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1.
Eur J Neurol ; 29(4): 1222-1226, 2022 04.
Article in English | MEDLINE | ID: covidwho-1583570

ABSTRACT

BACKGROUND AND PURPOSE: Creutzfeldt-Jakob disease (CJD) is lethal and transmissible. We assessed the impact of the COVID-19 pandemic on UK CJD surveillance. We hypothesized that (i) disruptions prolonged diagnostic latency; (ii) autopsy rates declined; and (iii) COVID-19 infection negatively affected diagnosis, care, and survival. METHODS: We retrospectively investigated the first year of the pandemic, using the preceding year as a comparator, quantifying numbers of individuals assessed by the UK National CJD Research & Surveillance Unit for suspected CJD, time to diagnosis, disease duration, and autopsy rates. We evaluated the impact of COVID-19 status on diagnosis, care, and survival in CJD. RESULTS: A total of 148 individuals were diagnosed with CJD in the pandemic (from a total of 166 individuals assessed) compared to 141 in the comparator (from 145 assessed). No differences were identified in disease duration or time to diagnosis. Autopsy rates were unchanged. Twenty individuals had COVID-19; 60% were symptomatic, and 10% had severe disease. Disruptions in diagnosis and care were frequently identified. Forty percent of COVID-19-positive individuals died; however, COVID-19 status did not significantly alter survival duration in CJD. CONCLUSIONS: The COVID-19 pandemic has not impacted UK CJD case ascertainment or survival, but diagnostic evaluation and clinical care of individuals have been affected.


Subject(s)
COVID-19 , Creutzfeldt-Jakob Syndrome , COVID-19/epidemiology , Creutzfeldt-Jakob Syndrome/diagnosis , Creutzfeldt-Jakob Syndrome/epidemiology , Humans , Pandemics , Patient Care , Retrospective Studies , SARS-CoV-2 , United Kingdom/epidemiology
2.
Neurodegener Dis Manag ; 12(1): 29-34, 2022 02.
Article in English | MEDLINE | ID: covidwho-1547169

ABSTRACT

Background: Accurate diagnosis and management of patients with rapidly progressive dementia may be challenging during the COVID-19 pandemic, which has negatively influenced the diagnostic performances, medical resource allocation and routine care for all non-COVID-19 diseases. Case presentation: We herein present a case of a 57-year-old male with rapidly progressive cognitive decline, headache, diplopia, myalgia, unsteady gait, aggression, depression, insomnia, hallucinations and delusions of persecution. COVID-19-associated encephalitis was briefly considered as a differential diagnosis. However, this hypothesis was rejected upon further investigation. A final diagnosis of sporadic Creutzfeldt-Jakob disease was made. Conclusion: A timely and accurate diagnosis of Creutzfeldt-Jakob disease gives patients and their families the chance to receive a good standard of healthcare and avoid extensive evaluations for other conditions.


Subject(s)
COVID-19/complications , Creutzfeldt-Jakob Syndrome/complications , Creutzfeldt-Jakob Syndrome/diagnosis , Encephalitis/complications , Encephalitis/diagnosis , COVID-19/epidemiology , Diagnosis, Differential , Humans , Male , Middle Aged , Pandemics
3.
BMJ Case Rep ; 14(5)2021 May 11.
Article in English | MEDLINE | ID: covidwho-1226742

ABSTRACT

Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, fatal neurodegenerative disorder belonging to the family of transmissible spongiform encephalopathies. The disease is believed to be caused by an abnormal isoform of a cellular glycoprotein known as the prion protein. Our patient is an 84-year-old Caucasian man who presented to the geriatric clinic for evaluation of short-term memory loss and decreased concentration which started 3 months prior to initial evaluation. Rapid progression of dementia demonstrated by severe impairment in tasks with a predominantly visual component, including visual scanning, perceptual reasoning and visual spatial processing. Diagnosis of CJD was determined by characteristic ribboning on brain MRI as well as notable real-time quaking-induced conversion on cerebrospinal fluid.


Subject(s)
Creutzfeldt-Jakob Syndrome , Prion Diseases , Aged , Aged, 80 and over , Creutzfeldt-Jakob Syndrome/complications , Creutzfeldt-Jakob Syndrome/diagnosis , Humans , Magnetic Resonance Imaging , Male
4.
Brain Behav Immun ; 89: 601-603, 2020 10.
Article in English | MEDLINE | ID: covidwho-651618

ABSTRACT

We describe a man whose first manifestations of Creutzfeldt-Jakob disease occurred in tandem with symptomatic onset of coronavirus disease 2019 (COVID-19). Drawing from recent data on prion disease pathogenesis and immune responses to SARS-CoV-2, we hypothesize that the cascade of systemic inflammatory mediators in response to the virus accelerated the pathogenesis of our patient's prion disease. This hypothesis introduces the potential relationship between immune responses to the novel coronavirus and the hastening of preclinical or manifest neurodegenerative disorders. The global prevalence of both COVID-19 and neurodegenerative disorders adds urgency to the study of this potential relationship.


Subject(s)
Brain/diagnostic imaging , Coronavirus Infections/complications , Creutzfeldt-Jakob Syndrome/complications , Pneumonia, Viral/complications , Aged , Betacoronavirus , Brain/physiopathology , COVID-19 , Coronavirus Infections/immunology , Creutzfeldt-Jakob Syndrome/diagnosis , Creutzfeldt-Jakob Syndrome/immunology , Creutzfeldt-Jakob Syndrome/physiopathology , Disease Progression , Electroencephalography , Fluorodeoxyglucose F18 , Humans , Magnetic Resonance Imaging , Male , Pandemics , Pneumonia, Viral/immunology , Positron-Emission Tomography , Radiopharmaceuticals , SARS-CoV-2
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