Corticobasal Degeneration (CBD) is a rare and progressive neurodegenerative disease believed to be related to an abnormal accumulation of a protein called tau in the brain. The exact prevalence of the disease is still unknown, but researchers estimate that it affects 1-5 in 100 000 people.
The tau protein is normally present in the brain, here it plays an important role in the structure and function of the nerve cells. If this protein is biochemically changed it can become dysfunctional and twisted. The nerve cells are unable to expose of this form of toxic tau, so it will accumulate and destroy the affected cells. This mechanism is the cause of several neurodegenerative diseases that takes different forms depending on which part of the brain it affects and consequently degenerates. In CBD, this is primarily the basal ganglia and the cortex.
One of the biggest challenges with CBD today is the impossibility to give a definite diagnosis while the patient is still alive. This, combined with its rarity and nonspecific symptoms, is the reason why CBD often is assumed to be either Parkinsons or Alzheimers disease. A misdiagnosis most unfortunate for patients and their families.
A clinical diagnosis is primarily given by eliminating other neurodegenerative diseases first and is currently only based on certain symptoms associated with CBD. The existing available treatments focus only on alleviating symptoms. Unfortunately, the results are moderate and can only provide short term relief for the patient.