Verbal perseveration as the initial symptom in a case of Creutzfeldt‐Jakob disease

Abstract

Background: In the literature on cortical degenerative processes and primary progressive aphasia, language disorders have been discussed as the initial sign of higher cortical dysfunction. Verbal perseveration has also been cited to precede the occurrence of other cortical signs. In this paper we present language data from an autopsy-confirmed case of Creutzfeldt-Jakob disease (CJD) who exhibited profound verbal perseveration as the initial symptom. Aims: This case report aims to provide a description of the extent and pattern of verbal perseveration in a client with the rapidly progressing fatal CJD. Language test data and medical data, i.e., EEG and neuropathology-which confirm the diagnosis of CJD-are presented.Methods & Procedures: Language testing was performed with our patient to determine the nature of the language disorder at a time when the diagnosis of CJD had not yet been made. The tape-recorded sessions were transcribed and the language data analysed.Outcomes & Results: The pervasive perseverative behaviour mirrors the underlying language-processing difficulties and the magnitude of the progression of the disease process. Perseverative errors account for between 87% and 95% of the languageprocessing errors on the various language tasks. In general, the first items of each task were responded to correctly, indicating that our client was trying to meet the various test demands and at the time of testing was still able to process language, albeit in a very restricted manner. Conclusions: The rapid deterioration of language functions in our case and in other reported cases necessitates acting quickly without delay. Immediate recognition and analysis of the language disturbance and verbal perseverative behaviour may provide relevant information for differentially diagnosing clients presenting with various cortical degenerative diseases at an early stage.

Isolated language impairment as the primary presentation of sporadic Creutzfeldt Jakob Disease

“…Illness progression usually involved more than one aspect of language and other cortical functions before appearance of other features such as ataxia or myoclonus. Asymmetrical limb symptoms, with earlier affection of the right side was seen in three of our cases (cases 6,10 and 12) and two of the patients in literature, supporting earlier affection of the left hemisphere in these patients.…”