Subacute sclerosing panencephalitis. A review and a case report
Wulff, C.H.
Ugeskrift for Laeger 143(51): 3457-3460
1981
ISSN/ISBN: 0041-5782 PMID: 7336504 Document Number: 181797
Subacute sclerosing panencephalitis (SSPE) is a slowly progressive and fatal encephalitis affecting children mainly 5-15 yr of age. The disease starts with personality changes and behavior disturbances and progresses to dementia with myoclonic jerks, walking difficulties, drop attacks and loss of higher cortical functions. The terminal stage is characterized by rigidity progressing to a decerebrate stage. SSPE develops after previous measles infection, apparently because of a failure to eliminate the measles virus. The characteristic periodic complexes in the EEG are diagnostic together with a high titer of measles antibodies in serum and presence of measles antibodies in CSF. Although it seems easy to make the correct diagnosis, it may be difficult in the clinic because the disease is very rare. Various psychiatric disorders in children are important for differential diagnosis. EEG recording and estimation of the concentration of measles antibodies should be undertaken in all children in whom insidious dementia is encountered even when no myoclonic jerks or grand mal seizures are present.