scholarly journals Sporadic Creutzfeldt-Jakob Disease Presenting as Corticobasal Syndrome: A Rare Case

2018 ◽  
Vol 24 (2) ◽  
pp. 177-179 ◽  
Author(s):  
Murat Gültekin ◽  
Recep Baydemir ◽  
Mehmet Fatih Göl ◽  
Meral Mirza
Keyword(s):  
Rare Case ◽  
Corticobasal Syndrome ◽  
Jakob Disease

scholarly journals A Corticobasal Syndrome Variant of Familial Creutzfeldt-Jakob Disease with Stroke-Like Onset

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Ján Necpál ◽  
Martin Stelzer ◽  
Silvia Koščová ◽  
Michal Patarák
Keyword(s):  
Corticobasal Degeneration ◽  
Early Death ◽  
Prnp Gene ◽  
Corticobasal Syndrome ◽  
Progressive Dementia ◽  
Rare Presentation ◽  
Alien Hand ◽  
Jakob Disease ◽  
Neurological Features ◽  
Characteristic Features

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.

Characterising the uncommon corticobasal syndrome presentation of sporadic Creutzfeldt-Jakob disease

2013 ◽  
Vol 19 (1) ◽  
pp. 81-85 ◽  
Author(s):  
Will Lee ◽  
Marion Simpson ◽  
Helen Ling ◽  
Catriona Mclean ◽  
Steven Collins ◽  
...  
Keyword(s):  
Corticobasal Syndrome ◽  
Jakob Disease

scholarly journals Sparing of the substantia nigra in sporadic Creutzfeldt-Jakob disease presenting as an acute corticobasal syndrome

2007 ◽  
Vol 22 (11) ◽  
pp. 1668-1669 ◽  
Author(s):  
Wim Vandenberghe ◽  
Raf Sciot ◽  
Philippe Demaerel ◽  
Koen Van Laere
Keyword(s):  
Substantia Nigra ◽  
Corticobasal Syndrome ◽  
Jakob Disease

Familial Creutzfeldt–Jakob disease with M232R mutation presented with corticobasal syndrome

2014 ◽  
Vol 36 (7) ◽  
pp. 1291-1293 ◽  
Author(s):  
Jung Geol Lim ◽  
Eungseok Oh ◽  
Sangmin Park ◽  
Yong-Sun Kim ◽  
Aeyoung Lee
Keyword(s):  
Corticobasal Syndrome ◽  
Jakob Disease

scholarly journals A Rare Case of Creutzfeldt-Jakob Disease in an 80-Year-Old Male

Cureus ◽  
2020 ◽  
Author(s):  
Mario Dervishi ◽  
Travis Lambert ◽  
Maria Markosyan Karapetyan ◽  
Nader Warra ◽  
Ziyad Iskenderian
Keyword(s):  
Rare Case ◽  
Jakob Disease

scholarly journals A rare case of Creutzfeldt‐Jakob disease reported from Nepal

2021 ◽  
Vol 9 (9) ◽  
Author(s):  
Durga Neupane ◽  
Prashant Kumar Gupta ◽  
Sushil Sharma Subedi ◽  
Dilip Gupta ◽  
Sunit Chhetri
Keyword(s):  
Rare Case ◽  
Jakob Disease

scholarly journals Pearls & Oy-sters: Typical Atypical Creutzfeld-Jakob Disease: A Case Presenting as a Rapidly Progressive Corticobasal Syndrome Without Dementia

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000012613
Author(s):  
Zoe Olga Marinides ◽  
Timothy R Malone ◽  
Yitao Ma ◽  
Jason Hawley
Keyword(s):  
Diagnostic Testing ◽  
Behavioral Changes ◽  
Corticobasal Syndrome ◽  
Rapid Progression ◽  
Early Disease ◽  
Early Dementia ◽  
Progressive Neurodegeneration ◽  
Characteristic Imaging ◽  
Jakob Disease ◽  
High Index Of Suspicion

Creutzfeld-Jakob Disease (CJD) is a rare disease but a common cause of rapidly progressive neurodegeneration. Although the “classic” presentation involves early dementia or behavioral changes, there are well-described atypical variants with less prominent cognitive symptoms at onset. One such variant is Corticobasal Syndrome (CBS), which may be seen with other underlying neurodegenerative processes, but when due to prion disease pathology involves much more rapid progression and certain characteristic imaging findings. This report presents a case presenting as CBS without cognitive or behavioral changes at onset, which rapidly progressed and was determined ultimately to be due to underlying CJD. This case illustrates the need for a high index of suspicion for CJD in order to drive appropriate diagnostic testing and careful review of imaging and EEG findings, which may be subtle in early disease.

scholarly journals A rare case of Creutzfeldt-Jakob disease presenting with amyotrophic lateral sclerosis

2021 ◽  
Author(s):  
Yethindra Vityala ◽  
Elmira Mamytova ◽  
Dzhamalbek Turgumbaev ◽  
Altynai Zhumabekova ◽  
Tugolbai Tagaev ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Amyotrophic Lateral Sclerosis ◽  
Magnetic Resonance ◽  
Rare Case ◽  
Upper Extremities ◽  
Resonance Imaging ◽  
History Of ◽  
Jakob Disease ◽  
Thalamic Lesions ◽  
Lateral Sclerosis

Here, we present 76-year-old woman, who presented with a history of quadriparesis from three years ago, initially in the lower extremities and ascended towards the upper extremities (symmetrical and progressive). Magnetic resonance imaging revealed bilateral thalamic lesions (Figure 1), which could identify it as a variant of Creutzfeldt-Jakob disease.

scholarly journals Neurosurgery in a Case of Suspected Prion Disease: Challenges Faced

2020 ◽  
Vol 09 (01) ◽  
pp. 67-72
Author(s):  
Anooja Abdul Salam ◽  
Arvind Dubey
Keyword(s):  
Prion Disease ◽  
Disease Transmission ◽  
Healthcare Workers ◽  
Rare Case ◽  
Control Measures ◽  
Main Concern ◽  
Infection Control Measures ◽  
Jakob Disease ◽  
Risk Of Disease ◽  
Sphenoid Wing Meningioma

AbstractThis report refers to a rare case of a patient with suspected prion disease and the steps taken for the infection control measures in postsurgical management of a large sphenoid wing meningioma. Stringent measures are needed to prevent the transmission of Creutzfeldt–Jakob disease (CJD) to other patients or healthcare workers. The main concern, especially in a neurosurgical case, is the handling of highly infective tissue and hence the higher risk of disease transmission. This report provides guidance based on the experience in this case, as an aid to similar surgeries.

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