Blindness, dancing extremities, and corpus callosum and brain stem involvement: an unusual presentation of fulminant subacute sclerosing panencephalitis.
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.
Pediatric Neurology and Neurodevelopment unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India doctorpratibhasinghi@gmail.com.
Pratibha
P
Singhi
Pediatric Neurology and Neurodevelopment unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Arushi Gahlot
AG
Saini
Pediatric Neurology and Neurodevelopment unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Naveen
N
Sankhyan
Department of Ophthalmology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Pankaj
P
Gupta
Department of Radiodiagnosis, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Sameer
S
Vyas
D001766:Blindness / Q000209:etiology*; D001933:Brain Stem / Q000473:pathology*; D002675:Child, Preschool; D002819:Chorea / Q000209:etiology*; D003337:Corpus Callosum / Q000473:pathology*; D005121:Extremities / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D055213:Retinal Pigment Epithelium / Q000473:pathology; D013344:Subacute Sclerosing Panencephalitis / Q000150:complications*
acute vision loss; chorioretinitis; pons; splenium; subacute sclerosing panencephalitis