Ataxia is the scientific term for a lack of coordination or clumsiness. The part of the brain most commonly associated with coordination is the cerebellum. According to Social Security, Spinocerebellar Ataxia (SCA) refers to a group of genetic disorders characterized by slowly progressive difficulties with gait, hand movements, speech and abnormal eye movement. Spinocerebellar ataxia is usually inherited, if one of the parents has the disorder, there is about a 50 percent chance that a child will have the disease as well according to many studies.
Spinocerebellar ataxia quickly afflicts the cerebellum. People with SCA have progressive damage in the areas of the brain that control movement in the arms, legs, hands, and eyes. When this type of brain damage occurs, the cells in the part of the brain that controls movement degenerate (atrophy) resulting in ataxia. This disorder causes a slow progression of ataxia of gait, stance, limbs, and speech disturbance with or without movement of the eyeball. The prevalence of SCA’s is estimated to be about 1-4/100,000. Genetic testing and MRI can distinguish genetic from acquired (non-genetic) causes of ataxia. The rate of progression for SCA varies with the gene mutation identified and, in general, is faster with earlier onset or increased length. In SCA1, 2, and 3, time to becoming wheelchair dependent is 13-15 years and time to death is 20-30 years. The prognosis for SCA6 and SCA11 is less severe with a very slow worsening of symptoms, and persons with SCA8 and SCA11 have a normal lifespan. There is currently no cure or treatment to slow the progression of SCA. Medications can help manage the symptoms (stiffness, depression, spasticity and sleep disorders). Occupational therapy can be helpful in developing ways to accommodate the individual in performing daily activities.
Social Security has an obligation to provide benefits quickly to applicants whose medical conditions are so serious that their conditions obviously meet disability standards. Compassionate Allowances (CAL) are a way of quickly identifying diseases and other medical conditions that invariably qualify under the Listing of Impairments based on minimal objective medical information. Compassionate Allowances allow Social Security to target the most obviously disabled individuals for allowances based on objective medical information that we can obtain quickly. Compassionate Allowances is not a separate program from the Social Security Disability Insurance or Supplemental Security Income programs. Michael J. Astrue, Commissioner of Social Security, announced in February 2010 that the agency is adding 38 more conditions to its list of Compassionate Allowances, Spinocerebellar Ataxia (SCA) was officially added to that list. Mr. Astrue stated “there can be no higher priority than getting disability benefits quickly to those Americans with these severe and life-threatening conditions.”
