What is it?

Slow virus infection of central nervous system includes Creutzfeldt-Jakob Disease (CJD) and Kuru disease. CJD is a human prion disease. It is a rare, degenerative, neurological disorder that presents with rapidly progressive dementia and eventually death. The disease is a part of a group called transmissible spongiform encephalopathies (TSEs). In these diseases a protein called prion is produced abnormally. CJD can be transmitted either sporadically or by inheritance- familial CJD. Also, a small number of patients can get contaminated with CJD after being exposed to infected human tissue such as corna or skin transplant., In the 1990s, some people in Britain developed a form of the disease after eating meat from diseased cattle. Risk factors for CJD include older age, familial history, exposure to contaminated tissue. Kuru disease is a rare and fatal disease of the central nervous system. It was common in the highlands of New Guinea, in people who performed cannibalism. The disease’s main symptoms include muscle twitching and loss of coordination. The disease has three stages, a prodrome that includes headache and joint pain followed by three stages in which the body coordination deteriorates, till the third stage in which the person is usually bedridden and incontinent. Kuru is a part of a group of diseases called transmissible spongiform encephalopathies (TSEs), also called prion diseases, such as CJD.

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Signs & symptoms

Creutzfeldt-Jakob Disease (CJD) early symptoms may include personality change, memory loss, impaired thinking, blurred vision, blindness, insomnia, lack of coordination, difficulty to speak or swallow, jerky movements. Later on, symptoms may include coma, heart failure, lung failure, pneumonia and other infections and also death. Symptoms of Kuru include difficulty walking, poor coordination, difficulty swallowing, slurred speech, behavior changes, dementia, muscle twitching, tremors, random laughing or crying.

Diagnosis

CJD diagnosis is done upon findings from physical examination. Other common tests may include EEG, MRI and cerebral spinal fluid examination on a spinal tap- mostly to rule other neurological conditions. A newer test called RT-QuIC can detect the presence of prion protein and help establish the diagnosis. A definitive diagnosis can be done in a brain biopsy. Diagnosis of Kuru is based upon neurological examination, blood tests, EEG, and brain MRI. None of these makes a definite diagnosis.

Treatment

Treatment for CJD and Kuru disease does not exist yet, it mostly consists of supportive treatment such as pain relief.

☝️ This is not a substitute for professional medical advice. Please consult with your physician before making any medical decision.

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