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06-10 Behavioral problems can signal Huntington’s disease

Behavioral problems can signal Huntington’s disease

Ronnie was 14 years old when he beat his little sister with a golf club for getting a hole-in-one at miniature golf. His sister was all right, but Ronnie’s mother grew very concerned about him. His grades were slipping in school and he had to be reminded to do every little thing. His temper was clearly becoming dangerous. An abnormal neurological exam led doctors to discover that Ronnie had juvenile Huntington’s disease (HD).

HD is a devastating illness that causes progressive decline of the mind and body. HD is genetic and usually passed on by the father. Worldwide, there are about 3 to 6 people per 100,000 with the disease, 5 to 10 percent of whom are diagnosed before age 21.

Psychiatric symptoms usually appear first and can be very severe. Personality changes, mood swings, aggression, impulsivity, agitation, inattention, obsessiveness, apathy and sleep disturbances can all be early symptoms of HD. Behavior and cognitive problems are common and worsen over time.

Woody Guthrie, the folk singer who wrote “This Land Is Your Land,” was famously misdiagnosed with alcoholism and schizophrenia when he first started having behavioral symptoms of HD in the late 1940s. Guthrie was finally diagnosed in 1952 based on his advanced symptoms. It wasn’t until 1993 that the gene that causes HD was identified, allowing for earlier and more accurate diagnosis.

A complicated disease

The psychological effects of having received a diagnosis of HD are profound for obvious reasons and complicate the psychiatric symptoms caused by the disease itself. Like those who receive a terminal cancer diagnosis, depression is not uncommon and can be severe. Additional stress and anxiety may also come up following diagnosis over the ethical questions about testing other family members.

Physical symptoms include gait disturbance, involuntary movements, rigidity, seizures and speech regression. These symptoms progress to difficulty swallowing and immobility, with about 90 percent of afflicted individuals eventually dying from aspiration pneumonia. Most people live 10 to 15 years from the time of diagnosis.

Quality of life can be improved with medications and behavioral therapy. Treatment is currently focused on symptom management, although a new medication that “silences” the causative gene is undergoing safety testing in humans. Stem cell therapy may also offer hope for those with this cruel disease. For Ronnie and so many others, a cure can’t come soon enough.

Sometimes, behavioral problems are actually caused by underlying medical problems or substance use. The Sovereign Health Group offers comprehensive evaluation and accurate diagnosis, so that underlying or co-occurring conditions can be treated accordingly. Sovereign Health of Rancho San Diego is a leader in the treatment of adolescent mental illness, substance use and co-occurring conditions. Our programs integrate state-of-the-art neurocognitive treatments with alternative approaches like experiential therapies, while promoting healthy lifestyle habits and lasting recovery. To find out more about specialized programs at Sovereign Health, please call us at our 24/7 helpline.

About the author

Dana Connolly, Ph.D., is a senior staff writer for the Sovereign Health Group, where she translates current research into practical information. She earned her Ph.D. in research and theory development from New York University and has decades of experience in clinical care, medical research and health education. The Sovereign Health Group is a health information resource and Dr. Connolly helps to ensure excellence in our model. For more information and other inquiries about this article, contact the author at news@sovhealth.com.

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