October 1979

The Variable Natural History of Subacute Sclerosing PanencephalitisA Study of 118 Cases From the Middle East

Author Affiliations

From the School of Medicine (Dr Risk) and the Section of Neurological Surgery (Dr Haddad), American University Medical Center, Beirut, Lebanon. Dr Risk is now with the University of Iowa Hospitals and Clinics, Iowa City. Presented in part at the First International Congress of Child Neurology, Toronto, Oct 6, 1975.

Arch Neurol. 1979;36(10):610-614. doi:10.1001/archneur.1979.00500460044004

• A variable natural history was found in a standardized follow-up study of 118 patients with subacute sclerosing panencephalitis (SSPE). Only 20% followed the sequence: behavior change, mental deterioration, periodic attacks, severe debility, and death within a year. Six stages were identified: O, subtle psychointellectual symptoms; 1, obvious psychointellectual and neurological changes; 2, stereotyped attacks; 3, vegetative psychomotor condition; 4, improvement; 5, relapse. Illness began in stage 0 for 32%; 1, 36%; 2,19%; 1 and 2 simultaneously, 13%. In early development, 77% were hyperactive, and 27% had psychointellectual difficulties one to five years prior to obvious illness. The average duration of each stage was as follows: stage O, two years; 1, 2.5 months; 2, 7.5 months; 3, 5.5 months; 4, 3.5 years; 5, 1.5 years. Noteworthy improvements and plateaus occurred in more than half of the patients. In stage 4, improvement was long-term and substantial for 5% and modest for 18%. Survival followed an exponential curve with an average half-life of 1.8 years; 41% of the patients survived beyond two years. Half of the patients passed through neither stage 0 nor stage 4; their half-life was 0.7 years. The remainder had a half-life of 3.0 years.