Prions and Neurodegenerative Diseases

Abstract

CREUTZFELDT–JAKOB disease (CJD), kuru, and the Gerstmann–Sträussler syndrome are human neurodegenerative diseases that can be transmitted experimentally to animals. In 1920, Creutzfeldt described a progressive dementing illness in a 22-year-old woman. The following year, Jakob described four older patients with a clinically similar presentation and course.¹ During the ensuing four decades, numerous cases of CJD were described clinically and pathologically. Although most cases of CJD present with a progressive dementia characterized initially by loss of memory, diminished intellect, and poor judgment, a few cases present as progressive cerebellar syndromes, with diminished coordination, tremor, and ataxia. Patients with the ataxic form . . .