The continuous decrease in the number of SSPE annual cases ten years after compulsory anti-measles immunization.

The record of SSPE cases diagnosed immunochemically and serologically in our laboratory, standing for about 60% of the total new cases reported in our country, shows a significant decrease in the incidence in 1988-89 (from 5.21 new cases per year per million total population in 1987 to 1.82 cases in 1988). In 85% of the patients, SSPE onset occurred at the age of 10 years or more, suggesting the possibility of a primary measles infection before anti-measles immunization became compulsory. High serum and CSF anti-measles antibody titres in recently diagnosed patients show subclinical long-term courses. Further serologic tests for viruses inducing persistent infections (herpes viruses, AgHBs and HIV) do not show any difference when compared to a control group excepting an increased incidence of anti-cytomegalic titres.

Polyneuropathy with lipid deposits in Schwann cells and axonal degeneration in cerebrotendinous xanthomatosis.

The present paper is a histological, histochemical and electron microscopic study of biopsied specimens from both right and left Achilles tendon, sural nerve and gastrocnemius muscle in a case of peripheral neuropathy with decreased sensory conduction velocity within a cerebrotendinous xanthomatosis confirmed biochemically in a 29-year-old woman. The tendon specimens contained large deposits of complex, non-homogeneous lipids, distributed intra- and extracellularly. The right sural nerve specimen showed a very severe neuropathy with massive diffuse myelinated fiber loss, presence of foamy macrophages and lipid droplets in Schwann cells. Segmental de- and remyelination was noted in 17% of the teased myelinated fibers. No onion bulbs were observed. Two years later, the left sural nerve specimen revealed a mild diffuse myelinated fiber loss, a more active segmental de- and remyelination (23%) without onion bulbs, and an active regeneration. Lipid storage aspects were absent. The gastrocnemius muscle specimens exhibited slight alterations of neurogenic origin. The pathogenesis of this neuropathy is discussed.

SSPE: epidemiological and serological features in a series of cases recorded between 1975 and 1984.

A number of 632 new cases of subacute sclerosing panencephalitis (SSPE) could be detected during a 10-year survey. The continuous increase in the number of annual cases suggests a possible correlation with the changes induced in measles epidemiology by the initiation of compulsory vaccination. On the other hand, a comparison of epidemiological and serological characteristics revealed significant differences between the series of SSPE cases recorded in the period preceding and in that following measles vaccination.