02/19/2012 1 Comment Contact Our News Editors

Huntington’s disease is a rare nightmare.

“It’s a horrible disease,” said Dr. Edward Pegg, a Bloomington neurologist who has treated six patients with Huntington’s in his 26 years of practice. He is not involved with the Tonya McKee case.
“It is one of the most devastating neurological diseases,” he said. “This takes people down in their prime. You lose motor function and you literally lose your mind.

“It’s like having ALS (Lou Gehrig’s disease), Alzheimer’s and psychosis.”
Because Huntington’s is relatively rare — about one in 10,000 to 20,000 people have it — it is a lesser-known disease. There is no cure, but doctors prescribe medicine, therapy and counseling to try to lessen the disease’s broad range of symptoms.

Huntington’s is an inherited disease caused by a defect in one gene. There are theories as to what causes the defect, but no one knows for sure, Pegg said.

If one parent has Huntington’s, each child has a 50 percent chance of inheriting the gene, according to the Mayo Clinic website. “Anyone who gets the gene, gets the disorder,” Pegg said.

That’s why a common cause of death for people with Huntington’s is suicide. “You watched your loved one go through it and you realize it could happen to you,” Pegg said.

Most people with Huntington’s develop symptoms in their 30s, 40s and 50s. The disease damages the basal ganglia — structures deep in the brain that coordinate movement, Pegg said.
As nerve cells in the basal ganglia break down, a person’s ability to move, think and feel is hindered. The brain actually shrinks in size by 25 to 30 percent, Pegg said.

“When you have something that affects so much of the brain, it affects how you move your body parts, your thinking and your ability to have normal emotions,” he said.

Movement disorder symptoms include involuntary jerking (not a repetitive tremor), involuntary contraction of muscles, muscle rigidity, slow and uncoordinated hand movements, abnormal eye movements, impaired gait and difficulty with speech and swallowing, said Pegg and Mayo.

Cognitive disorders include difficulty planning and learning new information, an inability to start a task or conversation, getting stuck on a thought, lack of impulse control that can result in acting without thinking, and sexual promiscuity, falls and lack of awareness of one’s own behaviors.

Psychiatric disorders include depression (including social withdrawal, fatigue, thoughts of suicide), obsessive-compulsive disorder, mania and bipolar disorder.

“A person may become compulsive, paranoid and easily agitated,” Pegg said.
People who begin to experience these symptoms should see their doctor. If a diagnosis is made, doctors will prescribe medicines to lessen some of the movement and psychiatric disorders, as well as therapy and counseling to help the person deal with the physical, speech and psychiatric impairments.
But there is no cure. A person with Huntington’s may live 10 to 30 years following diagnosis. Eventually, the person requires help with all activities of daily living and care. Common causes of death include pneumonia and complications related to the inability to swallow.