Blindness, dancing extremities, and corpus callosum and brain stem involvement: an unusual presentation of fulminant subacute sclerosing panencephalitis

J Child Neurol. 2015 Jan;30(1):87-90. doi: 10.1177/0883073813520498. Epub 2014 Apr 2.

Abstract

A 4-year-old girl presented with acute visual loss followed 2 weeks later with loss of speech and audition, fulminant neuroregression, and choreo-athetoid movements of extremities. Fundus showed bilateral chorioretinitis. Electroencephalography showed periodic complexes. Measles antibody titers were elevated in both serum and cerebrospinal fluid, consistent with subacute sclerosing panencephalitis. Neuroimaging showed discontiguous involvement of splenium of the corpus callosum and ventral pons with sparing of cortical white matter. Our case highlights the atypical clinical and radiologic presentations of subacute sclerosing panencephalitis. Pediatricians need to be aware that necrotizing chorioretinitis in a child and/or atypical brain stem changes could be the heralding feature of this condition in endemic countries.

Keywords: acute vision loss; chorioretinitis; pons; splenium; subacute sclerosing panencephalitis.

Publication types

  • Case Reports

MeSH terms

  • Blindness / etiology*
  • Brain Stem / pathology*
  • Child, Preschool
  • Chorea / etiology*
  • Corpus Callosum / pathology*
  • Extremities / physiopathology*
  • Female
  • Humans
  • Magnetic Resonance Imaging
  • Retinal Pigment Epithelium / pathology
  • Subacute Sclerosing Panencephalitis / complications*