Rapidly deteriorating speech and language in a case of probable sporadic Creutzfeldt‐Jakob disease

Abstract

Background: Language disorders are common features of the dementias but their use as diagnostic aids has probably been under utilised. Patterns of language disturbance tend to differ according to the cause of dementia. Identifying the type of language disturbance may assist in the diagnosis of the dementia. Furthermore, when a patient presents with unusual patterns of language disturbance, a detailed description of the presentation and the evolution of the language difficulties may aid in the diagnosis of similar cases in the future. We describe a 68‐year‐old man who presented to a university teaching hospital with a rapid onset of unusual language disturbance and concurrent cognitive deterioration. A diagnosis of probable sporadic Creutzfeldt‐Jakob disease (CJD) was made with the aid of serial EEGs and CSF analysis. Aims: This article aims to provide a summary of some of the typical language presentations documented in other causes of dementia and in other reported cases of sporadic CJD, to provide a description of the patterns of language disturbance present in our patient with probable sporadic CJD, and to compare these with the pattern of language disturbance reported in other cases of CJD. Main Contribution: A summary of some of the patterns of language change documented in the dementias is provided and a detailed description of rapidly progressing language disturbance in a case of probable sporadic CJD, which differs from those already documented in the literature. This language disturbance was characterised by an initial, relatively fluent, aphasia‐like language disturbance affecting mainly semantic ability and comprehension, with a rapid progression (on a daily basis) through to an eventual global language and cognitive failure. The unusual patterns of language change in this case are described and comparisons are made to the reported patterns of language change in other cases of sporadic CJD. The need for detailed description of language change in the dementias is highlighted. Conclusions: There are very few documented cases of rapid deterioration in language function in probable sporadic CJD. Of those documented, the type and speed of progression of the language disturbance differs greatly. We present this case of probable CJD to provide further description of language change which might highlight the need to add to the sparse number of reported cases in order to help with assessment and management in the future.