NEUROLOGIC DISEASES OF MAN WITH SLOW VIRUS ETIOLOGY

Abstract

This chapter focuses on neurologic diseases of man with slow virus etiology. To date, two subacute degenerative diseases of the central nervous system of man, kuru and Creutzfeldt–Jakob disease (C-J), a presenile dementia of worldwide distribution with a basically similar lesion at the cellular level, have been shown to be caused by a very similar, very unconventional virus. Kuru is clinically characterized by cerebellar ataxia and a shivering-like tremor, which progresses to complete motor incapacity and death in about one year from onset. It has had a yearly incidence rate and prevalence ratio of about 1% of the population. During the early years of investigation, the disease was observed to affect all ages beyond infancy, being common in children of both sexes and in adult females, but rare in adult males. The Creutzfeldt–Jakob disease, one of the types of presenile dementias of man, has been successfully transmitted independently from 14 human cases to 18 chimpanzees. Incubation periods have ranged from 11 to 16 months, which is considerably shorter than those observed on primary transmission of kuru to chimpanzees.