Department: Ask the Experts
Lawrence I. Golbe, M.D., is professor of neurology at the University of Medicine and Dentistry of New Jersey-Robert Wood Johnson Medical School. He is also director of Research and Chair of the Scientific Advisory Board of CurePSP, the Society for Progressive Supranuclear Palsy.
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Q My husband was just diagnosed with progressive supranuclear palsy. Is there any treatment for this disease?
A Progressive supranuclear palsy is a rare brain disorder that causes serious and permanent problems with control of gait and balance. People with this disease may also experience changes in their mood and behavior (such as depression and apathy), as well as difficulty with organizing mental tasks and dealing with abstractions. These problems slowly worsen over time. Eventually, most people also develop problems with swallowing and eye movement.
Figure. DR. LARRY GO...Image Tools
The drugs levodopa-carbidopa (Sinemet) or amantadine (Symmetrel) may provide modest and temporary relief for the limb stiffness that interferes with movement. However, dosages of amantadine beyond 100 mg twice a day may cause confusion, and if there is no benefit within a week, you should stop taking it. The levodopa-carbidopa should be titrated up (meaning the doctor will increase the dosage level until it produces an effect) to at least 1,200 mg per day before concluding that it is ineffective.
Donepezil (Aricept) or other “memory drugs” intended for Alzheimer's disease may help with dementia symptoms. Depression may be treated with antidepressants, sleepiness with modafinil (Provigil), and insomnia with a sleeping medication such as zolpidem (Ambien)—however, it usually is not a good idea to take modafinil and zolpidem together because they have opposite effects. The urinary urgency may respond to tolterodine (Detrol) and oxybutynin (Ditropan), though these drugs often worsen the constipation that comes with the disease.
A swallowing evaluation, during which a doctor will usually take an x-ray of the person swallowing, should be performed as soon as he or she starts to cough on liquids. The disinhibition that is often part of this disease is a result of degeneration of the brain's frontal lobe and may cause people to overload the mouth or to suddenly stand and run while eating.
While physical therapy helps little with balance, it can help doctors determine the best devices to aid gait and can be also be used to prescribe a safe exercise regimen to improve joint flexibility.