Syndi Seinfeld

Design and phenomenology of the FEBSTAT study.

Purpose:  Febrile status epilepticus (FSE) has been associated with hippocampal injury and subsequent hippocampal sclerosis (HS) and temporal lobe epilepsy. The FEBSTAT study was designed to prospectively examine the association between prolonged febrile seizures and development of HS and associated temporal lobe epilepsy, one of the most controversial issues in epilepsy. We report on the baseline phenomenology of the final cohorts as well as detailed aims and methodology.

Emergency Management of Febrile Status Epilepticus: Results of the FEBSTAT Study.

Treatment of seizures varies by region, with no standard emergency treatment protocol. Febrile status epilepticus (FSE) is often a childs first seizure; therefore, families are rarely educated about emergency treatment.

Plasma cytokines associated with febrile status epilepticus in children: A potential biomarker for acute hippocampal injury

Our aim was to explore the association between plasma cytokines and febrile status epilepticus (FSE) in children, as well as their potential as biomarkers of acute hippocampal injury.

Cognitive functioning one month and one year following febrile status epilepticus

OBJECTIVE The objective of this study was to determine early developmental and cognitive outcomes of children with febrile status epilepticus (FSE) one month and one year after FSE. METHODS One hundred ninety four children with FSE were evaluated on measures of cognition, receptive language, and memory as part of the FEBSTAT study and compared with 100 controls with simple febrile seizures (FSs). RESULTS Children with FSE did not differ dramatically on tasks compared with FS controls at one month after FSE but demonstrated slightly weaker motor development (p=0.035) and receptive language (p=0.034) at one year after FSE. Performances were generally within the low average to average range. Within the FSE cohort, non-White children performed weaker on many of the tasks compared with Caucasian children. At the one-year visit, acute hippocampal T2 findings on MRI were associated with weaker receptive language skills (p=0.0009), and human herpes virus 6 or 7 (HHV6/7) viremia was associated with better memory performances (p=0.047). CONCLUSION Febrile status epilepticus does not appear to be associated with significant cognitive impairment on early developmental measures, although there is a trend for possible receptive language and motor delay one year after FSE. Further follow-up, which is in progress, is necessary to track long-term cognitive functioning.

Benzodiazepine use in seizure emergencies: A systematic review

PURPOSE The aim of this review was to systematically examine safety and efficacy outcomes, as well as patient/caregiver satisfaction, from clinical studies in pediatric and adult patients treated with benzodiazepines (BZDs) through various administration routes in response to seizure emergencies. METHODS A literature search was conducted to identify articles describing the use of various routes of administration (RoAs) of BZDs for the treatment of seizure emergencies through April 21, 2015, using Embase™ and PubMed®. Eligible studies included (a) randomized controlled trials or (b) controlled nonrandomized clinical trials, either retrospective or prospective. Outcome assessments reviewed were 1) time to administration, 2) time to seizure termination, 3) rate of treatment failure, 4) prevention of seizure recurrence, 5) patient and caregiver treatment satisfaction, 6) adverse events related to BDZ treatment or RoA, and 7) respiratory adverse events. RESULTS Seventy-five studies evaluated safety and efficacy using individual or comparator BDZs of various RoAs for treating seizure emergencies in all-aged patients with epilepsy. Buccal, intranasal (IN), or intramuscular (IM) BZDs were often more rapidly administered compared with rectal and intravenous (IV) formulations. Time to seizure termination, seizure recurrence rates, and adverse events were generally similar among RoAs, whereas nonrectal RoAs resulted in greater patient and caregiver satisfaction compared with rectal RoA. SIGNIFICANCE Results of this systematic literature review suggest that nonrectal and non-IV BZD formulations provide equal or improved efficacy and safety outcomes compared with rectal and IV formulations for the treatment of seizure emergencies.

Risk factors for subsequent febrile seizures in the FEBSTAT study

To identify risk and risk factors for developing a subsequent febrile seizure (FS) in children with a first febrile status epilepticus (FSE) compared to a first simple febrile seizure (SFS). To identify home use of rescue medications for subsequent FS.

Epilepsy After Febrile Seizures: Twins Suggest Genetic Influence

BACKGROUND A history of complex febrile seizures can increase the risk of epilepsy, but the role of genetic factors is unclear. This analysis evaluated the relationship between febrile seizures and epilepsy. METHODS Information on the history of seizures was obtained by a questionnaire from twin pairs in the Mid-Atlantic, Danish, and Norwegian Twin Registries. The information was verified using medical records and detailed clinical and family interviews. The initial study evaluated the genetic epidemiology of febrile seizures in this population. Further information was analyzed and used to evaluate genetic associations of different febrile seizure subtypes. RESULTS Histories of febrile seizures were validated in 1051 twins in 900 pairs. The febrile seizure type was classified as simple, complex, or febrile status epilepticus. There were 61% simple, 12% complex, and 7% febrile status epilepticus. There were 78 twins who developed epilepsy. The highest rate of epilepsy (22.2%) occurred in the febrile status epilepticus group. Concordance was highest in simple group. CONCLUSION A twin with febrile status epilepticus is at the highest risk of developing epilepsy, but simple febrile seizures gave the highest risk for the unaffected twin to develop seizures or other neurological issues. These results are consistent with previous findings. There is a subgroup of febrile seizures that can be associated with long-term consequences. This subgroup can be associated with a significant financial and emotional burden. It is currently not possible to accurately identify which children will develop recurrent febrile seizures, epilepsy, or neuropsychological comorbidities.

Epilepsy surgery in a patient with Dandy-Walker variant

Epilepsy surgery is an option for many pediatric patients diagnosed with intractable seizures. Identifying pediatric patients that are candidates for surgery can be complicated. This case presented a 19-year-old female with intractable epilepsy who had chronic immune thrombocytopenic purpura and Dandy-Walker variant. The patient had previously been prescribed three appropriate anti-epileptic drugs and continued to experience seizures weekly. The patient had thrombocytopenia, which represented a potential for increased complications. The pre-operative work-up demonstrated heterotopias and dysplasias, which were felt to be the source of the focal onset seizures. She underwent focal resection and at the time of the last follow-up visit, the patient had been seizure free for 2 yr. The outcome of the patient exemplified the importance of evaluating patients with posterior fossa abnormalities for epilepsy surgery. The data in patients with Dandy-Walker variant is limited.

Is there a need for further trials for the treatment of prolonged seizures

Prolonged seizures are associated with morbidity and mortality of varying degrees. It is important to recognize seizures early, and treat them appropriately. This leads to the best clinical outcome. There has been an emphasis on prompt treatment, but there exists a variety of poorly executed protocols. This review addresses the question of whether additional clinical trials are necessary, not only to answer for what purpose, but also, clearly, to examine the impact additional studies may have. Overall, the acute treatment of epilepsy emergencies in children has markedly improved with availability of out-of-hospital therapies, but additional studies to determine the most efficacious, maximally safe, and best tolerated treatments are needed.