Rare Neurological Disorders


                                           




                                              Rare Neurological Disorders

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, lethal brain disorder. CDJ is caused by an infectious agent called prions that results in rapid degeneration of the host’s brain tissue. It influences around one individual in every one million per year worldwidein the United States there are approximately 350 cases per year.  CJD will have symptoms like ataxia, or difficulty controlling body movements, abnormal gait, speech, and dementia etc.

type of CJD called variation CJD (or vCJD), disease similar to CJD called bovine spongiform encephalopathy (BSE) or, commonly, “mad cow” disease. Causes can be sporadicinherited, or acquired. It mostly affects individuals over the age of 60 years, and it is rare in individuals under 30 years old. CJD gradually destroys brain cells, and it causes tiny holes in the brain

There are three major categories of CJD.

•  In sporadic CJD, the infection appears even though the individual has no known risk factors for the disease
  In genetic CJD, the individual may have a family history of the illness and test positive for a hereditary change related with CJD
   In acquired CJD, the disorder is transmitted by introducing   to brain or nervous system. There's no proved that CJD is infectious through casual contact with somebody who has CJD.
CJD cannot be transmitted through the air or through touching or most other shapes of casual contact. Spouses and other family members of individuals with sporadic CJD have no higher chance of contracting the disease than the common population.
Several tests can help diagnose CJD.
  Electroencephalography (EEG), which records the brain’s electrical pattern, can be especially valuable because it shows a particular type of abnormality in major but not all types of CJD.
   Cerebrospinal fluid-based tests: A new diagnostic test for human prion diseases, called second generation Real Time-Quaking-Induced Transformation (RT-QuIC), which is based on an ultrasensitive detection of the pathogenic prion protein within the cerebrospinal fluid of people affected by CJD and other forms of human prion diseases.
  Magnetic resonance imaging (MRI) - It has recently been found to be accurate in about 90 % of cases.


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