Definition

Huntington’s disease (HD) is an inherited, neurodegenerative genetic disorder that causes the progressivebreakdown of nerve cells in the brain. It affects muscle coordination and leads to cognitive decline and behavioral changes. Symptoms typically manifest themselves in mid-adult life. The disease was formerly known as, “Huntington’s Chorea” because symptoms commonly include abnormal, involuntary writhing movements.

General information

Huntington’s disease causes movement, cognitive, and psychiatric problems in affected people, with a wide spectrum of signs and symptoms. Initial symptoms vary greatly. The disease is more common in people of western European descent than in those of Asian or African ancestry, but affects both men and women.

The disease is caused by a genetic defect on Chromosome 4. The defect causes a part of DNA, called a CAG repeat, to occur many more times than what is normal. This section of DNA should repeat itself 10 to 28 times. In people with Huntington’s disease, it is repeated 36 to 120 times. The mutation is dominant, which means if one of your parents has the disease, you have a 50 percent chance of getting the gene. If you get the gene from both your parents, you will develop the disease at some point in your life. Physical symptoms of HD can begin at any age from infancy to old age, but usually begin between 35 and 44 years of age.

As the gene is passed down through families, the number of CAG repeats tends to get larger. The larger the number of repeats, the higher your chance of developing symptoms at an earlier age. Therefore, as the disease is passed along in families, symptoms develop at younger and younger ages. The number of CAG repeats also determines the severity of symptoms. People with fewer repeats may have mild abnormal movements later in life and slow disease progression. Those with a large number of repeats may be severely affected at a young age.

There is no cure for Huntington disease and no known way to stop the progression of the disease. The goal of treatment is to slow the symptoms and help the person function for as long as possible. As the disease progresses, the person will need assistance and supervision, and may eventually need 24-hour care. Death usually occurs 15-20 years after the onset of the disease. The cause of death is often infection. Suicide also is common.

Social Security Administration Medical Listing 11.17 – Degenerative disease not listed elsewhere, such as Huntington’s Chorea.

Adult-Onset Huntington’s Disease is listed under the Social Security Administration’s (SSA) Compassionate Allowance Program, which was launched in 2008 to expedite certain disability claims. Applying for disability benefits under the Compassionate Allowance Program requires the same procedure every applicant must follow when applying for SSA disability benefits; however, you will be notified if your condition is being considered as a compassionate allowance.

Since Huntington’s disease is an inherited disorder, a diagnosis of the disease is based primarily on your answers to questions, a general physical exam, a review of your family’s medical history, and neurological and psychiatric examinations.

For the purposes of 11.17, documentation of the diagnosis must be by generally accepted methods consistent with the prevailing state of medical knowledge and clinical practice. The evidence should include documentation of a clinically appropriate medical history, and neurological findings consistent with the diagnosis.

Your representative may be able to assist in expediting your claim if you have been diagnosed with Huntington’s Disease.