Objective: Based on the current criteria, the diagnosis of "possible" or "probable" variant Creutzfeldt-Jakob disease (vCJD) implies the absence of periodic sharp wave complexes (PSWCs) in the electroencephalogram (EEG). To verify this point, we investigated the development of the EEG changes along the course of the disease in a pateint with vCJD.
Methods: Long-lasting EEG-polygraphic recordings were performed once a month during the last year of illness.
Results: We found the occurrence of a typical EEG periodic pattern in the late clinical stage of the vCJD patient.
Interpretation: In the light of our finding, the diagnostic criteria for vCJD should be amended to include the possibility of a typical periodic EEG in advanced stages of disease in cases with long survival.
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http://dx.doi.org/10.1002/ana.20768 | DOI Listing |
J Mol Graph Model
December 2024
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.
View Article and Find Full Text PDFCase Rep Neurol Med
November 2024
Department of Neurology, Duke University Hospital, Durham, North Carolina, USA.
Presenting symptoms of sporadic Creutzfeldt-Jakob disease (sCJD) are variable, and as imaging and EEG may be normal in the early to middle stages of the disease process, serial testing is vital when there is clinical suspicion for sCJD. We present a case of probable Heidenhain variant of sCJD (HvCJD) with notable rapid progression. A 72-year-old woman presented with neurological decline following new-onset visual changes.
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