Christine O'Dell

Infantile spasms: A U.S. consensus report

The diagnosis, evaluation, and management of infantile spasms (IS) continue to pose significant challenges to the treating physician. Although an evidence‐based practice guideline with full literature review was published in 2004, diversity in IS evaluation and treatment remains and highlights the need for further consensus to optimize outcomes in IS. For this purpose, a working group committed to the diagnosis, treatment, and establishment of a continuum of care for patients with IS and their families—the Infantile Spasms Working Group (ISWG)—was convened. The ISWG participated in a workshop for which the key objectives were to review the state of our understanding of IS, assess the scientific evidence regarding efficacy of currently available therapeutic options, and arrive at a consensus on protocols for diagnostic workup and management of IS that can serve as a guide to help specialists and general pediatricians optimally manage infants with IS. The overall goal of the workshop was to improve IS outcomes by assisting treating physicians with early recognition and diagnosis of IS, initiation of short duration therapy with a first‐line treatment, timely electroencephalography (EEG) evaluation of treatment to evaluate effectiveness, and, if indicated, prompt treatment modification. Differences of opinion among ISWG members occurred in areas where data were lacking; however, this article represents a consensus of the U.S. approach to the diagnostic evaluation and treatment of IS.

Predictors of multiple seizures in a cohort of children prospectively followed from the time of their first unprovoked seizure

The objective of this study was to assess the risk of multiple recurrences after an initial seizure recurrence in childhood. In a prospective study, 407 children were followed for a mean of 9.6 years from the time of their first unprovoked seizure. Data regarding each seizure recurrence were obtained and analyzed using statistical methods for survival analysis. The cumulative risk of a second seizure was 29%, 37%, 43%, and 46% at 1, 2, 5, and 10 years, respectively. Of the 182 children who experienced a second seizure, 131 (72%) experienced a third seizure, 105 (58%) have had 4 or more seizures, and 52 (29%) have experienced 10 or more seizures. The cumulative risk of a third seizure was 57%, 63%, and 71% at 1, 2, and 5 years, respectively, after the second seizure. After a third seizure, the cumulative risk of another seizure was 69%, 72%, and 81% at 1, 2, and 5 years, respectively. After a second seizure, factors associated with an increased risk of additional recurrences included a remote symptomatic etiology (rate ratio = 1.7) and the occurrence of a second seizure within 6 months of the first seizure (rate ratio = 1.7). After a second seizure, the risk of subsequent seizures was greater than 50% even in the lowest risk group. With the exception of etiology, factors associated with an increased risk of multiple recurrences after the initial seizure were different than those associated with multiple recurrences after a second seizure. Factors associated with multiple recurrent seizures may be different than those associated with an initial recurrence. As most patients who experience a second seizure experience further seizures, these data suggest that two seizures are a sufficient epidemiological criterion for the definition of epilepsy. Ann Neurol 2000;48:140–147

PHENOMENOLOGY OF PROLONGED FEBRILE SEIZURES: RESULTS OF THE FEBSTAT STUDY

Background: 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. Methods: 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 κ statistic. Results: Among 119 children, the median age was 1.3 years, the mean peak temperature was 103.2°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%. Conclusions: 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. GLOSSARY: ED = emergency department; FS = febrile seizures; FSE = febrile status epilepticus; HHV = human herpesvirus; ILAE = International League Against Epilepsy; IQR = interquartile range; MTLE = mesial temporal lobe epilepsy; MTS = mesial temporal sclerosis.

Distribution of Epilepsy Syndromes in a Cohort of Children Prospectively Monitored from the Time of Their First Unprovoked Seizure

Summary: Purpose: To assess the distribution of epilepsy syndromes and their stability in children.

Short-Term Outcomes of Children with Febrile Status Epilepticus

Summary: Febrile status epilepticus (SE) represents the extreme end of the complex febrile seizure spectrum. If there are significant sequelae to febrile seizures, they should be more common in this group. We have prospectively identified 180 children aged 1 month to 10 years who presented with febrile SE over a 10‐year period in Bronx, New York, and Richmond, Virginia. They were compared with 244 children who presented with their first febrile seizure (not SE) in a prospective study done in the Bronx.

Mortality following a first unprovoked seizure in children: A prospective study

In a prospective study, 407 children with a first unprovoked seizure were followed for a mean of 14.2 years. To date, nine have died. Death was unrelated to seizures in four subjects who had no further seizures and were on no medications. The remaining five subjects all had multiple seizures and were on medications. Treatment following the first seizure would not have altered mortality in any of the nine cases.

The personal and financial impact of repetitive or prolonged seizures on the patient and family

Individuals with epilepsy consistently report diminished quality of life. The clinical characteristics of seizures and the unpredictable nature of seizure occurrences are some factors that affect quality of life. Prolonged or repetitive seizures can impose psychologic comorbidities, social issues, and lifestyle restrictions that can affect quality of life of patients and their caregivers and family members, who also bear the considerable indirect costs of seizures, including time away from work or school, and even loss of employment. The availability and use of an at-home medication to terminate prolonged or repetitive seizures or in seizure emergencies improves quality of life for patients and their families. Fewer visits to the emergency department are associated with a reduction in the financial burden to families and the health care system. This article discusses factors that contribute to the personal and financial impact of prolonged seizures on adult and pediatric patients, their families, and caregivers.

Cognitive outcomes in children who present with a first unprovoked seizure

Purpose:  To determine the long‐term cognitive and educational outcomes in children prospectively identified at the time of a first unprovoked seizure.

Issues related to caring for infants to adults on an integrated epilepsy unit.

&NA; We integrated the care of patients of all ages (ranging thus far from 4 weeks to 73 years) in our dedicated 8‐bed Epilepsy Unit. Administrative issues pertaining to admission and discharge criteria, unit policies and procedures and an interdisciplinary quality assurance plan were examined in relation to the impact of combining both pediatric and adult patients. Clinical considerations included the diversified abilities needed to care for pediatric and adult patients both in relation to the technical skills as well as psychosocial skills required. The advantages of integrating patients of all ages on one unit include having a staff highly trained in assessment and intervention skills for a particular disorder. The psychosocial issues that arise in these patients, regardless of age, tend to encompass the entire family; therefore a holistic approach is appropriate for both children and adults. An autonomous nursing practice was established with the development of critical pathways and patient care protocols. Our experience suggests that integrated specialized units can enhance the care of patients with intractable seizures.