The State of Dystonia


Dystonia“You can’t buy a ticket to dystonia,” Billy McLaughlin jokingly tells audiences, but he certainly knows what it’s like to live there. The movement disorder that sounds like a tiny country in Eastern Europe is in fact a dreaded diagnosis for musicians. Approximately one in 200 of them are afflicted with focal dystonia (FD), a movement disorder that causes muscles to contract and spasm involuntarily. Roughly 300,000 people in the US suffer from some form of it. Sometimes for a musician, the condition arrives gradually, often in the form of muscle stiffness, spasms and/or twitching. A note is missed or a finger slips—and it can prove devastating to a professional musician’s career.

Dystonia is one of many movement disorders that include diseases such as Parkinson’s, and it’s categorized as either primary or secondary. “Primary dystonia is inherited, meaning at least one gene has been discovered,” explains E. Thomas Chappel, MD. “Secondary dystonia is due to damage to a part of the brain called the basal ganglia, where most movement disorders arise. This damage can result from trauma, stroke, tumor, infection or toxicities.”

FD occurs when the basal ganglia fails to transmit information properly to a given body part, resulting in uncontrolled movements or spasms. While there are different forms of dystonia, from generalized dystonia, which affects many body parts simultaneously, to cervical, the most common form—FD is task-specific, meaning it occurs only when doing often repetitive tasks, such as writing or playing music. Dystonia’s underlying causes remain something of a mystery, despite the fact that it’s the third most common movement disorder in the US. A progressive disease, it affects men, women and children of all ages.

While treatments exist similar to those used for other movement disorders, such as botulinum injections, oral medications, surgery, physical and occupational therapies, full recovery is rare. Medications used to enhance or replace the brain chemical dopamine, which is deficient in the basal ganglia of patients with movement disorders, are sometimes effective. Surgical treatment of movement disorders involves actually destroying areas of the brain that increase the symptoms, or placing a stimulator in an area that will diminish the symptoms.

Unlike Parkinson’s, dystonia can be hard to pinpoint, and it can take years before patients are correctly diagnosed. In fact, up until the mid-20th century, patients with dystonia were often diagnosed as having hysteria, meaning the condition was considered a psychogenic disorder. Today, it takes on average three to five years for a patient to receive a diagnosis, which is down from five to eight years, according to Janet Hieshetter, executive director of the Dystonia Medical Research Foundation (DMRF). The key to an accurate diagnosis, however,

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is finding a movement disorder specialist. Although more treatment options are available now than in the past, research on dystonia remains painstakingly slow as Hieshetter acknowledges. “There are some new treatments on the horizon, getting the pharmaceutical industry to commit to and follow through on clinical trials is tough, but we’re very optimistic.”

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McLaughlin, whose career was derailed with the onset of FD, now crusades to raise awareness of dystonia. He’s been the subject of the award-winning documentary by director Suzanne Jurva, Changing Keys—Billy McLaughlin and the Mysteries of Dystonia, which chronicles his emotional and physical odyssey. In 2010, the DMRF named him their first Ambassador for Public Awareness. He’s also a recipient of the American Academy of Neurology’s 2010 Leadership in Public Neurology Award, an accomplishment he shares with Julie Andrews, Michael J. Fox and Paul Allen.

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