What is CBGD?
CBGD stands for Cortical Basil Ganglionic Degeneration. CBGD ia a rare neurological disease that causes parts of the brain to deteriorate. Although it may be considered a 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 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, the disease will progress slower or faster in some individuals over a course of 5 to 8 years. It affects both men and women and crosses racial/cultural lines. A person with CBGD will usually become immobile due to rigidity within five years of noticeable symptoms. 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.
Causes: The cause of CBGD is unknown and so far there is no evidence to suggest that it 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 compared to non-neuronal cells The tau’s main function is to modulate the stability of axonal microtubules.
(axonal is a long slender projection of a nerve cell or neuron).
(microtubule is a hollow cylindrical tube that is used to transport material/nutrients inside cells)
Now that I’ve explained to the best of my ability what CBGD is why should you care?
CBGD is considered a rare neurological disorder rather than a syndrome. There is very little to be done to ease the suffering. Even though some of the symptoms may be similar to Parkinson’s, the therapies used for Parkinson’s don’t work for CBGD. Although it is not certain if this disease is hereditary, (We don’t know of anyone on my father’s side who had this) there is also no way for doctors to ascertain for certain if it is CBGD until after death and an autopsy is performed. My question then is this, how many people does it take for CBGD to be considered rare or not and when will more research be conducted?