Subacute Sclerosing Panencephalitis Presenting as Choreoathetosis and Basal Ganglia Hyperintensities

Subacute sclerosing panencephalitis is a devastating neurodegenerative disorder characterized by cognitive and memory deterioration, personality changes, behavior abnormalities, and a steady motor decline. Extrapyramidal manifestations are commonly seen in the advanced stage of the disease. We discuss a 10-year-old boy with subacute sclerosing panencephalitis presenting with generalized choreoathetosis, myoclonus, and cognitive decline. He had not been immunized for measles. His elder sister had a viral exanthematous illness 4 years ago. Magnetic resonance imaging revealed bilateral, asymmetric putaminal hyperintensities. A diagnosis of subacute sclerosing panencephalitis was confirmed by periodic complexes in the electroencephalograph and elevated antimeasles antibody titers in the blood and cerebrospinal fluid (1:625). Our case highlights that atypical clinical and radiological features of subacute sclerosing panencephalitis should always be borne in mind in children from endemic areas. Generalized choreoathetosis and bilateral putaminal involvement in the index case add to the spectrum of atypical presentations of subacute sclerosing panencephalitis in children