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Creutzfeldt-Jakob disease (CJD) is a prion disease characterized by rapidly progressive dementia that is often followed by behavioral disturbances, ataxia, myoclonus, and akinetic mutism. The initial symptoms of CJD reportedly vary, but the onset is usually gradual. Here, we report a case of CJD with a sudden, stroke-like onset of right hemiparesis to alert readers that CJD can mimic a stroke during its early stage.
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http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2020.104788 | DOI Listing |
J Neuropathol Exp Neurol
September 2025
Department of Medicine (RMH), The University of Melbourne, Parkville, VIC, Australia.
Prion diseases are rare neurodegenerative disorders that share misfolding of the normal cellular prion protein into disease-causing isoforms known as "prions" as the critical pathophysiological event. Definite diagnosis can only be achieved through neuropathological confirmation. The neuropathological features of prion disease are well described; however, some molecular subtypes are typified by characteristic neuropathological features that are subtle or absent.
View Article and Find Full Text PDFNeurology
September 2025
Department of Neurology, Mayo Clinic, Jacksonville, FL.
Background And Objectives: Timely access to palliative care, a specialty focusing on improving quality of life for patients with serious medical conditions, is central to patients with prion disease. To optimize evidence-informed utilization of palliative care resources, we systematically evaluated the frequency of clinical features amenable to supportive care, the frequency and patterns of referral to specialty palliative care, and the methods of palliative care delivery in patients with prion disease across a multicenter health care system.
Methods: A retrospective review was conducted on electronic medical records of patients diagnosed with definite (neuropathologically or genetically confirmed) or probable prion disease (meeting established clinical criteria for Creutzfeldt-Jakob disease) in inpatient and outpatient settings from January 2012 to August 2023 across the Mayo Clinic enterprise.
Neurol India
September 2025
Department of Neurology, Shangluo City Center Hospital, Shangluo, China.
Front Immunol
August 2025
Department of Neurology, The Second Clinical Medical College, Jinan University, Shenzhen, Guangdong, China.
Background: Anti-Ma2 antibody encephalitis is a rare paraneoplastic autoimmune encephalitis (AE) caused by anti-Ma2 antibody. Creutzfeldt-Jakob disease (CJD), a group of human prion diseases, is a rapidly advancing and fatal neurodegenerative disorder. The two diseases may display comparable clinical symptoms that are easily misdiagnosed.
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