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Subacute sclerosing panencephalitis
Other Resources UpToDate PubMed

Subacute sclerosing panencephalitis

Contributors: Andrea Wasilewski MD, Jamie Adams MD, Richard L. Barbano MD, PhD
Other Resources UpToDate PubMed


Subacute sclerosing panencephalitis (SSPE) is a progressive neuropsychiatric disorder occurring after natural infection with measles virus. There is typically a history of measles infection at an early age followed by an asymptomatic period, with onset of symptoms starting 7-10 years after primary measles infection. The pathogenesis is unknown but is thought to be related to infection with a variant of measles virus.

The disorder is characterized by development of personality and behavioral changes, progressive cognitive decline, and dementia with seizures, myoclonus, spasticity, ataxia, and vision loss. The final stages of the disease progress to flaccidity, decorticate rigidity, autonomic dysfunction, and eventual coma and death. Disease course is typically progressive but can fluctuate, with some patients having relapsing and remitting symptoms. Patients have elevated antimeasles antibody concentrations in serum and cerebral spinal fluid. Prognosis is poor, and death usually occurs within 3 years of symptom onset.


A81.1 – Subacute sclerosing panencephalitis

192685000 – Subacute sclerosing panencephalitis

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Differential Diagnosis & Pitfalls

  • Anti-N-methyl-D-aspartate receptor antibody syndrome
  • Wilson disease
  • Progressive myoclonic epilepsy (eg, neuronal ceroid lipofuscinosis, Lafora disease, Unverricht-Lundborg disease, sialidosis, myoclonus epilepsy and ragged red fibers [MERRF])
  • Dentatorubropallidoluysian atrophy (DRPLA)
  • Landau-Kleffner syndrome
  • Gaucher disease type 2
  • Drug toxicity (eg, Drug-induced ataxia)
  • Progressive multifocal leukoencephalopathy
  • Inclusion body encephalitis
  • Progressive rubella panencephalitis

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Last Reviewed:06/19/2019
Last Updated:06/20/2019
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Subacute sclerosing panencephalitis
A medical illustration showing key findings of Subacute sclerosing panencephalitis (Early Stage) : Personality changes, Cognitive decline
Copyright © 2024 VisualDx®. All rights reserved.