Huntington disease (HD) is a hereditary neurodegenerative disorder that is characterized by progressively worsening motor, cognitive, behavioral, and psychiatric symptoms. HD is caused by a mutation of the Huntington gene called a “CAG repeat expansion”. The mutation results in gradual neuronal degeneration in the basal ganglia of the brain, and progresses to involve other regions of the brain responsible for coordination of movements, thoughts, and emotions. Neuronal degeneration causes diffuse and severe brain atrophy that is comparable to late stage Alzheimer disease.
Clinical presentation of HD may include changes in personality, behavior, cognition, speech, and coordination. Physical changes include random uncoordinated extremity movements (chorea), rigidity, leg stiffness, clumsiness, slowness of movement, tremors and muscle spasms. As the disease progresses, concentration on cognitive tasks becomes increasingly difficult, and an individual may have difficulty swallowing and feeding himself. Family history of HD is usually but not always positive.
HD is genetic. Symptoms may show up early in childhood, but in most cases, the first noticeable signs are in a person’s 30s or 40s. There is no cure or treatment for HD. Treatments currently in use are designed solely for the purpose of managing the symptoms of the disease. Death usually occurs at about 15 to 20 years after onset of symptoms and is due to complications of the disease.
The Social Security Administration has long recognized that the length of the disability claims process is problematic, especially for those with terminal or degenerative conditions. Because of this, they began the Compassionate Allowances program in 2008. This program seeks to expedite the process for those whose conditions clearly qualify them as completely disabled.
The SSA began compiling a list of conditions which automatically qualify claimants for Social Security disability benefits. They have steadily added to this list. If you have been diagnosed with adult onset HD, your claim will automatically be flagged for a compassionate allowance. In most cases, your claim will be approved in a matter of two to six weeks.
HD has been approved for listing for Compassionate Allowances, the process of applying for Social Security disability benefits with the condition has become significantly easier. If you have been diagnosed, you no longer wonder whether you qualify for disability benefits, nor do you need to worry about going through a drawn-out approval process.
You will need to include the information pertinent to your adult onset HD with your claim. This will include your doctor’s diagnosis as well as the basis of that diagnosis. In most cases, this will mean including records of any neurological tests, genetic testing, and brain imaging which were used in making the diagnosis.
Other Medical documentation you will need includes:
Claimant’s medical source(s) records documenting progression of motor, cognitive, and psychiatric symptoms and signs; family history of HD; and abnormal neurological exam findings consistent with HD.
Laboratory testing showing a CAG repeat expansion in the HD gene (40 or more CAG repeats).
Brain imaging (MRI) may provide supporting evidence.
Psychological or psychiatric reports including neurocognitive testing.