subacute sclerosing panencephalitis
Also written as: SSPE — subacute sclerosing panencephalitis
A progressive demyelinating disease caused by persistent measles virus infection of the CNS.
Full Definition
Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, and ultimately fatal demyelinating disease of the central nervous system caused by persistent infection with a defective measles virus. The condition typically develops years after initial measles infection and is characterized by chronic inflammation, demyelination, and neuronal loss. Histologically, SSPE shows characteristic intranuclear and intracytoplasmic inclusion bodies (Cowdry type A inclusions) in neurons and glial cells, along with perivascular lymphocytic infiltration and reactive gliosis. The disease predominantly affects children and young adults, causing progressive neurological deterioration including cognitive decline, seizures, and movement disorders.
Usage
Usage note: Note association with prior measles infection and Cowdry inclusions.
In Context
- "The brain biopsy confirmed subacute sclerosing panencephalitis with pathognomonic Cowdry inclusions." — Pediatric neuropathology report
- "Elevated measles antibody titers supported the diagnosis of subacute sclerosing panencephalitis." — Clinical correlation note