Onset: 2 to 12 years following an episode of measles
Risk factors:
(1) measles infection before 24 months of age
(2) male gender
The patient presents with:
(1) marked behavioral and/or personality changes.
(2) declining performance in school or work
Eventually the patient develops the second stage of the disease with:
(1) myoclonic jerks
(2) seizures
(3) spasticity
(4) abnormal movements (choreoathetoid, ballistic)
(5) cerebellar ataxia
(6) chorioretinitis
(7) dementia
The third stage features:
(1) optic atrophy
(2) quadriparesis
(3) akinetic autism
(4) autonomic instability
(5) stupor and coma
Most patients will continue to deteriorate and die, while a small number will recover spontaneously.
Poor prognostic findings:
(1) acute course
(2) elevated intracranial pressure
Laboratory findings:
(1) high levels of IgG and IgM measles specific antibody in the serum
(2) high levels of measles specific antibody in the CSF
(3) oligoclonal bands in the CSF
A brain biopsy shows nuclear and cytoplasmic inclusions in neurons and glial cells. Immunofluorescence or molecular techniques can be used to demonstrate the measles virus.
