, according to a study of 63 patients with epilepsy or suspected seizures.

• A cutoff of ≤6 or ≤7 for RDS, which is part of the Wechsler Adult Intelligence Scale, is typically used in adult clinical populations.
• Maiman et al applied these thresholds to adult patients with epilepsy or suspected seizures, most of whom passed trial 2 of the Test for Memory Malingering with a score of 45 or above.
• A cutoff of 6 or less on the RDS subtest yielded a specificity of 85% while 7 or less yielded 77%.
• A secondary analysis concluded that a cutoff of 4 or less may be more appropriate for adults with epilepsy who have a low average IQ or lower.

Maiman M, Del Bene VA, MacAllister WS, et al. Reliable digit span: does it adequately measure suboptimal effort in an adult epilepsy population [published online ahead of print April 5, 2018]? Arch Clin Neuropsychol. doi: 10.1093/arclin/acy027

The commonly accepted cutoff scores for reliable digit span (RDS), a way to measure attention and working memory and assess suboptimal effort, are not appropriate for adults with epilepsy, according to a study of 63 patients with epilepsy or suspected seizures.

• A cutoff of ≤6 or ≤7 for RDS, which is part of the Wechsler Adult Intelligence Scale, is typically used in adult clinical populations.
• Maiman et al applied these thresholds to adult patients with epilepsy or suspected seizures, most of whom passed trial 2 of the Test for Memory Malingering with a score of 45 or above.
• A cutoff of 6 or less on the RDS subtest yielded a specificity of 85% while 7 or less yielded 77%.
• A secondary analysis concluded that a cutoff of 4 or less may be more appropriate for adults with epilepsy who have a low average IQ or lower.

Maiman M, Del Bene VA, MacAllister WS, et al. Reliable digit span: does it adequately measure suboptimal effort in an adult epilepsy population [published online ahead of print April 5, 2018]? Arch Clin Neuropsychol. doi: 10.1093/arclin/acy027

The commonly accepted cutoff scores for reliable digit span (RDS), a way to measure attention and working memory and assess suboptimal effort, are not appropriate for adults with epilepsy, according to a study of 63 patients with epilepsy or suspected seizures.

• A cutoff of ≤6 or ≤7 for RDS, which is part of the Wechsler Adult Intelligence Scale, is typically used in adult clinical populations.
• Maiman et al applied these thresholds to adult patients with epilepsy or suspected seizures, most of whom passed trial 2 of the Test for Memory Malingering with a score of 45 or above.
• A cutoff of 6 or less on the RDS subtest yielded a specificity of 85% while 7 or less yielded 77%.
• A secondary analysis concluded that a cutoff of 4 or less may be more appropriate for adults with epilepsy who have a low average IQ or lower.

Maiman M, Del Bene VA, MacAllister WS, et al. Reliable digit span: does it adequately measure suboptimal effort in an adult epilepsy population [published online ahead of print April 5, 2018]? Arch Clin Neuropsychol. doi: 10.1093/arclin/acy027

Performing epilepsy surgery on underserved Hispanic patients with intractable epilepsy relieves their depression and anxiety and improves their quality of life, according to study of 47 patients between 2008 and 2014.

• Hispanic patients, most of whom were immigrant and Spanish speaking, were treated at a comprehensive epilepsy center in an urban public health setting.
• They underwent presurgical and postsurgical neuropsychological evaluation and were identified retrospectively.
• Medium to large improvements on the Beck Depression Inventory and most quality of care scales were observed postsurgery.
• Less robust effects were also seen on the Beck Anxiety Inventory.

Smith JA, Armacost M, Ensign E, et al. Epilepsy surgery in the underserved Hispanic population improves depression, anxiety, and quality of life. Epilepsy Behav. 2018;83:1-6. https://doi.org/10.1016/j.yebeh.2018.03.015

Performing epilepsy surgery on underserved Hispanic patients with intractable epilepsy relieves their depression and anxiety and improves their quality of life, according to study of 47 patients between 2008 and 2014.

• Hispanic patients, most of whom were immigrant and Spanish speaking, were treated at a comprehensive epilepsy center in an urban public health setting.
• They underwent presurgical and postsurgical neuropsychological evaluation and were identified retrospectively.
• Medium to large improvements on the Beck Depression Inventory and most quality of care scales were observed postsurgery.
• Less robust effects were also seen on the Beck Anxiety Inventory.

Smith JA, Armacost M, Ensign E, et al. Epilepsy surgery in the underserved Hispanic population improves depression, anxiety, and quality of life. Epilepsy Behav. 2018;83:1-6. https://doi.org/10.1016/j.yebeh.2018.03.015

Performing epilepsy surgery on underserved Hispanic patients with intractable epilepsy relieves their depression and anxiety and improves their quality of life, according to study of 47 patients between 2008 and 2014.

• Hispanic patients, most of whom were immigrant and Spanish speaking, were treated at a comprehensive epilepsy center in an urban public health setting.
• They underwent presurgical and postsurgical neuropsychological evaluation and were identified retrospectively.
• Medium to large improvements on the Beck Depression Inventory and most quality of care scales were observed postsurgery.
• Less robust effects were also seen on the Beck Anxiety Inventory.

Smith JA, Armacost M, Ensign E, et al. Epilepsy surgery in the underserved Hispanic population improves depression, anxiety, and quality of life. Epilepsy Behav. 2018;83:1-6. https://doi.org/10.1016/j.yebeh.2018.03.015

Too few older adults are included in antiepileptic drug trials according to a systematic review and meta-analysis that included 184 studies.

• In 1991 to 1992, the mean age of patients included in clinical trials was 27 years, which had increased to about 42 years in 2015 to 2016.
• In 83 studies (44%), inclusion criteria included a maximum age limit.
• The requirement for limiting a participant’s maximum age did not decline significantly over time between the two time periods (r = 0.072, P=.816).
• The only disease-related exclusion criteria for entry into a clinical trial for antiepileptic drugs that was linked to a drop in the average age of enrolled patients were neurological conditions other than epilepsy.

Desmarais P, Miville C, Milán-Tomás A, et al. Age representation in antiepileptic drug trials: a systematic review and meta-analysis. Epilepsy Res. 2018;142:9-15.

Too few older adults are included in antiepileptic drug trials according to a systematic review and meta-analysis that included 184 studies.

• In 1991 to 1992, the mean age of patients included in clinical trials was 27 years, which had increased to about 42 years in 2015 to 2016.
• In 83 studies (44%), inclusion criteria included a maximum age limit.
• The requirement for limiting a participant’s maximum age did not decline significantly over time between the two time periods (r = 0.072, P=.816).
• The only disease-related exclusion criteria for entry into a clinical trial for antiepileptic drugs that was linked to a drop in the average age of enrolled patients were neurological conditions other than epilepsy.

Desmarais P, Miville C, Milán-Tomás A, et al. Age representation in antiepileptic drug trials: a systematic review and meta-analysis. Epilepsy Res. 2018;142:9-15.

Too few older adults are included in antiepileptic drug trials according to a systematic review and meta-analysis that included 184 studies.

• In 1991 to 1992, the mean age of patients included in clinical trials was 27 years, which had increased to about 42 years in 2015 to 2016.
• In 83 studies (44%), inclusion criteria included a maximum age limit.
• The requirement for limiting a participant’s maximum age did not decline significantly over time between the two time periods (r = 0.072, P=.816).
• The only disease-related exclusion criteria for entry into a clinical trial for antiepileptic drugs that was linked to a drop in the average age of enrolled patients were neurological conditions other than epilepsy.

Desmarais P, Miville C, Milán-Tomás A, et al. Age representation in antiepileptic drug trials: a systematic review and meta-analysis. Epilepsy Res. 2018;142:9-15.

An international team of experts has published standardized definitions of several seizure-related disorders, including new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related disorders.

• NORSE is described as a clinical presentation rather than a specific diagnosis.
• NORSE occurs in a patient who is not experiencing active epilepsy or any other preexisting neurological disorder.
• In NORSE, the patient does not have a clear acute or active structural, toxic or metabolic cause of their condition.
•  The expert group defined FIRES as a subtype of NORSE that involves a prior febrile infection that started between 2 weeks and 24 hours before the refractory status epilepticus began.
• The experts also offered standardized definitions for infantile hemiconvulsion-hemiplegia and epilepsy syndrome (IHHE) and for prolonged, refractory and super-refractory status epilepticus.

Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia. 2018;59(4):739-744.

An international team of experts has published standardized definitions of several seizure-related disorders, including new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related disorders.

• NORSE is described as a clinical presentation rather than a specific diagnosis.
• NORSE occurs in a patient who is not experiencing active epilepsy or any other preexisting neurological disorder.
• In NORSE, the patient does not have a clear acute or active structural, toxic or metabolic cause of their condition.
•  The expert group defined FIRES as a subtype of NORSE that involves a prior febrile infection that started between 2 weeks and 24 hours before the refractory status epilepticus began.
• The experts also offered standardized definitions for infantile hemiconvulsion-hemiplegia and epilepsy syndrome (IHHE) and for prolonged, refractory and super-refractory status epilepticus.

Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia. 2018;59(4):739-744.

An international team of experts has published standardized definitions of several seizure-related disorders, including new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related disorders.

• NORSE is described as a clinical presentation rather than a specific diagnosis.
• NORSE occurs in a patient who is not experiencing active epilepsy or any other preexisting neurological disorder.
• In NORSE, the patient does not have a clear acute or active structural, toxic or metabolic cause of their condition.
•  The expert group defined FIRES as a subtype of NORSE that involves a prior febrile infection that started between 2 weeks and 24 hours before the refractory status epilepticus began.
• The experts also offered standardized definitions for infantile hemiconvulsion-hemiplegia and epilepsy syndrome (IHHE) and for prolonged, refractory and super-refractory status epilepticus.

Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions. Epilepsia. 2018;59(4):739-744.