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 worldwide; in 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.
A type of CJD called variation CJD
(or vCJD), a disease similar to CJD called bovine spongiform encephalopathy
(BSE) or, commonly, “mad cow” disease. Causes can be sporadic, inherited, 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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