Creutzfeldt–Jakob disease
| Creutzfeldt–Jakob disease | |
|---|---|
 |
|
| Tonsil biopsy in variant CJD. Prion protein immunostaining. | |
| Pronunciation | /ˈkrɔɪtsfɛlt ˈjɑːkoʊb/ KROITS-felt YAH-kohb) |
| Classification and external resources | |
| Specialty | Neurology |
| ICD-10 | A81.0, F02.1 |
| ICD-9-CM | 046.1 |
| OMIM | 123400 |
| DiseasesDB | 3166 |
| MedlinePlus | 000788 |
| eMedicine | neuro/725 |
| Patient UK | Creutzfeldt–Jakob disease |
| MeSH | D007562 |
Creutzfeldt–Jakob disease (CJD) is an incurable and universally fatal neurodegenerative disease. CJD is at times called a human form of mad cow disease (bovine spongiform encephalopathy or BSE). However, given that BSE is believed to be the cause of Variant Creutzfeldt–Jakob disease (vCJD) in humans, the two are often confused.
CJD is caused by an infectious agent called a prion. Prions are misfolded proteins that replicate by converting their properly folded counterparts, in their host, to the same misfolded structure they possess. CJD causes the brain tissue to degenerate rapidly, and as the disease destroys the brain, the brain develops holes and the texture changes to resemble that of a kitchen sponge.
Types of CJD include:
The first symptom of CJD is rapidly progressive dementia, leading to memory loss, personality changes, and hallucinations. Other frequently occurring features include anxiety, depression, paranoia, obsessive-compulsive symptoms, and psychosis. This is accompanied by physical problems such as speech impairment, jerky movements (myoclonus), balance and coordination dysfunction (ataxia), changes in gait, rigid posture, and seizures. In most patients, these symptoms are followed by involuntary movements and the appearance of an atypical diagnostic electroencephalogram tracing. The duration of the disease varies greatly, but sporadic (non-inherited) CJD can be fatal within months or even weeks. Most victims die six months after initial symptoms appear, often of pneumonia due to impaired coughing reflexes. About 15% of patients survive for two or more years. Some patients have been known to live four to five years with mostly psychological symptoms while the disease progresses. It then causes more physical symptoms that lead to diagnosis, after which death usually occurs within a year.
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