Disease duration in E200K familial Creutzfeldt–Jakob disease is correlated with clinical, radiological, and laboratory variables
| Autor: | Esther Kahana, Oren S. Cohen, Chen Hoffmann, Shmuel Appel, Amos D. Korczyn, Joab Chapman, Hanna Rosenmann, Zeev Nitsan |
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| Rok vydání: | 2018 |
| Předmět: |
Adult
Male 0301 basic medicine medicine.medical_specialty Neurology tau Proteins Disease Creutzfeldt-Jakob Syndrome Prion Proteins 03 medical and health sciences 0302 clinical medicine Cerebrospinal fluid Neuroimaging Internal medicine mental disorders Humans Medicine Biological Psychiatry Disease burden Aged Expanded Disability Status Scale business.industry Brain Middle Aged Prognosis Magnetic Resonance Imaging Psychiatry and Mental health 030104 developmental biology Mutation Disease Progression Familial Creutzfeldt-Jakob Population study Female Neurology (clinical) business 030217 neurology & neurosurgery |
| Zdroj: | Journal of Neural Transmission. 126:607-611 |
| ISSN: | 1435-1463 0300-9564 |
| DOI: | 10.1007/s00702-018-1958-1 |
| Popis: | Previous studies have suggested that disease duration in Creutzfeldt-Jakob disease (CJD) may be related to the radiological findings or cerebrospinal fluid (CSF) tau levels; however, it is not yet established whether clinical, radiological, and laboratory findings at diagnosis can predict survival or have a prognostic value. The aim of this study was to examine whether the disease duration is correlated with clinical, radiological, and laboratory variables. The study population consisted of consecutive familial CJD (fCJD) patients that were assessed within 1 week from the diagnosis including the CJD neurological scale (CJD-NS), Minimental Status Examination, Frontal Assessment Battery, NIH Stroke Scale, and the expanded disability status scale. In addition, a single MRI study was done and measurements of the extent of the cortical and subcortical involvement were performed. CSF was examined as part of the workout, and tau levels were determined. Sixty-nine fCJD patients were included in the study (43 males, mean age 59.3 ± 8.4, range 44-79 years). The mean disease duration was 7.3 ± 6.9 months (median 5.6 months, range 2-20 months). A significant correlation was found between the disease duration and the CJD-NS, the disease burden as reflected by the degree of cortical involvement by DWI, and the CSF tau levels. The findings of the current study reveal that several findings at disease onset including the disease severity, the cortical changes, and the tau levels are each individually correlated with disease duration and can be used by the clinician as a tool to predict the disease course and prognosis. |
| Databáze: | OpenAIRE |
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