Adult-onset subacute sclerosing panencephalitis

Abstract

Subacute sclerosing panencephalitis (SSPE) is a lethal slow viral disease of the central nervous system caused by a defective measles virus. The onset is mostly in childhood, manifesting clinically as decline in academic performance, behavioural changes, motor dysfunction and myoclonus. Adult-onset SSPE is rare and can present as rapidly progressive dementia. We present a young man of Indian origin with adult-onset SSPE with rapidly progressive dementia but no localising neurological signs. The diagnostic clues were parieto-occipital white matter changes on MR brain scan and history of childhood fever with rash. High titres of antimeasles antibody in cerebrospinal fluid confirmed the diagnosis. The long latency from primary measles virus infection to symptom onset can be misleading in adults. SSPE should be considered in adults with dementia, especially in tropical countries where vaccination coverage is suboptimal.

The spectrum of psychiatric manifestations in subacute sclerosing panencephalitis: A systematic review of published case reports and case series

“…Studies were considered if they fulfilled the following criteria: (a) they were case reports or case series; (b) they described cases of psychiatric manifestations in a case of SSPE; (c) cohort studies were included only if, individual patient data were available; and (d) they comprehensively described the psychiatric manifestations in confirmed SSPE cases. Acute fulminant SSPE refers to a rapidly progressing condition where a patient either becomes akinetic mute or dies within 6 months of disease onset 2 …”

“…Acute fulminant SSPE refers to a rapidly progressing condition where a patient either becomes akinetic mute or dies within 6 months of disease onset. 2…”

“…A definitive diagnosis needs the demonstration of elevated measles antibody titers in the cerebrospinal fluid (CSF). 1,2 Infrequently, psychiatric manifestations (like mania, psychosis, catatonia, and malingering) have also been reported in many SSPE patients. In this systematic review, we evaluated the spectrum of psychiatric manifestations of SSPE.…”