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Encephalitis lethargica in 5 South African children

Ronald van Toorn1, Johan F Schoeman

  • 1Faculty 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

European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society
|April 22, 2008
PubMed

Insights

Five South African children experienced acute encephalopathic illness, diagnosed as encephalitis lethargica. While all survived, three developed learning disabilities, highlighting the need for early recognition and cognitive rehabilitation.

Area of Science:

  • Neurology
  • Pediatrics
  • Infectious Diseases

Background:

  • Encephalitis lethargica (EL) is a rare neurological disorder characterized by acute onset of encephalopathy, oculogyric crises, and parkinsonism.
  • Sporadic cases of EL in children are infrequently reported, particularly in sub-Saharan Africa.

Observation:

  • This study details five pediatric cases of sporadic encephalitis lethargica in South Africa between 2002 and 2006.
  • All children presented with acute encephalopathic illness, sleep disturbances, extrapyramidal signs, and neuropsychiatric symptoms.
  • Neuroimaging was unremarkable in all cases, aiding in the exclusion of other causes of acute childhood encephalopathy.

Findings:

  • Diagnosis was established based on clinical presentation and the exclusion of infectious, biochemical, metabolic, and autoimmune etiologies.
  • All five children survived the acute illness, but three experienced significant learning disabilities.
  • Cognitive rehabilitation was necessary for all affected children post-recovery.

Implications:

  • Encephalitis lethargica occurs in South African children and should be considered in the differential diagnosis of acute encephalopathic presentations.
  • Early recognition is crucial for appropriate parental counseling regarding the typically protracted course and potential for long-term cognitive deficits.
  • The findings underscore the importance of comprehensive exclusion of other causes when diagnosing EL in pediatric populations.

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