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[Creutzfeld-Jakob disease].

P Giraud1, A Alpérovitch, G Chazot

  • 1Service de neurologie, hôpital neurologique et neuro-chirurgical Pierre Wertheimer 69394 Lyon.

La Revue Du Praticien
|February 28, 2002
PubMed
Summary

Creutzfeldt-Jakob disease (CJD) is a fatal neurodegenerative disorder with four main types: familial, iatrogenic, sporadic, and a new variant linked to bovine spongiform encephalopathy. Diagnosis requires histological confirmation.

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Area of Science:

  • Neurology
  • Pathology
  • Genetics

Context:

  • Creutzfeldt-Jakob disease (CJD) was first described in Germany in the 1920s.
  • It is a rare, fatal prion disease affecting the brain.

Purpose:

  • To outline the distinct entities of Creutzfeldt-Jakob disease.
  • To describe the clinical presentation and diagnostic criteria for CJD.

Summary:

  • CJD presents in four forms: familial (8%), iatrogenic (5%), sporadic (87%), and a rare new variant linked to bovine spongiform encephalopathy.
  • Key clinical signs include rapidly progressing dementia, myoclonus, ataxia, and characteristic electroencephalographic abnormalities.
  • Definitive diagnosis of Creutzfeldt-Jakob disease is established through histological examination.

Impact:

  • Enhances understanding of CJD's diverse etiology and presentation.
  • Highlights the invariably fatal nature and rapid progression of the disease.
  • Emphasizes the importance of histological confirmation for accurate diagnosis.

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