Prion disease features in Japan based on the national surveillance from 1999 to 2023

 Prion disease features in Japan based on the national surveillance from 1999 to 2023

Authors: Tadashi Tsukamoto 1), Koki Kosami 2), Ryuusuke Ae 2), Tsuyoshi Hamaguchi 3), Nobuo Sanjo 4), Katsuya Satoh 5), Tetsuyuki Kitamoto 6), Masaki Takao 7), Masahito Yamada 8), and Hidehiro Mizusawa 1) and Prion Diseases Surveillance Committee in Japan

Affiliations: 1 Department of Neurology, National Center Hospital, National Center of Neurology and Psychiatry, Tokyo, Japan 2 Department of Public Health, Jichi Medical University, Shimotsuke, Japan 3 Department of Neurology, Kanazawa Medical University, Uchinada Town, Japan 4 Department of Neurology and Neurological Science, Graduate School, Tokyo Medical and Dental University, Tokyo, Japan 5 Department of Locomotive Rehabilitation Sciences, Nagasaki University Graduate School of Medicine, Nagasaki, Japan. 6 Department of Prion Protein Research, Division of CJD Science and Technology, Tohoku University Graduate School of Medicine, Sendai, Japan 7 Department of Clinical Laboratory, National Center of Neurology and Psychiatry, National Center Hospital, Tokyo, Japan. 8 Division of Neurology, Department of Internal Medicine, Kudanzaka Hospital, Tokyo, Japan.

Aim: To elucidate epidemiological features of prion disease (PrD) in Japan.

Methods: Nationwide surveillance has been conducted by Prion Disease Surveillance Committee in Japan since 1999. The committee was composed of 10 district members and specialists in epidemiology, neurosurgery, genetics, EEG, imaging, CSF biomarkers, and genetic counseling. Clinical information on suspected PrD cases was compiled and reviewed in the committee twice yearly, resulting in diagnoses of PrD and non-PrD.

Results: Information of 6936 suspected PrD patients was obtained and until February 2023 there were 4714 PrD cases [sporadic CJD 3578 cases, variant CJD 1, dura matter-associated CJD (dCJD) 93, and genetic PrD 1024 (genetic CJD 842, 174 Gerstmann-Sträussler-Scheinker disease 174, fatal familial insomnia 8)]. The annual incidence increased from 0.7 to 2.3 per million between 1999 and 2015. Genetic analysis showed a high ratio of methionine/methionine (95%) at codon 129 in sporadic CJD. Effects of polymorphisms at codon 129 and 219 to various PrDs were investigated.

Conclusions: Our surveillance confirmed the increase of PrD incidence and characteristic features of PrDs in Japan. Continuous surveillance of PrDs is very important and has contributed greatly to various types of research to overcome PrDs.

Funded by: 1) Research Committee of Surveillance and Infection Control of Prion Disease, the Ministry of Health, Labour, and Welfare of Japan

2) Research Committee of Prion Disease and Slow Virus Infection, Research on Policy Planning and Evaluation for Rare and Intractable Diseases, Health and Labour Sciences Research Grants

Grant number: 1) 22FC2002 2) 20FC1054

Acknowledgment: We are grateful to the prion disease specialists in the prefectures, physicians, patients with prion disease, and their families for providing clinical information.

https://prion2023.org/wp-content/uploads/2023/10/Meeting-book-final-version2.pdf

Professor John Collinge on tackling prion diseases sCJD around 1 in 5000 deaths worldwide

“The best-known human prion disease is sporadic Creutzfeldt-Jakob disease (sCJD), a rapidly progressive dementia which accounts for around 1 in 5000 deaths worldwide.”

it has been established that similar so-called “prion-like” mechanisms are involved in much commoner conditions including Alzheimer’s and Parkinson’s diseases.

We defined iatrogenic cerebral amyloid angiopathy as a new disease, with relevance to Alzheimer’s disease and public health.

https://creutzfeldt-jakob-disease.blogspot.com/2023/09/professor-john-collinge-on-tackling.html

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