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Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships

Only a few case reports of stroke-like onset of Creutzfeldt-Jakob disease (CJD) have previously been published. We aimed to analyze the neurological, imaging, electroencephalographic (EEG), and laboratory features of patients with this very rare phenomenon. Here, we review the clinical characteristi...

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Autores principales: Balash, Yacov, Korczyn, Amos D., Khmelev, Nadejda, Eilam, Anda, Adi, Meital, Gilad, Ronit
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7959761/
https://www.ncbi.nlm.nih.gov/pubmed/33732205
http://dx.doi.org/10.3389/fneur.2021.613991
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author Balash, Yacov
Korczyn, Amos D.
Khmelev, Nadejda
Eilam, Anda
Adi, Meital
Gilad, Ronit
author_facet Balash, Yacov
Korczyn, Amos D.
Khmelev, Nadejda
Eilam, Anda
Adi, Meital
Gilad, Ronit
author_sort Balash, Yacov
collection PubMed
description Only a few case reports of stroke-like onset of Creutzfeldt-Jakob disease (CJD) have previously been published. We aimed to analyze the neurological, imaging, electroencephalographic (EEG), and laboratory features of patients with this very rare phenomenon. Here, we review the clinical characteristics, onset features, and clinical course variants of stroke-like CJD in 23 such patients. The median age of the patients was 71 years (range: 56–84 years); 12 were women. In 20 patients, CJD was sporadic. Thirteen patients developed apoplexy-like onset of symptoms, whereas the others had prodromal non-specific complaints. Most often the patients manifested with pyramidal signs (n = 13), ataxia (n = 9), and aphasia (n = 8). On MRI DWI sequence, all subjects had abnormal hyperintensities in various parts of the cerebral cortex, striatum, or thalamus, while EEG detected periodic triphasic waves only in 11. CSF 14-3-3 protein and total τ-protein were abnormal in 17 of 23 cases. All patients died, median lifespan being 2 months (range: 19 days−14 months). In conclusion, a complex of clinical, radiological, and laboratory manifestations of stroke-like onset of CJD is outlined. The clinical relationships between CJD and stroke are considered, in an attempt to highlight this rare presentation of an uncommon disease.
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spelling pubmed-79597612021-03-16 Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships Balash, Yacov Korczyn, Amos D. Khmelev, Nadejda Eilam, Anda Adi, Meital Gilad, Ronit Front Neurol Neurology Only a few case reports of stroke-like onset of Creutzfeldt-Jakob disease (CJD) have previously been published. We aimed to analyze the neurological, imaging, electroencephalographic (EEG), and laboratory features of patients with this very rare phenomenon. Here, we review the clinical characteristics, onset features, and clinical course variants of stroke-like CJD in 23 such patients. The median age of the patients was 71 years (range: 56–84 years); 12 were women. In 20 patients, CJD was sporadic. Thirteen patients developed apoplexy-like onset of symptoms, whereas the others had prodromal non-specific complaints. Most often the patients manifested with pyramidal signs (n = 13), ataxia (n = 9), and aphasia (n = 8). On MRI DWI sequence, all subjects had abnormal hyperintensities in various parts of the cerebral cortex, striatum, or thalamus, while EEG detected periodic triphasic waves only in 11. CSF 14-3-3 protein and total τ-protein were abnormal in 17 of 23 cases. All patients died, median lifespan being 2 months (range: 19 days−14 months). In conclusion, a complex of clinical, radiological, and laboratory manifestations of stroke-like onset of CJD is outlined. The clinical relationships between CJD and stroke are considered, in an attempt to highlight this rare presentation of an uncommon disease. Frontiers Media S.A. 2021-02-25 /pmc/articles/PMC7959761/ /pubmed/33732205 http://dx.doi.org/10.3389/fneur.2021.613991 Text en Copyright © 2021 Balash, Korczyn, Khmelev, Eilam, Adi and Gilad. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neurology
Balash, Yacov
Korczyn, Amos D.
Khmelev, Nadejda
Eilam, Anda
Adi, Meital
Gilad, Ronit
Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships
title Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships
title_full Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships
title_fullStr Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships
title_full_unstemmed Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships
title_short Creutzfeldt-Jakob and Vascular Brain Diseases: Their Overlap and Relationships
title_sort creutzfeldt-jakob and vascular brain diseases: their overlap and relationships
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7959761/
https://www.ncbi.nlm.nih.gov/pubmed/33732205
http://dx.doi.org/10.3389/fneur.2021.613991
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