Neuropathology of 8 patients of the New Brunswick cluster of Neurological Syndrome of Unknown Cause; human Chronic Wasting Disease or blue-green algae?
Neuropathology of 8 patients of the New Brunswick cluster of Neurological Syndrome of Unknown Cause; human Chronic Wasting Disease or blue-green algae?
Gerard H. Jansena, Dean A. Fergussonb, John M. J. Woulfea, and Alexander S. Eastonc
aDepartment of Pathology and Laboratory Medicine, University of Ottawa, Ottawa, Ontario, Canada; bClinical Epidemiology Program, The Ottawa Hospital Research Institute, Ottawa, Ontario, Canada; cDepartment of Pathology, QEII Health Science Centre, Halifax, Nova Scotia, Canada
Aims: Since late 2019 patients were reported from one Canadian province to the Canadian CJD surveillance, and it was suggested these suffered from a new unknown neurological disease, possibly human CWD. In March 2021 this cluster became world news. 48 of the patients of this cluster of Neurological Syndrome of Unknown Cause were investigated by the Oversight Committee established by the New Brunswick government. 96% of cases had been referred by one local neurologist. Ten patients had died at the time of writing of this abstract, and eight came to autopsy. In April 2021, following the results of the first autopsies, suggestions for other environmental factors causing this cluster were made: aberrant amino acid BMAA from blue-green algae. The Oversight Committee was briefed in August 2021 of the results of our study, and partly based on those, on 24 February 2022 the province of New Brunswick concluded that there was no evidence to support a new mysterious illness.
Material and Methods: Brain tissue was obtained at autopsy as formalin fixed (8/8) and frozen (7/8). Routine stains were performed, and PrP (12F10), TDP43, β-amyloid, Tau, P62, α-Synuclein. Slides were reviewed by 2 independent neuropathologists. Western blotting for PrP was performed (7/8).
Results: In none of these 8 cases microscopical or immunohistochemical features of a prion disease were found. No misfolded prion protein was detected by WB. Pathology showed that the group of 8 consisted of: one case of vascular dementia; one case without morphological changes (consistent with clinical history); one case of neoplasia; and 5 cases of neurodegenerative disease. The latter group showed in each case mixed dementia with as main component Alzheimer disease (2/5), neocortical Lewy body pathology (2/5), or frontotemporal lobe dementia TDP43 (1/5).
Conclusions:
No prion disease was found in any of the 8 autopsy cases.
An inhomogeneous group of diagnoses was found in the 8 cases. Considering the 48 total cases and a conservative diagnostic acumen, the probability of having all 8 consecutive autopsy cases representing a new mystery illness is exteremly remote (p = 0.0001 using classical probability theorem). Therefore, the cluster constitutes not a new disease, but a cluster of clinical misdiagnoses.
The suggestion that blue-green algae BMAA would be causing known misfolding diseases, initiating this cluster, can be dismissed as 3/8 patients did not suffer from a misfolding disease. Moreover, the original presentation in this cluster is not consistent with this hypothesis as all 8 were initially suggested to have a ‘new-unknown’ disease, not a ‘known’ neurodegenerative disease.
Funded by: Pathology work-up and Western blotting was funded by Public Health Agency of Canada. No other funding/support was received.
Acknowledgement: The University of Ottawa Neuropathology Laboratory Services staff; Olga Agah, Eric Labelle, and Sharlene Faulkes. The National Microbiology Laboratory in Winnipeg staff: Dr. J. David Knox and Anne Peterson.
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Prion 2022 Conference abstracts: pushing the boundaries
some history here;
WEDNESDAY, OCTOBER 27, 2021
New Brunswick October 27, 2021 - Health minister availability Report on cluster of unknown neurological disorders update
THURSDAY, MARCH 18, 2021
New Brunswick monitoring more than 40 cases of unknown neurological disease symptoms are similar to Creutzfeldt-Jakob disease?
FIRST AND FOREMOST, LET US PRAY THIS IS NOT A TSE PRION DISEASE!
SUNDAY, MAY 08, 2022
USA National Prion Disease Pathology Surveillance Center Surveillance Update April 11th, 2022
TUESDAY, APRIL 05, 2022
Incidence of Creutzfeldt-Jakob Disease in the United States 1993-2014
FRIDAY, DECEMBER 24, 2021
***> Creutzfeldt Jakob Disease CJD TSE Prion Update December 25, 2021 <***
TUESDAY, MAY 24, 2022
Texas Creutzfeldt Jakob Disease CJD TSE Prion Update Singeltary FOIA Request Received May 23, 2022
MONDAY, JUNE 14, 2021
Texas Health and Human Services The Department of State Health Services Creutzfeldt Jakob Disease TSE Prion Report 2021?
Terry S. Singeltary Sr.
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