Creutzfeldt-Jakob Disease (CJD) is an infectious, progressive, degenerative neurological disorder. Unique CJD Precautions must be adhered to as the infectious agent is difficult to destroy. A regional group in Hamilton-Burlington Ontario developed CJD guidelines based on critical review of the current evidence of transmission in a Canadian healthcare environment, current published standards of practice in North America, the United Kingdom and Australia; and principles of laboratory and patient care safety as well as expert opinion.
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Sporadic Creutzfeldt-Jakob Disease: A Case Report and Literature Review.
Cureus
December 2024
Internal Medicine, Hospital Angeles Pedregal, Mexico City, MEX.
Prion disease is an uncommon entity characterized by exceptionally rapid neurodegenerative deterioration. There are three categories of prion disease: (1) sporadic: sporadic Creutzfeldt-Jakob disease (sCJD), sporadic fatal insomnia, and protease-sensitive prionopathy; (2) genetic: genetic Creutzfeldt-Jakob disease, familial fatal insomnia, and Gerstmann-Sträussler-Scheinker syndrome; and (3) acquired: Kuru, iatrogenic Creutzfeldt-Jakob disease, and variant Creutzfeldt-Jakob disease. Although it is an incurable disease, a specific pathophysiological mechanism exists involving neuronal loss, glial cell proliferation, absence of inflammatory response, development of vacuoles leading to a spongiform appearance, and the presence of prions.
View Article and Find Full Text PDF[Detection of brain MRI abnormalities before symptom onset in case of Creutzfeldt-Jakob disease with compound heterozygous PRNP mutation (V180I/M232R)].
Rinsho Shinkeigaku
January 2025
Department of Internal Medicine IV, Division of Neurology, Osaka Medical and Pharmaceutical University Faculty of Medicine.
In an 81-year-old man, brain diffusion-weighted MRI revealed punctate high-intensity lesions in the bilateral frontal cortex. Three months later, these lesions had extended into the cerebral cortices. Six months after the original MRI, the patient developed cognitive decline.
View Article and Find Full Text PDF[Diagnostic challenge: Sporadic Creutzfeldt-Jakob disease].
Semergen
January 2025
Unidad de Atención Primaria, Centro de Salud Las Lagunas, Distrito Costa del Sol, Mijas, Málaga, España.
Chaperones as Potential Pharmacological Targets for Treating Protein Aggregation Illness.
Curr Protein Pept Sci
January 2025
Department of Pharmacy, Panipat Institute of Engineering and Technology, India.
The three-dimensional structure of proteins, achieved through the folding of the nascent polypeptide chain in vivo, is largely facilitated by molecular chaperones, which are crucial for determining protein functionality. In addition to aiding in the folding process, chaperones target misfolded proteins for degradation, acting as a quality control system within the cell. Defective protein folding has been implicated in a wide range of clinical conditions, including neurodegenerative and metabolic disorders.
View Article and Find Full Text PDFRapid Neurological Decline due to Creutzfeldt-Jakob Disease.
J Community Hosp Intern Med Perspect
January 2025
Creutzfeldt-Jakob Disease (CJD) is an exceedingly rare condition with an incidence of one case per million people worldwide. It is diagnosed with symptoms of rapid neurological decline, positive CSF biomarkers, and diagnostic findings on MRI and EEG. There is no known effective therapy for CJD and prompt diagnosis is required to prepare for the expected disease prognosis and goals of care discussions.
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