Decreased heart rate and enhanced sinus arrhythmia during interictal sleep demonstrate autonomic imbalance in generalized epilepsy.

We hypothesized that epilepsy affects the activity of the autonomic nervous system even in the absence of seizures, which should manifest as differences in heart rate variability (HRV) and cardiac cycle. To test this hypothesis, we investigated ECG traces of 91 children and adolescents with generalized epilepsy and 25 neurologically normal controls during 30 min of stage 2 sleep with interictal or normal EEG. Mean heart rate (HR) and high-frequency HRV corresponding to respiratory sinus arrhythmia (RSA) were quantified and compared.

Tailored disconnection based on presurgical evidence in catastrophic epilepsy: report of 2 cases.

Catastrophic epilepsy in infants, often due to extensive cortical dysplasia, has devastating consequences with respect to brain development. Conventional lobar, multilobar, or hemispheric resection in these infants is challenging, carrying an increased operative risk compared with that in older children. Removing a larger tissue volume versus removing or disconnecting the epileptogenic region does not always guarantee better seizure outcome.

Angiocentric glioma: report of clinico-pathologic and genetic findings in 8 cases.

Angiocentric glioma has recently been described as a novel epilepsy associated tumor with distinct clinico-pathologic features. We report the clinical and pathologic findings in 8 additional cases of this rare tumor type and extend its characterization by genomic profiling. Almost all patients had a history of long-standing drug-resistant epilepsy.

Familial subacute sclerosing panencephalitis in two siblings.

Subacute sclerosing panencephalitis is a form of chronic persistent measles encephalitis in childhood which rarely manifests after wild virus infection. One previous case of familial subacute sclerosing panencephalitis in two siblings and a number of reports of subacute sclerosing panencephalitis in one member of monozygous twins have been reported in the literature. This report describes a second sibling set who both contracted subacute sclerosing panencephalitis after concurrent sporadic measles infection.

Pseudostatus epilepticus in childhood.

Pseudostatus epilepticus in childhood has not been well reported in the literature. We describe the clinical presentation and management of a 9-year-old child with well-controlled epilepsy who presented in a prolonged period of pseudoseizures. Intensive care management over a number of weeks with multiple high-dose antiepileptic drugs, anesthesia, and ventilation at a tertiary care pediatric center was performed before the diagnosis of pseudostatus epilepticus was made.

Epilepsy surgery in bilateral Sturge-Weber syndrome.

Two infants with severe drug refractory focal epilepsy caused by Sturge-Weber syndrome and extensive cerebral leptomeningeal angiomatosis were referred for preoperative video-electroencephalographic evaluation. Brain imaging with computed tomography and gadolinium-enhanced magnetic resonance imaging demonstrated bilateral disease in both children with a predominance of involvement of one hemisphere. Clinical examination and neurophysiology with ictal video recording demonstrated epileptogenesis from one hemisphere.