Amyotrophic lateral sclerosis with progressive dementia

Abstract
A case with features of amyotrophic lateral sclerosis (ALS) and Creutzfeldt-Jakob''s disease is reported. The patient presented a clinical picture of ALS with atypical mental symptoms. The pathology showed changes characteristic of ALS and, in addition, bitemporal cortical atrophy, status spongiosus, and astroglial proliferation, as seen in the Creutzfeld-Jakob syndrome. The case is discussed within the framework of a continuum of allied neurologic disorders to which it appears to belong. The concept of the continuum is reviewed and further elaborated.