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Human prion diseases (PrDs) are a group of transmissible neurodegenerative diseases that can be clarified as sporadic, genetic and iatrogenic forms. In this study, we have analysed the time and geographic distributions of 2011 PrD cases diagnosed by China National Surveillance for Creutzfeldt-Jakob disease (CNS-CJD) since 2006, including 1792 spora...
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Sporadic Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative condition and a human prion disease. Rapid progressive dementia, myoclonus, visual disturbances, cerebellar signs, and pyramidal/extrapyramidal symptoms are observed in such patients. However, these are non-specific symptoms and can manifest in a variety of other conditions. The o...
Creutzfeldt-Jakob disease (CJD) constitutes an aggressively advancing, terminal neurodegenerative condition classified within the spectrum of transmissible spongiform encephalopathies. The difficulty in establishing a diagnosis before death arises from the condition's rarity and the resulting limited level of suspicion attributed to it. The polymor...
The N-terminus of the PRNP gene normally contains a 5-octapeptide repeat (R1-R2-R2-R3-R4), and insertions at this locus can cause hereditary prion diseases. In the present study, we found a 5-octapeptide repeat insertion (5-OPRI) in a sibling case of frontotemporal dementia. Consistent with previous literature, 5-OPRI rarely met the diagnostic crit...
Background
Prions are renowned for their distinct resistance to chemical or physical inactivation including steam sterilization. Impaired efficacy of inactivation poses a risk to patients for iatrogenic transmission of Creutzfeldt-Jakob disease (CJD) via contaminated surgical instruments.
Aim
Most established prion inactivation methods were valida...
Background
Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, neurodegenerative disease. In Ireland, clinical diagnostics and laboratory testing remain the responsibility of the managing clinician and the Neuropathology Department at the Beaumont Hospital, respectively. Centralized review of individual cases is not undertaken.
Aims
To deter...
Citations
... Over the past few decades, there have been increased reports on sCJD. Some studied regional or national geographical distribution or temporal occurrence, but their cases occurred during periods of 9 to 15 years (14)(15)(16)(17)(18). Few case series focused on cases with similar clinical presentation without patients' geographic information (19,20), or on cases over 5 years in the same region (21). ...
... Geographical clusters of sCJD have been reported, but most clusters contained cases distributed over many years (14,17,18,(44)(45)(46)(47)(48), and few were temporo-spatial clusters. A French cluster of three cases of CJD occurring in 1998 reported that two of the patients lived in the same village. ...
Background
Creutzfeldt–Jakob disease (CJD) is a rare, rapidly progressive, and uniformly fatal neurodegenerative disease. The reported incidence of CJD is 1 to 2 per million people worldwide annually, with fewer than 1,000 cases in the United States per year. In this study, we report a unique case series on temporo-spatial clusters of CJD cases in West Michigan.
Methods
A total of five CJD cases consisting of two temporal clusters were seen from July 2021 to June 2022 at Corewell Health West hospitals. All patients had brain MRI, EEG, and CSF tests. Four patients underwent autopsies.
Results
All patients' MRIs showed characteristic CJD patterns. Four patients had positive CJD panels in CSF. One patient had typical CJD EEG findings. Four patients were confirmed as sporadic CJD by autopsy. All patients died within 3 months after CJD was suspected.
Discussion
All patients lived within a 90-mile radius of Grand Rapids, MI, and two lived in the same county. West Michigan has a population of 1.6 million people, and the four counties where five patients lived have a combined population of 395,104, indicating CJD's new case rate of 3.1 and 12.5 per million people, respectively. Corewell Health is one of the three major healthcare systems in West Michigan. The actual incidence of CJD in West Michigan is likely even higher. This dense temporal and spatial cluster of CJD cases poses a serious public health challenge and warrants urgent investigation.
