CREUTZFELDT-JAKOB disease usually occurs as a sporadic condition, although Jakob1 did consider the possibility of its familial occurrence. Kirschbaum,2 Meggendorfer,3 Stender,4 and Jacob et al5 described the clinicopathologic and genetic aspects of the first reported family afflicted by this disease, the Backer family, and Davison6 and Davison and Rabiner7 reported the occurrence of the disease in three siblings. Some authorities have questioned whether Creutzfeldt-Jakob disease should be considered a nosologic entity.8 A historical survey and a discussion of the clinical aspects of this disease has been published.9 It 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.