Hemiconvulsion-hemiplegia-epilepsy syndrome in South African children: insights from a retrospective case series.

INTRODUCTION: Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is a recognized sequel of febrile partial status in children younger than 4 years. OBJECTIVE: To describe the clinical features, neuroradiology and outcome in 8 South African children with HHE syndrome. METHOD: A retrospective descriptive study of 8 consecutive cases of HHE syndrome presenting to tertiary hospitals in the Western Cape over a 2 year period. RESULTS: The median age of onset of convulsive status was 16 months (range: 9-36 months). Gender distribution was equal. The duration of the initial episode of status exceeded 2 h in all children. All children were reported to have been developmentally normal prior to the onset of the first seizure and none previously suffered seizures or had a family history of febrile seizures and epilepsy. In 7 of the 8 cases the initial seizure was not associated with fever or preceding illness. Imaging demonstrated cerebral hemiatrophy in all and additional crossed cerebellar atrophy in 2 children. Moderate to severe intellectual disability ensued in the majority of children. The severity of the intellectual disability correlated with the degree of the motor deficit and occurred irrespective of the cerebral hemisphere involved. CONCLUSION: In contrast to developed countries, HHE syndrome is still prevalent in South Africa. The neurological morbidity in South African children is significant and highlights the need for improved emergency care of status epilepticus.

Progressive multifocal leukoencephalopathy after initiation of highly active antiretroviral therapy in a child with advanced human immunodeficiency virus infection: a case of immune reconstitution inflammatory syndrome.

Five weeks after commencing highly active antiretroviral therapy, a 12-year-old boy with advanced human immunodeficiency virus infection presented with acute cerebellar dysfunction and hemiparesis. Progressive multifocal leukoencephalopathy was diagnosed by cerebrospinal fluid polymerase chain reaction for JC virus and magnetic resonance imaging of the brain. Rapid and sustained improvement followed a prolonged course of glucocorticosteroid therapy while continuing antiretrovirals.

Hydatid disease of the spine in South African children.

We present three patients with neurologic complications of the spine from hydatid disease. The first was a 6-year-old girl with lower limb paralysis evolving over 2 weeks. Neuroimaging revealed a cystic mass compressing the spinal cord at the level of T8 and extending from the vertebral body. She underwent surgical decompression. Histopathologic examination confirmed hydatid disease. At 6-month follow-up, functional improvement had occurred with full ambulation. She continues on long-term albendazole therapy. Two other patients are described, one with primary spinal disease and the other with cerebral disease and secondary seeding to the spine. Spinal hydatid disease is a rarity, even more so in children. Although secondary disease, primarily affecting bone, carries a poorer long-term outlook, the first patient made a dramatic recovery and has raised therapeutic dilemmas as to the total duration of continuing albendazole therapy. The literature documents some 37 reports, mostly in adults. Considering the frequency of hydatid disease in South Africa, the incidence in our population could be under-recorded. Unless the clinician includes spinal hydatid disease, in endemic areas, as part of the differential list for paralysis and considers performing neuroimaging, this potentially treatable diagnosis will be missed.