Adult-Onset Subacute Sclerosing Panencephalitis With a 30-Year Latent Period. (April 2020)

• Record Type: Journal Article
• Authors: Holmes, Brandon B.; Conell-Price, Jessamyn; Kreple, Collin J.; Ashraf, Davin; Betjemann, John; Rosendale, Nicole
• Is Part Of: Neurohospitalist. Volume 10:Number 2(2020)
• Page Start: 127
• Page End: 132
• Publication Date: 2020-04
• Subjects: SSPE -- subacute sclerosing panencephalitis -- encephalitis -- measles -- retinitis -- neuroinfection; Nervous system -- Diseases -- Periodicals; Neurology -- Periodicals; 616.8
• DOI: 10.1177/1941874419869713
• Languages: English
• ISSNs: 1941-8744

Abstract

Subacute sclerosing panencephalitis (SSPE) is a rare progressive neuroinfectious disease due to a late complication of the measles virus. The hallmark clinical features of this disease include behavioral changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs. The presence of characteristic high-amplitude periodic complexes on electroencephalography and raised antibody titers against measles in the cerebrospinal fluid help solidify the diagnosis. We present a case of a 40-year-old patient with SSPE who initially developed ophthalmologic manifestations 30 years after the primary measles infection. This case highlights both typical and atypical features of SSPE and provides a diagnostic framework for evaluating cases that fall outside of the standard scope of this disease.