Encephalitis lethargica in 5 South African children.
The clinical features and cognitive outcome in 5 South African childhood cases of sporadic encephalitis lethargica seen between 2002 and 2006 are discussed. All children presented with an acute encephalopathic illness complicated by sleep disturbance, extrapyramidal and neuropsychiatric symptoms. Diagnosis was based on shared clinical features with other cases described in the literature and exclusion of known infective, biochemical and metabolic causes of acute childhood encephalopathy. The negative findings on neuro-imaging in all cases strongly supported the diagnosis. All children survived but 3 cases became learning disabled and all required cognitive rehabilitation after recovery. The cases demonstrate that encephalitis lethargica does indeed occur among South African children. The condition should be considered in any previously well child that presents with an acute encephalopathic illness with prominent extrapyramidal and neuropsychiatric symptoms and negative infectious, biochemical, autoimmune, metabolic and radiologic investigations. Recognition is important as it allows counseling of parents regarding the protracted course but generally favorable outcome of the condition.
van Toorn
Ronald
R
Faculty of Health Sciences, Department of Pediatrics and Child Health, University of Stellenbosch, Tygerberg Children's Hospital, Western Cape 7550, South Africa. vtoorn@sun.ac.za
Schoeman
Johan F
JF
D000208:Acute Disease; D001480:Basal Ganglia Diseases / Q000209:etiology* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology* / Q000503:physiopathology; D003937:Diagnosis, Differential; D004671:Encephalitis, Arbovirus / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D006801:Humans; D008297:Male; D009483:Neuropsychological Tests / Q000592:standards; D020127:Recovery of Function / Q000502:physiology; D012893:Sleep Wake Disorders / Q000209:etiology* / Q000503:physiopathology; D013019:South Africa