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01-12-2016 | Neurology and Preclinical Neurological Studies - Original Article

Clinical radiological correlation in E200K familial Creutzfeldt–Jakob disease

Authors: Oren S. Cohen, Joab Chapman, Amos D. Korczyn, Oliver L. Siaw, Naama Warman-Alaluf, Zeev Nitsan, Shmuel Appel, Esther Kahana, Hanna Rosenmann, Chen Hoffmann

Published in: Journal of Neural Transmission | Issue 12/2016

Abstract

The use of diffusion MRI improved the accuracy of diagnosis in Creutzfeldt–Jakob disease (CJD) and expanded our knowledge of the changes occurring in the brain during the disease. The aim of this study was to test whether in patients with E200K familial CJD (fCJD) the clinical severity correlates with the disease burden as reflected by the extent of cortical involvement in DWI MRI. Consecutive fCJD patients were examined by a neurologist who performed several tests including the CJD neurological scale (CJD-NS), MiniMental status examination (MMSE), Frontal Assessment Battery (FAB), NIH Stroke Scale (NIHSS), and the expanded disability status scale (EDSS). A simultaneously acquired MRI was analyzed by measuring the extent of cortical involvement in the DWI axial sequence. Correlations were tested for using Pearson test. Fifty-two fCJD patients (35 males, mean age 59.4 ± 5.7 years) were recruited to the study. Significant negative correlation was found between the extent of cortical involvement and the cognitive performance of the patients as reflected by their MMSE and FAB scores. In addition, a significant positive correlation was found between the MRI and the clinical disease severity scales CJD-NS and EDSS. The correlation between clinical scales of severity and cognitive dysfunction and the disease burden confirms the reliability of the CJD-NS scale. Further studies are warranted to examine whether MRI may serve not only for diagnosis but also as a biomarker for follow-up of disease progression and the efficacy of potential treatments.

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Title: Clinical radiological correlation in E200K familial Creutzfeldt–Jakob disease
Authors: Oren S. Cohen
Joab Chapman
Amos D. Korczyn
Oliver L. Siaw
Naama Warman-Alaluf
Zeev Nitsan
Shmuel Appel
Esther Kahana
Hanna Rosenmann
Chen Hoffmann
Publication date: 01-12-2016
Publisher: Springer Vienna
Published in: Journal of Neural Transmission / Issue 12/2016
Print ISSN: 0300-9564
Electronic ISSN: 1435-1463
DOI: https://doi.org/10.1007/s00702-016-1617-3

At a glance: The STEP trials

Springer Medicine

Clinical radiological correlation in E200K familial Creutzfeldt–Jakob disease

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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