n South African Medical Journal - Subacute sclerosing panencephalitis still occurring in South Africa : clinicians need to remain vigilant : correspondence




: We report on another child with subacute sclerosing panencephalitis (SSPE), expanding the case series reported by Kija to six affected children from the Western Cape region of South Africa (SA) diagnosed in the past 18 months. The child presented to Red Cross War Memorial Children's Hospital, Cape Town, at 6 years and 3 months of age. She had recently returned to SA after living for several years in Zimbabwe. Her caregivers in SA had not seen her regularly, but understood that her previous health and development had been normal. She was admitted with a reported 1-day history of sudden onset weakness and abnormal movements. She had no known history of toxin exposure or prodromal symptoms prior to her presentation. She had neuro developmental regression and demonstrated emotional lability with incoherent speech and disorientation. She was not independently mobile.Her tone and power were normal and symmetrical with brisk deep tendon reflexes. Her abnormal movements were noted to be myoclonic jerks and atonic seizures which manifested as 'head nods'. She was systemically well with no evidence of sepsis, and while HIV-exposed she had tested negative. Cerebrospinal fluid (CSF) microscopy and chemistry results were normal. An electroencephalogram (EEG) was initially slow with no epilepti form activity correlating with the jerks. Owing to concern about her dramatic presentation and apparent acute-onset encephalopathy, an emergency magnetic resonance imaging (MRI) scan of the brain was performed. This showed high signal on T2 and fluid attenuation inversion recovery (FLAIR) in the posterosuperior putamina, seen to a greater extent on the left than the right (Fig. 1). On the basis of this finding and the unusual clinical phenotype, SSPE was suspected. The child's CSF measles IgG was positive, while CSF measles polymerase chain reaction (PCR) tested negative.


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