Hippocampal malrotation is characterized by incomplete hippocampal inversion with a rounded shape... more Hippocampal malrotation is characterized by incomplete hippocampal inversion with a rounded shape and blurred internal architecture. There is still debate about whether hippocampal malrotation has pathologic significance. We present findings from the Consequences of Prolonged Febrile Seizures in Childhood (FEBSTAT) study on the frequency of and risk factors for hippocampal malrotation. FEBSTAT is a prospective multicenter study investigating the consequences of febrile status epilepticus in childhood. MRI studies of 226 patients with febrile status epilepticus were analyzed visually by two board-certified neuroradiologists blinded to clinical details and were compared with MRI studies of 96 subjects with first simple febrile seizure. Quantitative analysis of hippocampal volume was performed by two independent observers. Hippocampal malrotation was present in 20 of 226 (8.8%) patients with febrile status epilepticus compared with two of 96 (2.1%) control subjects (odds ratio [OR], 4....
We examined the associations of lifetime and current histories of psychiatric disorders and of su... more We examined the associations of lifetime and current histories of psychiatric disorders and of suicidal thoughts and behaviors with childhood-onset epilepsies in a community-based cohort of young adults. Cases were neurotypical (normal neurologic, cognitive, and imaging examinations and no evidence of a brain insult responsible for the epilepsy) young adults with childhood-onset epilepsy followed since the onset of their epilepsy approximately 15 years earlier and recruited as part of a community-based study. They were compared to two different control groups: siblings and external controls from the National Comorbidity Survey-Replication (NCS-R). The Diagnostic Interview Survey assessed lifetime and current Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR) diagnoses of mood disorders and anxiety disorders. Suicidal thoughts and suicide attempt were assessed using the Diagnostic Interview Survey for Children-IV and the Diagnostic Interv...
We considered whether a subset of children with sudden unexplained death in childhood (SUDC) and ... more We considered whether a subset of children with sudden unexplained death in childhood (SUDC) and a history of febrile seizures (FS) may parallel those in sudden unexpected death in epilepsy (SUDEP). The prevalence of a history of FS was examined, and factors that may distinguish SUDC cases with and without FS were described. Characteristics were assessed in 123 consecutive children with SUDC reported to the SUDC program (4/1/11-3/31/14) by their parents. Parental interview covered the decedent's medical history, circumstances of death, environmental factors, cause of death, and family medical history. Features of SUDC cases were compared by FS history. Overall, 31.7% of SUDC cases had a history of FS, among which 74.4% had simple FS. Compared to those without a history of FS, a history of FS was associated with a greater median age at death (p = 0.03) and death during the weekdays (p = 0.02). Terminal fever was similar in those with and without FS. The median time from FS to death was 6.0 months (interquartile range [IQR] 3.0-10.0). In all SUDC cases, prone position at death, death during sleep, and unwitnessed deaths predominated. There are parallels among SUDC, sudden infant deaths, and sudden unexpected death in epilepsy (SUDEP) with regard to prone position, unwitnessed deaths mostly during sleep, and male predominance. In children with SUDC and a history of FS, terminal fever may increase the risk for an unwitnessed terminal seizure. The greater than expected prevalence of a FS history and the proportion with terminal fever or illness in this cohort suggests that some SUDC deaths may be seizure related and therefore have potential commonalities with SUDEP.
OBJECTIVE.To determine the likelihood of intracranial pathologic conditions requir- ing emergency... more OBJECTIVE.To determine the likelihood of intracranial pathologic conditions requir- ing emergency neurosurgical or medical intervention among children without meningitis who presented to the pediatric emergency department after a first complex febrile seizure. METHODS.We performed a retrospective review of prospectively collected data for children in neurologically normal condition who presented to a single pediatric emergency department after a first complex febrile
Febrile status epilepticus (FSE) has been associated with hippocampal injury and subsequent mesia... more Febrile status epilepticus (FSE) has been associated with hippocampal injury and subsequent mesial temporal sclerosis and temporal lobe epilepsy. However, little is known about the semiology of FSE. A prospective, multicenter study of the consequences of FSE included children, aged 1 month through 5 years, presenting with a febrile seizure lasting 30 minutes or more. Procedures included neurologic history and examination and an MRI and EEG within 72 hours. All information related to seizure semiology was reviewed by three epileptologists blinded to MRI and EEG results and to subsequent outcome. Inter-rater reliability was assessed by the kappa statistic. Among 119 children, the median age was 1.3 years, the mean peak temperature was 103.2 degrees F, and seizures lasted a median of 68.0 minutes. Seizure duration followed a Weibull distribution with a shape parameter of 1.68. Seizures were continuous in 52% and behaviorally intermittent (without recovery in between) in 48%; most were partial (67%) and almost all (99%) were convulsive. In one third of cases, FSE was unrecognized in the emergency department. Of the 119 children, 86% had normal development, 24% had prior febrile seizures, and family history of febrile seizures in a first-degree relative was present in 25%. Febrile status epilepticus is usually focal and often not well recognized. It occurs in very young children and is usually the first febrile seizure. Seizures are typically very prolonged and the distribution of seizure durations suggests that the longer a seizure continues, the less likely it is to spontaneously stop.
The Journal of Trauma: Injury, Infection, and Critical Care, 2002
In 1995, evidence-based guidelines for the management of severe traumatic brain injury (TBI) were... more In 1995, evidence-based guidelines for the management of severe traumatic brain injury (TBI) were published and disseminated. Information regarding their implementation is limited. During 1999 to 2000, we contacted all designated U.S. trauma centers caring for adults with severe TBI to determine the degree of guideline compliance and to identify predictors. Of 924 centers identified, 828 participated (90%). Four hundred thirty-three with intensive care units caring for severe TBI were surveyed. Three hundred ninety-five centers transferring patients were excluded. Full guideline compliance was rare (n = 68 [16%]). In multivariate analyses, treatment protocols (odds ratio [OR], 3.6; 95% confidence interval [CI], 1.9-6.6), neurosurgery residency program (OR, 5.0; 95% CI, 2.6-9.8), and state (OR, 2.7; 95% CI, 0.62-12) or American College of Surgeons (OR, 5.1; 95% CI, 1.1-23) designation increased the likelihood of full compliance versus noncompliance. Although evidence-based guidelines were published and disseminated in 1995, implementation is infrequent. Focus must turn to changing physician practice and transport decisions to provide guideline-compliant care and improve patient outcome.
On April 30th, 2011 the National Institute of Neurological Disorders and Stroke (NINDS) held a wo... more On April 30th, 2011 the National Institute of Neurological Disorders and Stroke (NINDS) held a workshop to identify key problems in recent epilepsy clinical trials and propose approaches to address the barriers that impede development of new therapeutic options for epilepsy. Preliminary recommendations were made for selection criteria for subjects entered into epilepsy trials that maximize the scientific impact of the trial and increase the ability to recruit appropriate subjects efficiently and safely. These recommendations were further refined by the authors following the workshop, and subsequently shared with all NINDS workshop participants and with the participants of the 2011 AED XI workshop on epilepsy trials (approximately 200 participants) for further comment. The working group agreed to a final set of criteria that include updated considerations of subject age, clinical semiology, EEG and imaging results, use of prior and current therapies, co-occurring conditions, and suicidality, among others.
Stigma is associated with prevalent epilepsy, but its association with incident epilepsy is unkno... more Stigma is associated with prevalent epilepsy, but its association with incident epilepsy is unknown. We identified 209 children and adults with incident seizures from the diverse impoverished community of northern Manhattan. We interviewed 94 participants, aged 16 and older, about lifetime history of depression, health status, medical history, and stigma. At baseline, 18 (22.5%) participants reported experiencing stigma. Stigma was reported by 9 (50.0%) with depression and 9 (14.5%) without depression (P=0.002). At 1 year, 7 (8.1%) participants reported experiencing stigma. Stigma was reported by 5 (31.3%) with depression versus 1 (1.6%) without depression (P<0.0001). At both time points, odds of stigma increased when lifetime history of depression and fair/poor health was present. Previous work revealed negative effects of prevalent epilepsy on stigma. In the low-income, predominantly Hispanic community of northern Manhattan, we found incident epilepsy was associated with stigma when lifetime history of depression or fair/poor health was present.
Hippocampal malrotation is characterized by incomplete hippocampal inversion with a rounded shape... more Hippocampal malrotation is characterized by incomplete hippocampal inversion with a rounded shape and blurred internal architecture. There is still debate about whether hippocampal malrotation has pathologic significance. We present findings from the Consequences of Prolonged Febrile Seizures in Childhood (FEBSTAT) study on the frequency of and risk factors for hippocampal malrotation. FEBSTAT is a prospective multicenter study investigating the consequences of febrile status epilepticus in childhood. MRI studies of 226 patients with febrile status epilepticus were analyzed visually by two board-certified neuroradiologists blinded to clinical details and were compared with MRI studies of 96 subjects with first simple febrile seizure. Quantitative analysis of hippocampal volume was performed by two independent observers. Hippocampal malrotation was present in 20 of 226 (8.8%) patients with febrile status epilepticus compared with two of 96 (2.1%) control subjects (odds ratio [OR], 4....
We examined the associations of lifetime and current histories of psychiatric disorders and of su... more We examined the associations of lifetime and current histories of psychiatric disorders and of suicidal thoughts and behaviors with childhood-onset epilepsies in a community-based cohort of young adults. Cases were neurotypical (normal neurologic, cognitive, and imaging examinations and no evidence of a brain insult responsible for the epilepsy) young adults with childhood-onset epilepsy followed since the onset of their epilepsy approximately 15 years earlier and recruited as part of a community-based study. They were compared to two different control groups: siblings and external controls from the National Comorbidity Survey-Replication (NCS-R). The Diagnostic Interview Survey assessed lifetime and current Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision (DSM-IV-TR) diagnoses of mood disorders and anxiety disorders. Suicidal thoughts and suicide attempt were assessed using the Diagnostic Interview Survey for Children-IV and the Diagnostic Interv...
We considered whether a subset of children with sudden unexplained death in childhood (SUDC) and ... more We considered whether a subset of children with sudden unexplained death in childhood (SUDC) and a history of febrile seizures (FS) may parallel those in sudden unexpected death in epilepsy (SUDEP). The prevalence of a history of FS was examined, and factors that may distinguish SUDC cases with and without FS were described. Characteristics were assessed in 123 consecutive children with SUDC reported to the SUDC program (4/1/11-3/31/14) by their parents. Parental interview covered the decedent's medical history, circumstances of death, environmental factors, cause of death, and family medical history. Features of SUDC cases were compared by FS history. Overall, 31.7% of SUDC cases had a history of FS, among which 74.4% had simple FS. Compared to those without a history of FS, a history of FS was associated with a greater median age at death (p = 0.03) and death during the weekdays (p = 0.02). Terminal fever was similar in those with and without FS. The median time from FS to death was 6.0 months (interquartile range [IQR] 3.0-10.0). In all SUDC cases, prone position at death, death during sleep, and unwitnessed deaths predominated. There are parallels among SUDC, sudden infant deaths, and sudden unexpected death in epilepsy (SUDEP) with regard to prone position, unwitnessed deaths mostly during sleep, and male predominance. In children with SUDC and a history of FS, terminal fever may increase the risk for an unwitnessed terminal seizure. The greater than expected prevalence of a FS history and the proportion with terminal fever or illness in this cohort suggests that some SUDC deaths may be seizure related and therefore have potential commonalities with SUDEP.
OBJECTIVE.To determine the likelihood of intracranial pathologic conditions requir- ing emergency... more OBJECTIVE.To determine the likelihood of intracranial pathologic conditions requir- ing emergency neurosurgical or medical intervention among children without meningitis who presented to the pediatric emergency department after a first complex febrile seizure. METHODS.We performed a retrospective review of prospectively collected data for children in neurologically normal condition who presented to a single pediatric emergency department after a first complex febrile
Febrile status epilepticus (FSE) has been associated with hippocampal injury and subsequent mesia... more Febrile status epilepticus (FSE) has been associated with hippocampal injury and subsequent mesial temporal sclerosis and temporal lobe epilepsy. However, little is known about the semiology of FSE. A prospective, multicenter study of the consequences of FSE included children, aged 1 month through 5 years, presenting with a febrile seizure lasting 30 minutes or more. Procedures included neurologic history and examination and an MRI and EEG within 72 hours. All information related to seizure semiology was reviewed by three epileptologists blinded to MRI and EEG results and to subsequent outcome. Inter-rater reliability was assessed by the kappa statistic. Among 119 children, the median age was 1.3 years, the mean peak temperature was 103.2 degrees F, and seizures lasted a median of 68.0 minutes. Seizure duration followed a Weibull distribution with a shape parameter of 1.68. Seizures were continuous in 52% and behaviorally intermittent (without recovery in between) in 48%; most were partial (67%) and almost all (99%) were convulsive. In one third of cases, FSE was unrecognized in the emergency department. Of the 119 children, 86% had normal development, 24% had prior febrile seizures, and family history of febrile seizures in a first-degree relative was present in 25%. Febrile status epilepticus is usually focal and often not well recognized. It occurs in very young children and is usually the first febrile seizure. Seizures are typically very prolonged and the distribution of seizure durations suggests that the longer a seizure continues, the less likely it is to spontaneously stop.
The Journal of Trauma: Injury, Infection, and Critical Care, 2002
In 1995, evidence-based guidelines for the management of severe traumatic brain injury (TBI) were... more In 1995, evidence-based guidelines for the management of severe traumatic brain injury (TBI) were published and disseminated. Information regarding their implementation is limited. During 1999 to 2000, we contacted all designated U.S. trauma centers caring for adults with severe TBI to determine the degree of guideline compliance and to identify predictors. Of 924 centers identified, 828 participated (90%). Four hundred thirty-three with intensive care units caring for severe TBI were surveyed. Three hundred ninety-five centers transferring patients were excluded. Full guideline compliance was rare (n = 68 [16%]). In multivariate analyses, treatment protocols (odds ratio [OR], 3.6; 95% confidence interval [CI], 1.9-6.6), neurosurgery residency program (OR, 5.0; 95% CI, 2.6-9.8), and state (OR, 2.7; 95% CI, 0.62-12) or American College of Surgeons (OR, 5.1; 95% CI, 1.1-23) designation increased the likelihood of full compliance versus noncompliance. Although evidence-based guidelines were published and disseminated in 1995, implementation is infrequent. Focus must turn to changing physician practice and transport decisions to provide guideline-compliant care and improve patient outcome.
On April 30th, 2011 the National Institute of Neurological Disorders and Stroke (NINDS) held a wo... more On April 30th, 2011 the National Institute of Neurological Disorders and Stroke (NINDS) held a workshop to identify key problems in recent epilepsy clinical trials and propose approaches to address the barriers that impede development of new therapeutic options for epilepsy. Preliminary recommendations were made for selection criteria for subjects entered into epilepsy trials that maximize the scientific impact of the trial and increase the ability to recruit appropriate subjects efficiently and safely. These recommendations were further refined by the authors following the workshop, and subsequently shared with all NINDS workshop participants and with the participants of the 2011 AED XI workshop on epilepsy trials (approximately 200 participants) for further comment. The working group agreed to a final set of criteria that include updated considerations of subject age, clinical semiology, EEG and imaging results, use of prior and current therapies, co-occurring conditions, and suicidality, among others.
Stigma is associated with prevalent epilepsy, but its association with incident epilepsy is unkno... more Stigma is associated with prevalent epilepsy, but its association with incident epilepsy is unknown. We identified 209 children and adults with incident seizures from the diverse impoverished community of northern Manhattan. We interviewed 94 participants, aged 16 and older, about lifetime history of depression, health status, medical history, and stigma. At baseline, 18 (22.5%) participants reported experiencing stigma. Stigma was reported by 9 (50.0%) with depression and 9 (14.5%) without depression (P=0.002). At 1 year, 7 (8.1%) participants reported experiencing stigma. Stigma was reported by 5 (31.3%) with depression versus 1 (1.6%) without depression (P<0.0001). At both time points, odds of stigma increased when lifetime history of depression and fair/poor health was present. Previous work revealed negative effects of prevalent epilepsy on stigma. In the low-income, predominantly Hispanic community of northern Manhattan, we found incident epilepsy was associated with stigma when lifetime history of depression or fair/poor health was present.
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