I. SUBACUTE SCLEROSING PANENCEPHALITIS (SSPE)

1. Clinical features

a. Onset: 5-20 years of age, predominantly in males. The disease has always been preceded by an attack of measles in the distant past or, occasionally, by measles immunization.

b. Clinical presentation: personality changes, intellectual deterioration; periodic involuntary movements; decerebrate rigidity, severe dementia

c. Duration: death usually in several years

d. CSF changes: elevated gamma globulin; antibody titer elevated to measles

2. Pathological changes

a. Gross pathology: Cortical atrophy, demyelination and ventricular dilatation may all be present; changes are mild and are not diagnostic.

b. Microscopic pathology: Inclusion bodies in oligodendroglia, neurons and astrocytes; chronic inflammatory reaction (perivascular cuffing by lymphocytes and plasma cells); some neuronal loss, gliosis and demyelination. The entire brain may be involved, but lesions are often more severe in neocortex, white matter, hippocampus, thalamus, ventral brainstem. Ultrastructural changes include paramyxovirus nucleocapsid tubules characteristic of measles virus in the inclusion bodies.

This low power microscopic view shows perivascular cuffing in a case of subacute sclerosing panencephalitis, with mononuclear cells clustered around a vessel.

This high power microscopic image shows nuclear inclusions (look for cells at right side and left side) in a case of subacute sclerosing panencephalitis.

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