jakob-creutzfeldt disease

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Definition

Noun: A rare, typically fatal neurodegenerative disorder, usually occurring in middle age, caused by an unidentified slow-acting infectious agent (prion). It is characterized by rapidly progressive dementia, neurological deterioration, and gradual loss of muscle coordination.

Usage and Examples
  • As a subject:
    • Jakob-Creutzfeldt disease is a serious concern for neurologists.
  • As an object:
    • The autopsy confirmed the presence of Jakob-Creutzfeldt disease.
  • With modifiers:
    • The sporadic form of Jakob-Creutzfeldt disease is the most common.
Advanced Usage
  • In medical literature: The term is used in clinical and pathological contexts to describe a specific prion disease with distinct histopathological features, such as spongiform encephalopathy.
  • In public health: Discussions often focus on its transmission risks, differentiating it from variant Creutzfeldt-Jakob disease (vCJD) linked to bovine spongiform encephalopathy (BSE).
Variants and Related Words
  • Creutzfeldt-Jakob disease (CJD): The more common and standard form of the term, often used interchangeably.
  • Variant Creutzfeldt-Jakob disease (vCJD): A distinct form linked to the consumption of contaminated beef products.
  • Prion disease: The broader category of transmissible spongiform encephalopathies to which this disease belongs.
Synonyms
  • CJD (the standard abbreviation).
  • Classic CJD (to distinguish from the variant form).
  • Subacute spongiform encephalopathy (a descriptive medical term).
Notes on Meaning

This term specifically refers to the classic form of the disease, primarily sporadic or familial in origin. It is distinct from other prion diseases like kuru or Gerstmann-Sträussler-Scheinker syndrome. The defining characteristics are its rapid progression, dementia, myoclonus, and typical electroencephalogram (EEG) patterns.

Noun
  1. rare (usually fatal) brain disease (usually in middle age) caused by an unidentified slow virus; characterized by progressive dementia and gradual loss of muscle control

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