Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare and devastating illness. It is the most frequently encountered form of the spongiform encephalopathies with 50 new cases a year in the UK. It presents with a myriad of symptoms reflecting central nervous system dysfunction and is characterized by a rapidly progressive dementia leading to death. The disease process can pose multiple challenges: diagnostic conundrums, complexities in management, and palliative care issues. Good coordinated care between services and information is paramount in adequate management and delivery of care for patients suffering from sCJD.Psychiatry services frequently become involved in the assessment and management of sCJD.
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Multiomic analyses direct hypotheses for Creutzfeldt-Jakob disease risk genes.
Brain
January 2025
Medical Research Council Prion Unit, University College London Institute of Prion Diseases, London, W1W 7FF, UK.
Prions are assemblies of misfolded prion protein that cause several fatal and transmissible neurodegenerative diseases, with the most common phenotype in humans being sporadic Creutzfeldt-Jakob disease (sCJD). Aside from variation of the prion protein itself, molecular risk factors are not well understood. Prion and prion-like mechanisms are thought to underpin common neurodegenerative disorders meaning that the elucidation of mechanisms could have broad relevance.
View Article and Find Full Text PDFCase report: Atypical young case of MV1 Creutzfeldt-Jakob disease with unusually long survival.
Front Cell Neurosci
January 2025
Department of Pathology, Case Western Reserve University, School of Medicine, Cleveland, OH, United States.
Creutzfeldt-Jakob disease (CJD) is a rare, fatal, rapidly progressive neurodegenerative disease resulting from an accumulation of misfolded prion proteins (PrP). CJD affects 1-2 new individuals per million each year, and the sporadic type accounts for 90% of those cases. Though the median age at onset and disease duration vary depending on the subtype of sporadic CJD (sCJD), the disease typically affects middle-aged to elderly individuals with a median survival of 4-6 months.
View Article and Find Full Text PDFMood Alterations in the Prodromal Phase of Sporadic Creutzfeldt-Jakob Disease.
JAMA Neurol
December 2024
Department of Neurology, Medical University of Vienna, Vienna, Austria.
Importance: Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare, rapidly progressive and fatal neurodegenerative disease. Definite sCJD diagnosis can only be made post mortem, and little is known about the prodromal phase of the disease.
Objective: To compare drug prescription patterns before the clinical onset of sCJD between patients and matched controls for exploration of potential risk factors and to assess correlations between drug exposure and sCJD survival.
Diagnostic challenge of sporadic Creutzfeldt-Jakob disease revealed by a psychiatric presentation.
Psychogeriatrics
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Department of Internal Medicine, Centre Hospitalier Victor Dupouy, Argenteuil, France.
Characteristics of Creutzfeldt-Jakob disease at Siriraj Hospital, Thailand: Case series and literature review.
Clin Park Relat Disord
November 2024
Department of Neurology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Introduction: Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive, fatal, neurodegenerative disease classified as prion diseases. There are many subtypes of this disease, but information about clinical presentation and investigation findings in Thailand is scarce.
Objective: To describe the clinical presentation, radiological and electroencephalographic characteristics of CJD encountered at Siriraj hospital in the past 10 years (between January 1, 2006 and December 31, 2015).
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