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Creutzfeldt Jakob Disease - Causes, Symptoms and Treatment


Creutzfeldt-Jakob disease ( CJD ) is a very rare and incurable degenerative neurological disorder ( brain disease ) that is ultimately fatal. Among the types of transmissble spongiform encephalopathy, it is the most common. It affects about one person in every one million people per year worldwide; in the United States there are about 200 cases per year. Typically, onset of symptoms occurs at about age 60. There are three major categories of CJD:  sporadic CJD, hereditary CJD, and acquired CJD. There is currently no single diagnostic test for CJD. The first concern is to rule out treatable forms of dementia such as encephalitis or chronic meningitis. The only way to confirm a diagnosis of CJD is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small piece of tissue from the patient's brain so that it can be examined by a neurologist. Because a correct diagnosis of CJD does not help the patient, a brain biopsy is discouraged unless it is need to rule out a treatable disorder. While CJD can be transmitted to other people, the risk of this happening is extremely small.

Creutzfeldt-Jakob disease is a rapidly progressive viral disease that attacks the central nervous system, causing dementia accompanied by neurologic symptoms such as myoclonic jerking, ataxia, aphasia, visual disturbances, and paralysis. Creutzfeldt jakob disease have been found among Libyan Jewish immigrants to Israel, residents of Czechoslovakia, and North African immigrants to France. Most die of pneumonia within 3 to 12 months of onset of symptoms; 5 to 10 percent survive for 2 years or more. A new variant of creutzfeldt jakob disease emerged in Europe in 1996. Symptoms of CJD include forgetfulness, nervousness, jerky trembling hand movements, unsteady gait, muscle spasms, chronic dementia, balance disorder, and loss of facial expression. CJD is classified as a spongiform encephalopathy. Most cases occur randomly (sporadically), but inherited forms exist. There is neither treatment nor cure for CJD. Other names for CJD include Creutzfeldt-Jakob syndrome, Jakob's disease, and spastic pseudoparalysis.

Symptoms of Creutzfeldt Jakob Disease

Some of the common sign and symptoms of the disease Creutzfeldt Jakob Diseaseare as follows

  • Memory failure.
  • Impaired judgment.
  • Language and comprehension disturbances.
  • Poor judgment.
  • Disorientation .
  • Dementia.
  • Personality changes and psychosis.
  • Hallucinations.
  • Behavioral changes.
  • Difficulty concentrating.

Treatment of Creutzfeldt Jakob Disease

  • Treatments are available to alleviate some symptoms of Creutzfeldt-Jakob Disease, such as morphine for muscle pain.
  • Antenatal screening may be possible in those with the rarer inherited type of Creutzfeldt-Jakob Disease.
  • The medication, called pentosan polysulphate (PPS) and used to treat interstitial cystitis , is infused into the patient's lateral ventricle within the brain. PPS does not seem to stop the disease from progressing.
  • A neurologist or a psychiatrist is normally the primary consultant for Creutzfeldt-Jakob Disease. Physical therapist may also be required.

 

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