Not all these features are present in any one illness

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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 -ยป dementia. Seizures, ataxia

Dystonia.

Progression to death

10% fulminant course ^ 80%

months

10% - in this group, periods of stabilisation and even improvement may transiently occur.

years years

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.

Investigations

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.

Pathology

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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