Hereditary Spinal Ataxia and Receiving Social Security Disability
Hereditary spinal ataxia is an inherited disease that results in progressive damage to your nervous system. This damage affects your spinal cord and the nerves that control muscle movement in your legs and arms. Ataxia is usually a sign or symptom of coordination problems, such as unsteadiness or awkward or clumsy movements. Ataxia is present in many different diseases and conditions. The ataxia in hereditary spinal ataxia results from the degeneration of nerve tissue in your spinal cord. In particular, this is your sensory neurons that are required (through connections with your cerebellum) for directing muscle movement of your legs and arms. Hereditary spinal ataxia causes your spinal cord to become thinner and nerve cells lose some of their myelin sheath (the insular covering on some nerve cells that helps conduct nerve impulses). Hereditary spinal ataxia affects around 1 in 50,000 people in the United States. This disease affects men and women equally. As mentioned earlier, hereditary spinal ataxia is an inherited disease. It results from an abnormality in one of your genes, called X25, located in the ninth chromosome pair. The pattern of inheritance is what is known as autosomal recessive. What this means is that hereditary spinal ataxia can only occur when you inherit the defective (faulty) gene from both of your parents. If you get a defective gene from only one of your parents, you become a carrier of the disease. The signs and symptoms of hereditary spinal ataxia usually start between the ages of 5 and 15. However, with late onset of the disease, signs and symptoms may begin in your 20s or 30s. There are several signs and symptoms that you may have with hereditary spinal ataxia. Some of these include:- Your feet becoming deformed and rigid
- Muscle weakness in your arms and legs
- Diabetes
- Paralysis of a lower limb
- Hearing loss
- Curvature of your spine
- Vision impairment
- Heart disorders
- Slurred speech
- Problems swallowing
- Trembling when you are standing still
- Progressive weakness of your legs that may appear as a staggering, lurching gait when you are walking
- Clumsiness
- Partial loss of your sensitivity to pain and temperature or your sense of touch
- Loss of coordination.
