Creutzfeldt-Jakob, disease

• Article
• 2021-03-07

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disease. Symptoms usually start around the age of 60. There are three main categories of CJD: sporadic (the most common form, where people have no known risk factors for the disease); hereditary (where the person has a relative with the disease and tests positive for a genetic mutation associated with CJD), and acquired (where the disease is transmitted through exposure to brain and nervous system tissue, usually through certain medical procedures). A form called variant CJD can be obtained by eating meat from cattle affected by a disease similar to CJD called bovine spongiform encephalopathy (commonly referred to as "mad cow disease"). CJD cannot be transmitted by air or by touch or most other forms of accidental contact. Early symptoms of CJD include problems with muscle coordination, personality changes including progressive and impaired thinking and judgment, vision problems that can lead to blindness, and involuntary twitching called myoclonus. People eventually lose the ability to move and speak and fall into a coma. Tests that help diagnose CJD include electroencephalography (which records the brain's electrical pattern), detection of certain proteins in the fluid surrounding the brain and spinal cord, and magnetic resonance imaging. The only way to confirm a diagnosis of CJD is through a brain biopsy or autopsy. A brain biopsy is not recommended unless it is necessary to rule out a treatable condition. CJD belongs to a family of diseases known as prion diseases - derived from "protein" and "infectious."

Therapy

There is no treatment that can cure or control CJD, although studies on a variety of drugs are currently underway. Current treatment aims to relieve symptoms and make the person as comfortable as possible. Opiate medications can help relieve pain, and the drugs clonazepam and sodium valproate can help relieve involuntary muscle twitching. Intravenous fluids and artificial nutrition may be required in later stages of the disease.

Prognosis

CJD is rapidly progressive. About 70 percent of people die within a year. In the early stages of the disease, people can have poor memory, behavioral changes, lack of coordination, and visual disturbances. As the disease progresses, the mental decline becomes pronounced and people can develop involuntary movements, blindness, and limb weakness. People eventually lose the ability to move and speak and fall into a coma.

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