CREUTZFELDT-JAKOB disease usually occurs as a sporadic condition, although Jakob1did consider the possibility of its familial occurrence. Kirschbaum,2Meggendorfer,3Stender,4and Jacob et al5described the clinicopathologic and genetic aspects of the first reported family afflicted by this disease, the Backer family, and Davison6and Davison and Rabiner7reported the occurrence of the disease in three siblings. Some authorities have questioned whether Creutzfeldt-Jakob disease should be considered a nosologic entity.8A historical survey and a discussion of the clinical aspects of this disease has been published.9It is the purpose of this report to present the clinical and genetic findings of another family affected by this disease, comparing it with the previously reported families, and to argue in favor of a unitarian concept for the subacute spongiform encephalopathy and the "classical" form while reconsidering the "amyotrophic" form.