NR AOUZ
AU Koertner,K.; Bartl,M.; Jastrow,U.; Mollenhauer,B.; Poser,S.; Zerr,I.
TI Utility of DSM-IV and ICD-10 criteria for diagnosis of dementia in sporadic CJD
QU International Conference - Prion diseases: from basic research to intervention concepts - TSE-Forum, 08.10.-10.10.2003, Gasteig, München - Poster session - DG-58
PT Konferenz-Poster
AB Presence of dementia is currently a requisite for the clinical diagnosis of CJD. However, studies to assess the actual prevalence and features of dementia in CJD are lacking. Data from 39 autopsy-proven and 33 probable CJD cases were studied in a retrospective fashion. Presence of dementia based on DSM-IV and ICD-10 criteria as well as neurologic and psychiatric features were assessed for each patient over the course of illness. 55 patients (76%) fulfilled DSM-IV criteria for diagnosis of dementia, whereas only 34 (47%) could be classified according to ICD-10 (despite omission of the 6 month duration limit requested for diagnosis). In 17 patients (24%) diagnosis of dementia was not possible by the use of either DSM-IV or ICD-10. These patients suffered more often from psychotic and sensory symptoms, mutism and disorders of consciousness. Furthermore, female patients and Valin homozygotes displayed less often symptoms of dementia and there were no heterozygotes in the group without dementia diagnosis. Age at onset, duration of illness and the typical neurological features of CJD (cerebellar, extrapyramidal, pyramidal, myoclonus, akinetic mutism) were similar among all patients. Overall, at disease onset neurological symptoms were more prevalent than symptoms of dementia, the former being the most frequent reason for hospital admission. Following single case analysis psychiatric symptoms (psychosis: N=5, apathy: N=2, anxiety: N=1), debilitating neurological signs (N=5) disturbed consciousness (N=3) and aphasia (N=1) prevented testing of dementia. Our findings suggest that dementia in CJD has several distinguishing features and that modified criteria for its classification might be necessary.This could prove of practical importance for early disease recognition.Further studies are necessary, in order to determine the prevalence of psychiatric symptoms in sporadic CJD. Also,the relationship between features of dementia and genotype and gender should be investigated.
AD Katrin Koertner, Mario Bartl, Ute Jastrow, Brit Mollenhauer, Sigrid Poser, Inga Zerr, University of Göttingen, Department of Neurology, Germany
SP englisch
PO Deutschland