A Corticobasal Syndrome Variant of Familial Creutzfeldt-Jakob Disease with Stroke-Like Onset
Joint Authors
Necpal, Jan
Stelzer, Martin
Koščová, Silvia
Patarák, Michal
Source
Case Reports in Neurological Medicine
Issue
Vol. 2016, Issue 2016 (31 Dec. 2016), pp.1-3, 3 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2016-10-10
Country of Publication
Egypt
No. of Pages
3
Main Subjects
Abstract EN
Creutzfeldt-Jakob disease (CJD) is an untreatable rare human prion disease characterized by rapidly progressive dementia along with various neurological features, including myoclonus and sometimes other movement disorders.
The clinical course is typically insidious and rapid, leading to an early death.
In general, the most common form is sporadic CJD; however, Slovakia is typical for a high percentage of genetic cases.
We present an unusual case report of a 65-year-old man with a sudden, stroke-like onset of motor aphasia with right-sided levodopa unresponsive parkinsonism, alien hand, and other characteristic features of corticobasal syndrome (CBS), with rapid deterioration and death on the 32nd day of the disease.
Various neurodegenerative disorders are manifested with CBS as a clinical phenotype, including corticobasal degeneration (CBD), progressive supranuclear palsy, Alzheimer’s disease, and CJD.
In our patient, mutation E200K and M129M polymorphism of the PRNP gene and typical immunohistochemical findings pointed to a diagnosis of CJD.
The patient’s mother died of CJD many years ago.
Several CBS-CJD cases were described, but the atypical stroke-like onset of CBS-CJD, an extremely rare presentation of CJD, makes our case unique worldwide.
American Psychological Association (APA)
Necpal, Jan& Stelzer, Martin& Koščová, Silvia& Patarák, Michal. 2016. A Corticobasal Syndrome Variant of Familial Creutzfeldt-Jakob Disease with Stroke-Like Onset. Case Reports in Neurological Medicine،Vol. 2016, no. 2016, pp.1-3.
https://search.emarefa.net/detail/BIM-1101276
Modern Language Association (MLA)
Necpal, Jan…[et al.]. A Corticobasal Syndrome Variant of Familial Creutzfeldt-Jakob Disease with Stroke-Like Onset. Case Reports in Neurological Medicine No. 2016 (2016), pp.1-3.
https://search.emarefa.net/detail/BIM-1101276
American Medical Association (AMA)
Necpal, Jan& Stelzer, Martin& Koščová, Silvia& Patarák, Michal. A Corticobasal Syndrome Variant of Familial Creutzfeldt-Jakob Disease with Stroke-Like Onset. Case Reports in Neurological Medicine. 2016. Vol. 2016, no. 2016, pp.1-3.
https://search.emarefa.net/detail/BIM-1101276
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1101276