Neurology · MU Sofia

48. Subacute sclerosing panencephalitis

Covers subacute sclerosing panencephalitis as a rare, progressive CNS disease caused by persistent mutant measles virus, linking it to childhood measles infection and to demyelination, neuronal loss and gliosis. It walks through the four clinical stages in order, from behavioural change to the terminal stage, then lists cardinal and examination findings, diagnostic features on EEG, CSF and MRI, differential diagnoses, prognosis and symptomatic treatment. A staged progression table is not included, but the stages are set out in sequence with a picture accompanying the diagnosis section.

Inside: 1 section · 1 labelled picture · 15 flashcards · 2 min

Topic 48 of 88 in the MU Sofia syllabus for Neurology (Medicine, Year 4). The free AceMed note covers it in 1 section, about 276 words (2 min of reading), with 15 flashcards.

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  1. Subacute sclerosing panencephalitis

Subacute sclerosing panencephalitis

Definition: Subacute sclerosing panencephalitis (SSPE) is a rare, progressive, chronic degenerative disease of the CNS caused by persistent mutant measles virus infection, leading to widespread inflammation and demyelination of brain tissue.

Etiology & Pathogenesis

  • Caused by measles virus (paramyxovirus)
  • Occurs years after primary measles infection (usually childhood infection)
  • Defective viral particles persist in CNS → slow progressive neuronal damage
  • Leads to: Demyelination.Neuronal loss. Gliosis (“sclerosis”)

Epidemiology

  • Children and adolescents (typically 5–15 years)
  • History of measles infection at young age (often before age 2)
  • More common in unvaccinated populations

Clinical Course (Staged Progression)

Stage I – Behavioral/Cognitive changes: Subtle school performance decline. Personality changes. Irritability. Memory impairment

Stage II – Myoclonic stage (classic): Myoclonic jerks (hallmark). Seizures. Visual disturbances. Progressive cognitive decline

Stage III – Neurological deterioration: Severe dementia. Spasticity (UMN signs). Motor impairment. Loss of speech

Stage IV – Terminal stage: Vegetative state. Decerebrate rigidity. Death

Key Clinical Features

  • Cardinal signs: Progressive dementia. Myoclonic jerks.Seizures. Visual impairment
  • Neurological signs: UMN signs (hyperreflexia, Babinski). Extrapyramidal features (rigidity)

Examination Findings: Cognitive decline (global dementia). Myoclonus triggered by sensory stimuli. Spastic quadriparesis. Hyperreflexia. Visual loss (cortical involvement)

Diagnosis

  • EEG (very characteristic): Periodic, stereotyped high-voltage slow-wave complexes
  • CSF: Mild ↑ protein. Normal or mild lymphocytosis. Elevated anti-measles antibodies (diagnostic)
  • MRI: White matter demyelination. Cortical and subcortical atrophy

Differential Diagnosis

1. Viral encephalitis: Acute onset.Fever present. CSF inflammatory

2. Epilepsy: No progressive cognitive decline

3. Huntington disease: Chorea + psychiatric symptoms. Genetic, no infection history

4. Metabolic leukodystrophies: Early childhood onset. No measles history

Prognosis: Progressive and fatal. Death usually occurs within 1–3 years after onset. No curative treatment

Treatment: No definitive cure

  • Symptomatic: Anticonvulsants (for myoclonus/seizures).
  • Experimental therapies (limited benefit)

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