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Dudley Moore Fights PSP
Actor Dudley Moore, known for his comic performances in 10, Arthur, and other films, is being treated for progressive supranuclear palsy (PSP), a rare, degenerative brain disorder affecting motor skills and vision. Since May, 1999 he has been under the care of Lawrence I. Golbe, MD, professor of neurology at UMDNJ-Robert Wood Johnson Medical School (RWJMS). Golbe, an internationally recognized expert on PSP, is in the Division of Movement Disorders in the Department of Neurology at RWJMS, where Moore is currently enrolled in a clinical trial of the drug pramipexole. The drug, a treatment for Parkinson's, may also help control symptoms of PSP and slow the rate of brain cell degeneration. The trial is at seven centers across the U.S. and Canada. Moore is also undergoing therapy at Kessler Institute of Rehabilitation to improve both physical and cognitive function. PSP is caused by progressive damage to a group of cells in the midbrain and other brain areas. The earliest and most common symptom is loss of balance while walking, often resulting in falls. Other symptoms include forgetfulness, changes in personality, slurring of speech, slow movements, and difficulty swallowing. The loss of balance and slurred speech sometimes lead to a misdiagnosis of alcoholic intoxication. (Ironically, Moore's pratfalls have always left movie audiences laughing, and one of his most memorable roles was that of an alcoholic in Arthur.) Problems with eyesight also develop, and are in fact a primary tool in diagnosing PSP. "Supranuclear" refers to the area of the brainstem that controls up and down eye movements. While some patients describe their vision as blurred, the actual problem is an inability to aim the eyes properly because of weakness or paralysis of the muscles that move the eyeballs. Patients with PSP are unable to coordinate eye movements for reading or maintaining eye contact. The 64-year-old actor is in the early stages of the disease, but has been feeling its effects for five years. Moore, who is also a concert pianist, experienced a slowing and lack of coordination of his hand movements. Because he had few other signs of PSP, a diagnosis at first eluded physicians. Some suspected he suffered from mild strokes or another unidentified degenerative condition. Eventually Moore began having problems with balance. In 1998, he was referred to Martin Gizzi, MD, PhD, clinical assistant professor of physical medicine and rehabilitation at RWJMS. Gizzi, who has tested methods for distinguishing PSP from Parkinson's disease by recording eye movements, made the diagnosis in 1998. Some 20,000 people in the U.S. are estimated to have PSP, but only 5,000 have been diagnosed. Because the early signs of the disease are subtle, a diagnosis is often delayed for several years. The disease strikes 1.4 in 100,000 people, typically middle-aged and elderly adults. Slightly more men than women are affected. PSP is related to Parkinson's disease, and has similar symptoms. However, unlike Parkinson's, there is no effective treatment for PSP. Several medications used to treat Parkinson's disease can help some patients with PSP, but in general, PSP does not respond as well. Golbe, who is chair of the medical advisory board for The Society for PSP, the leading organization for PSP research in the U.S., is studying the genetics of the disease. Although fewer than one in 100 patients with PSP knows of another case in the family, there is evidence of a subtle genetic component to the cause of PSP. A protein called "tau," which helps maintain the structure of brain cells, shows signs of "clumping" in PSP patients. The connection between this "clumping" and the loss of brain cells is being examined. Moore went public about his illness in October to raise awareness about PSP and help others with it who remain undiagnosed. |
