DEAR DR. GOTT: My son was diagnosed with PSP — progressive supranuclear palsy. I have never heard of this before. He was originally told that he had Parkinson’s. Is there any hope for him? He is 44 years old. I have read as much as possible on this condition and don’t see any relief for him. He falls, slurs his speech, and has difficulty with pain in his legs. These don’t happen all the time, though; some days he is “normal.” Thank you.
DEAR READER: Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is a rare brain disorder. As the name states, it is progressive, meaning it slowly worsens over time. The condition can cause some life-threatening complications such as swallowing difficulty, which may lead to choking or pneumonia.
The characteristic symptoms include loss of balance when walking, which causes an increased risk of falls and an inability to focus the eyes properly, which can result in blurred or double vision. This can also cause the sufferer to appear disinterested during conversation. Other symptoms include forgetfulness, dizziness, personality changes, apathy (loss of interest in previously pleasurable activities), depression, anxiety, laughing or crying without reason, problems with speech and swallowing, slow or stiff movements, tremor, loss of coordination and more. PSP is most often seen in people around age 60. Men are slightly more at risk than women.
There is no known cause of PSP, but research is bringing us closer. We now know that the symptoms are the result of deterioration of brain cells in four areas of the brain — the basal ganglia, cerebral cortex, cerebellum and brainstem. Each of these areas is responsible for helping control body movements. It has also been found that the deteriorated cells contain abnormal levels of the protein tau, which is also seen in other neurodegenerative disorders.
There is no cure, but some of the symptoms are treatable. Medications used to treat Parkinson’s disease as well as certain antidepressants may be helpful. Special eyeglass lenses and physical and occupational therapy are often beneficial. Even with treatment, brain function will continue to decline, with death commonly occurring within five to seven years.
Your note is brief. Without knowing what testing your son has undergone, when his symptoms began and more, I cannot hazard a guess as to whether this may be an accurate diagnosis. He is relatively young to have received the diagnosis, but that doesn’t mean it is incorrect. Pain is not listed as a typical symptom in this syndrome, but it could be the result of muscle stiffness, injury from repeated falls or his unsteady gait. I did not find anything stating that symptoms come and go. Furthermore, you did not mention difficulties with his eye control, a somewhat characteristic feature of PSP. Progressive supranuclear palsy is not a pleasant diagnosis, and the prognosis is fairly grim. I trust his physician’s abilities, but based solely on your brief note, I can’t be sure of his current diagnosis. I urge your son to undergo a thorough physical examination, blood work and imaging studies with another neurologist in order to confirm the diagnosis. If possible, seek out a specialist affiliated with a teaching hospital or university. Better still, I suggest he make an appointment with a physician who specializes in PSP. Help may also be found at www.PSP.org.