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Doença de príon

Последний просмотренный: 15 Aug 2025
Last updated: 27 Sep 2024

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
Полная информация

Другие диагностические факторы

  • sintomas sensoriais dolorosos
  • distúrbios de movimento
Полная информация

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
Полная информация

Диагностические исследования

Исследования, которые показаны в первую очередь

  • ressonância nuclear magnética (RNM) cranioencefálica
  • eletroencefalograma (EEG)
Полная информация

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)
Полная информация

Алгоритм лечения

ПРОДОЛЖЕНИЕ

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

Our in-house evidence and editorial teams collaborate with international expert contributors and peer reviewers to ensure that we provide access to the most clinically relevant information possible.

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.

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