Recurrent spontaneous spinal epidural hematoma in a child: first case report.

Spontaneous spinal epidural hematomas are a rare cause of spinal cord compression. These are typically isolated events. Recurrent spontaneous spinal epidural hematomas have been reported only in two instances in adults. There have been no reports of recurrent spontaneous spinal epidural hematomas involving a child. This report details the case of a 10-year-old female with a recurrent spontaneous spinal epidural hematomas who presented with two episodes of transient lower extremity paralysis, 2 months apart, followed by a third episode a year later which resulted in permanent paralysis.

Survival following decompressive hemicraniectomy for hemiconvulsion-hemiplegia-epilepsy syndrome: case report.

Hemiconvulsion-hemiplegia-epilepsy (HHE) is an uncommon epileptic syndrome that affects young children. Typical management includes early initiation of benzodiazepines to abate the initial seizure activity quickly. Patients in whom epilepsy develops require prolonged use of antiepileptic agents. Herniation due to diffuse cerebral edema from HHE is rare; however, decompressive craniectomy has been described as a lifesaving measure. The authors present the case of a patient in whom a decompressive craniectomy was performed. They advocate a proactive approach in the detection and management of cerebral edema in HHE causing intracranial hypertension. In HHE cases that exhibit radiographic evidence of malignant cerebral edema (although not previously described in this disease, but similar to the setting of stroke and trauma), the authors advocate early neurosurgical consultation and evaluation for insertion of an intracranial pressure monitor for those patients who do not have a reliable neurological examination (i.e., Glasgow Coma Scale score ≤ 8).

Hippocampal Formation Maldevelopment and Sudden Unexpected Death across the Pediatric Age Spectrum.

Sudden infant death syndrome (SIDS) and sudden unexplained death in childhood (SUDC) are defined as sudden death in a child remaining unexplained despite autopsy and death scene investigation. They are distinguished from each other by age criteria, i.e. with SIDS under 1 year and SUDC over 1 year. Our separate studies of SIDS and SUDC provide evidence of shared hippocampal abnormalities, specifically focal dentate bilamination, a lesion classically associated with temporal lobe epilepsy, across the 2 groups. In this study, we characterized the clinicopathologic features in a retrospective case series of 32 children with sudden death and hippocampal formation (HF) maldevelopment. The greatest frequency of deaths was between 3 weeks and 3 years (81%, 26/32). Dentate anomalies were found across the pediatric age spectrum, supporting a common vulnerability that defies the 1-year age cutoff between SIDS and SUDC. Twelve cases (38%) had seizures, including 7 only with febrile seizures. Subicular anomalies were found in cases over 1 year of age and were associated with increased risk of febrile seizures. Sudden death associated with HF maldevelopment reflects a complex interaction of intrinsic and extrinsic factors that lead to death at different pediatric ages, and may be analogous to sudden unexplained death in epilepsy.

Absence epilepsy with focal clinical and electrographic seizures.

Primary generalized seizures occur in half of all children with epilepsy. Correct classification carries important diagnostic, therapeutic, and prognostic information. Approximately half of all patients with generalized juvenile myoclonic epilepsy may have a partial semiology or focal electroencephalographic features although similar findings have not been described in absence epilepsy. Two examples, one with semiology characteristic of focal seizures and the other with a video-electroencephalogram documented partial seizure emanating after the completion of typical 3-Hz generalized spike-wave absence epilepsy, are discussed.