We report a case of variant Creutzfeldt-Jakob disease(vCJD) in a 74-year old man in whom diagnosis was made at necropsy. The occurrence of vCJD in an individual in this age group is unlikely to be an isolated event. Doctors need to be aware that vCJD can arise in elderly patients so that appropriate investigations (including magnetic resonance imaging) can be done, and permission for neuropathological necropsy requested, in suspected cases. This case could also have important implications for public health policy decisions and surveillance programmes that target the younger age range of vCJD cases.
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http://dx.doi.org/10.1016/s0140-6736(00)04504-9 | DOI Listing |
Sleep Med
December 2024
Department of Neurology, Henan Provincial People's Hospital, Zhengzhou University People's Hospital, Zhengzhou, Henan Province, China. Electronic address:
Fatal familial insomnia (FFI) is a rare autosomal dominant neurodegenerative disorder characterized by rapidly progressive dementia, severe sleep disturbances, and autonomic dysfunction. The clinical manifestations of FFI can exhibit substantial variations, making it crucial to rule out other conditions, such as autoimmune encephalitis and Creutzfeldt-Jakob disease, during early diagnosis. In this study, we describe the case of a 58-year-old man who experienced persistent insomnia, autonomic symptoms, gait instability, and rapidly progressive dementia.
View Article and Find Full Text PDFJ Mol Graph Model
March 2025
Department of Chemistry, Faculty of Science and Technology, University of Nairobi, P.O. Box 30197-00100, Nairobi, Kenya.
The human prion protein gene (PRNP) consists of two common alleles that encode either methionine or valine residues at codon 129. Polymorphism at codon 129 of the prion protein (PRNP) gene is closely associated with genetic variations and susceptibility to specific variants of prion diseases. The presence of these different alleles, known as the PRNP codon 129 polymorphism, plays a significant role in disease susceptibility and progression.
View Article and Find Full Text PDFClin 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).
Neurol India
November 2024
Department of Neurology, Xuanwu Hospital, Capital Medical University, Beijing, China.
Neurology
January 2025
From the Department of Neurology (Z.C., D.J., Y.K., J.Z., C.M., H.Y., J. Li, L. Wang, L. Wu), and Department of Radiology and Nuclear Medicine (S.B., S.Y., J. Lu), Xuanwu Hospital, Capital Medical University, Beijing, China.
Background And Objectives: Abnormal glymphatic system-related proteins have been identified in a small-scale pathologic study of patients with Creutzfeldt-Jakob disease (CJD). However, it remains unclear whether glymphatic dysfunction occurs in vivo in patients with CJD and whether this decline begins during the preclinical stage. This study aimed to investigate the relationship between glymphatic dysfunction and clinical characteristics in patients with CJD, as well as potential glymphatic impairment in preclinical CJD.
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