Резюме
Определение
Анамнез и осмотр
Ключевые диагностические факторы
- comprometimento cognitivo
- ataxia de marcha e/ou de membros
- mioclonia
- parkinsonismo
- sintomas psiquiátricos
- alterações visuais
- fim da terceira década de vida ou meados/fim da sétima década de vida
- insônia, disautonomia
- história familiar positiva
- sintomas constitucionais ou inespecíficos
Другие диагностические факторы
- sintomas sensoriais dolorosos
- distúrbios de movimento
Факторы риска
- predisposição genética
- instrumentos cirúrgicos contaminados por príons
- transfusão de sangue ou de hemoderivados (variante da doença de Creutzfeldt-Jakob)
- consumo de carne do Reino Unido de 1980 a 1996
- consumo de carne bovina dos EUA
- caça de cervos, uapitis (espécie de veado) e alces em regiões endêmicas dos EUA e do Canadá
- uso de hormônio do crescimento (GH) humano
Диагностические исследования
Исследования, которые показаны в первую очередь
- ressonância nuclear magnética (RNM) cranioencefálica
- eletroencefalograma (EEG)
Исследования, проведение которых нужно рассмотреть
- conversão induzida por estremecimento (CIE)
- biomarcadores do líquido cefalorraquidiano (LCR)
- teste genético para gene da proteína príon
- biópsia (cérebro, amígdala)
Алгоритм лечения
todos os pacientes
Составители
Авторы
Valerie Sim, MD, FRCPC
Associate Professor
Department of Medicine
Division of Neurology
Centre for Prions and Protein Folding Diseases
University of Alberta
Edmonton
Alberta
Canada
Раскрытие информации
VS is principal and co-investigator on several research grants through the University of Alberta, to study prion disease and related neurodegenerative diseases. VS has one patent approved for the development of IgE-based prion therapy. VS is currently a site lead for the Ionis PrProfile trial. VS has been invited to give an educational session on prion disease at the American Academy of Neurology meeting in 2024.
Выражение благодарностей
Dr Valerie Sim would like to gratefully acknowledge Dr Michael D. Geschwind, Dr Amy Kuo, and Dr R. Ronald Finley, the previous contributors to this topic. MDG participates in the speakers' bureau for Pfizer, Forest, and Novartis; is consultant for MedaCorp, Gerson-Lehman Group, and Clinical Advisors Incorporated; and is an author of a number of references cited in this topic. RRF participates in the speakers' bureau for Pfizer, Forest, and Novartis. AK declares that she has no competing interests.
Рецензенты
Ali Hassoun, MD, FACP, FIDSA, AAHIVS
Infectious Disease Specialist
Alabama Infectious Diseases Center
Huntsville
AL
Раскрытие информации
AH declares that he has no competing interests.
Robert A. Larsen, MD
Associate Professor of Medicine
University of Southern California
Keck School of Medicine
Los Angeles
CA
Раскрытие информации
RAL declares that he has no competing interests.
William A. Petri, Jr., MD, PhD, FACP
Chief and Professor of Medicine
Division of Infectious Diseases and International Health
University of Virginia Health System
Charlottesville
VA
Раскрытие информации
WAP declares that he has no competing interests.
Peer reviewer acknowledgements
BMJ Best Practice topics are updated on a rolling basis in line with developments in evidence and guidance. The peer reviewers listed here have reviewed the content at least once during the history of the topic.
Disclosures
Peer reviewer affiliations and disclosures pertain to the time of the review.
Список литературы
Основные статьи
Department of Health. Minimise transmission risk of CJD and vCJD in healthcare settings. Nov 2021 [internet publication].Полный текст
Centers for Disease Control and Prevention. BSE (Bovine spongiform encephalopathy, or mad cow disease). Sep 2021 [internet publication].Полный текст
American College of Radiology. ACR appropriateness criteria: movement disorders and neurodegenerative diseases. 2019 [internet publication].Полный текст
Статьи, указанные как источники
A full list of sources referenced in this topic is available to users with access to all of BMJ Best Practice.
Отличия
- Demência de Alzheimer (DA)
- demência com corpos de Lewy
- Demência frontotemporal
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