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Creutzfeldt-Jakob Disease
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Brain Nerves

Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative brain disorder that progresses rapidly, typically leading to dementia, coma, and death within a year.

Overview

Symptoms of Creutzfeldt-Jakob disease usually manifest around age 60. Early signs include memory problems, behavior changes, vision problems, and poor muscle coordination, which progress quickly.

Categories of CJD

There are three main categories of CJD:

  • Sporadic CJD, which occurs for no known reason.
  • Hereditary CJD, which runs in families.
  • Acquired CJD, which occurs from contact with infected tissue, usually during a medical procedure.

Bovine Spongiform Encephalopathy

Cattle can develop a disease related to CJD called bovine spongiform encephalopathy (BSE), or "mad cow disease." While there is concern that humans may contract a variant of CJD from consuming beef from an infected animal, there is currently no direct proof to support this claim.

Frequently Asked Questions

Unlike Alzheimer's disease or vascular dementia, which typically progress over several years or decades, CJD has a rapid, aggressive course. Patients generally decline from mild memory problems, mood changes, or balance instability to severe dementia, muscle rigidity, involuntary jerking (myoclonus), blindness, and an inability to speak or move within weeks to months. The median survival time is approximately 4 to 6 months from symptom onset, and around 90 percent of patients die within one year.
While a brain biopsy or post-mortem autopsy is the definitive gold standard, clinicians today diagnose probable CJD with high accuracy using real-time quaking-induced conversion (RT-QuIC) testing of cerebrospinal fluid, which detects abnormal prion proteins. This is combined with high-resolution brain MRI showing characteristic signals in the basal ganglia and cerebral cortex (cortical ribboning), electroencephalography (EEG) showing periodic sharp wave complexes, and testing for elevated 14-3-3 and total tau proteins in spinal fluid.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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