S.S.P.E.: some clues to pathogenesis from epidemiological data.

This study is a preliminary report of the national registry of S.S.P.E. cases started in 1987 which continue early epidemiological inquiries concerning incidence in Romania during the decade 1978-1987. The analysis pointed out the high incidence of this disease: a mean incidence rate of 6.35 cases per year per million total population in the last three years. Since 1979 there has been in Romania a sharp decline in the incidence of measles induced by the initiation of compulsory vaccination. As yet this decline is not reflected in S.S.P.E. incidence. The male/female ratio varied between 1.78-3.3 according to the year of observation. The mean age at S.S.P.E. onset increased from 6-7 years at the beginning of the interval to 9.63 years in 1987. The S.S.P.E. incidence is unevenly distributed between rural and urban area and clusters of S.S.P.E. cases were observed in certain countries. Other categories of information which may be relevant to pathogenesis are discussed: immunological history, previous hospitalization and illnesses, family history, recent health problems prior to S.S.P.E. onset, education, vacations, food habits and animal-exposure history.

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.