SUBACUTE SCLEROSING PANENCEPHALITIS (SSPE)
Caused by measles-like paramyxovirus - isolated from brain biopsy.
Less common with the availability of widespread primary measles vaccination.
Clinical features: A world-wide disorder. Incidence: 1 per million per year. Onset: between ages 7-10 years.
Stage 1 Stage 2 Stage 3
Behavioural problem Chorioretinitis, Lapses into rigid
Declining school performance Myoclonic jerks comatose state.
Progression -» dementia. Seizures, ataxia
Progression to death
10% fulminant course ^ 80%
10% - in this group, periods of stabilisation and even improvement may transiently occur.
The illness may occur after measles vaccination or following clinical infection at an early age (under 2 years). Accompanying features of infection, i.e. pyrexia, leucocytosis, are absent.
CSF examination shows elevated y globulin with IgG oligoclonal bands; elevated measles antibodies (75% of total CSF IgG).
EEG - shows periodic high voltage slow wave complexes on a low voltage background trace.
Changes involve both white and grey matter, especially in the posterior hemispheres. Brain stem, cerebellum and spinal cord are also affected.
Oligodendrocytes contain eosinophilic inclusion bodies. Marked gliosis occurs with perivascular lymphocyte and plasma cell cuffing.
Treatment: There is no effective treatment. Since the introduction of measles vaccination there has been a marked reduction of SSPE.
Subacute measles encephalitis may follow measles infection in children on immunosuppressive drug treatment or with hypogammaglobulinaemia. The clinical course is different however from SSPE and EEG and CSF findings are less specific.
Blood examination shows elevated serum measles antibodies.
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