A Case of Jakob-Creutzfeldt Disease*†

Abstract

The disease or syndrome first described by Creutzfeldt in 1920 (1) and further elucidated by Jakob in 1921 (2) is defined by clinical and pathological criteria. Clinically the disease is characterized by a subacute progressive presenile dementia with or without associated tremors, abnormal postures, spasticity, rigidity, and amyotrophy. The youngest reported cases were those of Stender (3), whose patient was 21 years old and Creutzfeldt (1), whose patient was 23 years old; the oldest reported case was that of a 60 year old patient seen by McMenemey and Pollack (4). The most frequent onset appears to be in the fifth decade. Most cases are sporadic, but a family with 5 affected members in 3 generations has been reported (5). The sexes are equally affected. The process has been invariably fatal. The average life span of the victims after onset of symptomatology has been 15 months, but cases as short as 3 months and as long as 44 months have been reported (5, 6). Frequently the onset is insidious and the initial diagnosis is that of a functional disorder. As the dementia progresses the syndrome becomes clearly organic in nature. The patient often passes through a period of agitation following which marked apathy supervenes, and the dementing process becomes more severe. Meanwhile, the development of difficulty in walking, weakness, tremors, rigidity, and other motor manifestations indicate the unusual nature of the neuropathological process. Pathologically the disease is characterized by the non-specific degeneration of neuronal bodies in the cerebral cortex and to a variable extent in other nuclear masses, particularly those serving motor functions. There is relative preservation of nerve cell processes and of myelin; and there is a reactive hypertrophy of astroglial cells. Nerve cell degeneration occurs without inflammatory reaction or the development of phagocytic activity. No specific histological or histochem-