Subacute sclerosing panencephalitis (SSPE) [ Definition: a let | 👼Pediatrics. Books,Videos, MCQs...

Subacute sclerosing panencephalitis (SSPE) [
Definition: a lethal, generalized, demyelinating inflammation of the brain caused by persistent measles virus infection [8][9]
Epidemiology
Primarily affects males between 8 and 11 years of age 
Usually develops ≥ 7 years after measles infection
Clinical presentation: characterized by four clinical stages
Stage I: dementia, personality changes
Stage II: epilepsy, myoclonus, autonomic dysfunction
Stage III: decerebration, spasticity, extrapyramidal symptoms
Stage IV: vegetative state, autonomic failure
Diagnosis
Electroencephalography
Paroxysmal delta waves (very slow, 1–3/sec)
Periodic sharp and slow wave complexes
Cerebrospinal fluid: ↑ anti-measles IgG
Prognosis: SSPE leads to death within 1–3 years of diagnosis)