Cortico Basal Ganglionic Degeneration
Cortico Basal Ganglionic Degeneration is a rare progessive degenerative disorder that is a 4-repeat taupathy that causes parts of the brain to deteriorate. Although it may be considered an syndrome rather than a disease.The outer layer of the brain is especially effected. As the disease progresses it affects deeper regions of the brain. Including the region basal ganglia. The basal ganglia is a group or cluster of nerve cells that are interconnected with the cerebral cortex, thalamus and brainstem. These cells play an important part in producing and controlling movement for the body.
Symptoms begin around the ages approx between the ages of 50-70. It usually starts on one side of the body but will eventually spread to both sides. The symptoms are similar to Parkinsons such as poor coordination, abnormal muscle postures, stiffness, jerkiness, slowness, halting speech and difficulty swallowing. Depending on the individual, I the rate of progression will be slower or faster over a course of 5 to 8 years. It affects both men and women and crosses racial/cultural lines. A person with CBD will usually become immobile due to rigidity within five years of symptom Most often, within ten years of diagnosis ,a patient will usually succumb to life threatening complications such as pneumonia or other bacterial infections rather than from the disease itself.
The cause of CBGD is unknown and so far there is no evidence to suggest that is an genetically inherited disease. Studies have shown that those with CBGD show changes in certain cell characteristics. It is noted that these changes include a protein called Tau.
Tau protein is a highly soluble microtubule-associated protein (MAP). In humans, these proteins are mostly found in neurons. One of tau’s main functions is to modulate the stability of axonal ( axonal is a long slender projection of a nerve cell or neuron) microtubules ( microtubule is a hollow cylindrical tube that is used to transport material/nutrients inside cells).
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