Systematic assessment of psychopathology in sporadic Creutzfeldt-Jakob disease and possible associations with genetic polymorphism and clinical variables

Introduction: Psychiatric symptoms are regarded as a main and early feature of the new variant of Creutzfeldt-Jakob disease (vCJD). In contrast, few studies address psychiatric findings in sporadic Creutzfeldt-Jakob disease (CJD). Methods: We present two studies with 50 autopsy-proven (definite) and 49 probable cases of sporadic CJD assessing 12 psychiatric symptoms along with clinical data as well as genetic polymorphisms. In 72 of these patients (definite: 39; probable: 33) the evaluation was based on medical records held in the CJD Surveillance Unit at the University of Goettingen. 27 patients (definite: 11; probable: 16) were investigated by interviewing their relatives with the Neuropsychiatric Inventory. Results and conclusions: Our findings provide a detailed description of the psychopathology in the course of sporadic CJD compared to other dementias like Alzheimer`s disease, Lewy body dementia and dementia with Parkinson`s disease. Correlations between the different psychiatric symptoms and other clinical features such as age at onset, duration of illness and neurological symptoms are presented. The results elucidate the questions whether specific psychiatric symptoms in sporadic CJD could be related to genotype and gender or may be useful in the differential diagnosis of the disease.