Striatal degeneration, autosomal dominant, or ADSD, is a rare genetic disorder that is mainly characterized by mild problems articulating speech and a loss of muscle movement. Other symptoms of ADSD include slowing of movement, trouble with quick alternating movements, increased muscle tone, stiffness of the tongue, and problems swallowing. The symptoms usually begin to appear between the ages of 40 and 50 and may become worse over time.
The symptoms of ADSD are caused by a problem involving changes with the basal ganglia, the part of the brain that controls movement coordination. The condition is usually diagnosed by looking at the basal ganglia using brain scans. Sometimes, ADSD may be mistaken for degenerative diseases like Parkinson’s, but this kind of disease involves tremors, while ADSD does not. Doctors can make a definite diagnosis of ADSD by looking at brain scans.
ADSD is inherited in an autosomal dominant manner. We inherit our genes in pairs, typically one from each parent. Our genes are what control the growth, development, and function of our bodies. If a mistake or error occurs in a gene, it can cause problems. Autosomal dominant means an individual inherits only one copy of the changed gene, or mutation, in order to have the condition. If both parents carry one copy of the changed gene, they have the condition, and their children will have a 75% chance of having the condition. If only one parent carries a copy of the changed gene, their children have a 50% chance of having the condition.
There is currently no known treatment for ADSD, but individuals with the condition usually live normal lives and have a normal life expectancy. If you or a family member has been diagnosed with striatonigral degeneration, autosomal dominant, talk with your doctor about the most current treatment options. Support groups are also good resources of support and information.
Description Last Updated: Feb 18, 2018