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Six Things Every Family and Sufferer Need to Know About Progressive Supranuclear Palsy (PSP)

Published: October 1st, 2011

By: Nikolaus McFarland, MD, PhD

Category: education, MDC

6 Things Every Family and Sufferer Need to Know about PSP

  1. How do I know if I have PSP and not just Parkinson disease or “Parkinsonism?”

    Parkinsonism actually is not a diagnosis, but rather what we doctors use to describe the classic symptoms of Parkinson disease. These include 1) tremor—usually resting, 2) stiffness or rigidity, 3) slowed movement (bradykinesia), and 4) gait and postural instability. There are many causes of Parkinsonism, such as drugs, stroke, or hydrocephalus. However, the most common cause is Parkinson disease, which is a progressive neurodegenerative disorder characterized mainly by loss of dopamine cells in the brain. Closely related neurodegenerative disorders that cause Parkinsonism include PSP (and others, such as Lewy body dementia, multiple system atrophy, and corticobasal degeneration). PSP shares many features with Parkinson disease, but patients classically present with early onset gait instability and frequent falls in the first year or two of disease. Additional symptoms include decline in voluntary eye movements and facial expression (often leading to a stare-like look), slowing of movements, speech and swallow difficulty, and change in cognitive function and even emotion. In contrast to Parkinson disease, PSP symptoms have a limited response to typical Parkinson medications such as carbidopa/levodopa and can progress more rapidly. To be sure of your diagnosis, seek a neurologist familiar with Parkinsons and ask for specialist with movement disorders training, such as you will find here at the UF Center for Movement Disorders & Neurorestoration.

  2. Are there any treatments for PSP? What’s on the horizon?

    Unfortunately there is still no one pill that currently effectively treats or can “cure” PSP symptoms. Sinemet (carbidopa/levodopa) may be tried for the Parkinsonism and in some patients helps, but often the response is limited and higher doses only cause side effects. If stiffness/rigidity is severe, muscle relaxants sometimes are helpful. Other medications are used based on specific symptoms, such as scopolamine for drooling, antidepressants for mood, and sometimes drugs for cognitive dysfunction.  The mainstay of treatment, however, is primarily supportive and involves multiple disciplines, including physical, occupational, and speech therapists. There is hope around the corner though, with active research and several ongoing clinical trials (see http://www.clinicaltrials.gov/ct2/results?term=progressive+supranuclear+palsy) such as the Davunetide drug trial.

  3. I am falling frequently. What should I do?

    Falls are one of the leading causes of injury, disability, and even death in the elderly. Evaluation early on by a trained physical therapist, knowledgeable in Parkinsonism, is critical to help with gait, balance, and prevent falls. Assistive devices such as a walker may be prescribed early on and exercises to help with balance and walking. It is imperative to be followed and assessed routinely as disease symptoms to progress and needs will change. In addition, consider a home safety evaluation to help look for things like loose rugs and stairs which may be sources of falls.

  4. When should we consider placement of a G-tube for feeding?

    Swallow difficulty can be a significant problem for patients with PSP. Routine formal swallow evaluation is critical and should be performed about every 6 months or more often as needed. There are special swallow techiques that can be learned, as well ways to modify one’s diet to prevent choking. The main concern is aspiration, or swallowing something down the wind-pipe, which can cause a reactive (and even infectious) pneumonia. Pneumonias of course are a leading cause of death in patients. At some point late in the disease, swallowing may become very difficult and the risk of aspiration too high. It is vitally important that before that happens you should discuss with your doctor your wishes regarding a feeding tube. The choice is personal and there are several factors to consider. A feeding tube (gastric or G-tube) is placed though a simple surgical procedure. It can provide access for supplemental feeding, nutrition, liquids and medications. Eating for pleasure may still be possible as long as the risk is low. A feeding tube thus can potentially prolong life and health. However, a feeding tube will not stop disease progression in PSP, and the risk of aspiration is still there due to potential reflux (which can be reduced by sitting or keeping the head of bed up or raised by about 45 degrees).

  5. Ups and downs, “roller coaster” emotions

    In PSP emotional lability, or pseudobulbar affect, may occur and is characterized by sudden or inappropriate laughter or inconsolable crying. Other mood disturbance may occur including depression and apathy, but these “mood swings” can be quite disconcerting both for family members and the patient. Treatment often initially includes antidepressants. Additional therapy may include a dextromethorphan (a common medication in cough suppressants) and quinine which have been shown to be effective.

  6. Where do I get support for my family member with PSP?

    Start with your doctor. It is important that you always communicate your needs and concerns. We here at the UF Center for Movement Disorders & Neurorestoration are an excellent resource for patients with PSP, both clinically and for research, as well as for information and support. We have an interdisciplinary group interested in helping you and your family member with PSP. Increasingly, we also now have a network of patients and family members who are open to discussing their experiences with caring for someone with PSP. In addition, the CurePSP organization (www.psp.org) is a wonderful source of information online and includes an active internet support group. There are also other local PSP support groups that we may be able to refer you to for further information.

 

About the Author

Nikolaus McFarland

Nikolaus McFarland, MD, PhD

Assistant Professor of Neurology

Dr. Nikolaus McFarland is a movement disorders expert with special interest in atypical parkinsonisms/Parkinson-plus disorders. He directs the PSP/Atypical Parkinson's clinical-research program here at UF. He has a research lab that focus…

Find more posts by Nikolaus McFarland, MD, PhD »

3 Responses to “Six Things Every Family and Sufferer Need to Know About Progressive Supranuclear Palsy (PSP)”

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  1. Sharon Younkers

    It took over four years to diagnose my husband’s PSP. Neurologists at Mayo and, St. Vincent’s missed it as did a number of various private practice neurologists. The doctor at Mayo told my husband to get out of his wheelchair and walk. The doctor at St. Vincent’s kept leaning with Parkinsonism but did not provide follow-up to our calls or those of our primary care doctor. However, our primary care doctor directed us to Shands and subsequently, Dr. Oguh, who recognized PSP immediately. Unfortunately, she had seen very few cases and thought my husband could/would live another year or more. He died December 24, 2012. His death and suffering were horrific and Hospice dropped the ball by not providing pallative care as promised. At least we knew the diagnosis before he died. Not all physicians are equally knowledgeable about all diseases, especially such a rare one like PSP. Good luck & God Bless if you or a loved one suffers from PSP. Thank you Shands and Dr. Oguh.

  2. Nikolaus McFarland Author

    I appreciate your comment. Your experience is certainly distressing to hear and I hope not to be repeated. Our Neurology department in fact has had a growing and very strong movement disorders presence at UF for years. Referral for PSP should have been straightforward.

    That said, I came to UF 2 years ago and my recruitment was in part to create an interdisciplinary program and to forward a new initiative for atypical parkinsonisms, PSP in particular. The message is getting out. Our new clinic now is seeing many more referrals for PSP, and I really believe we are making a difference. I have also worked to get our message out in the state and beyond. Research too is a big focus at UF as we need to understand this devastating disorder better and find new treatments with a cure in mind. This year we actually held the first of a hopefully Annual PSP Symposium/Think Tank with over 100 attendees. The goal was to forward research and increase awareness of PSP. The symposium was quite successful and our second PSP Symposium is scheduled for next Spring.

    Please keep your comments coming and I will continue to do my job to get the message out about PSP, and other atypcial parkinsonisms, at UF and beyond. Our program has lots to offer, and we definitely want to see these patients and help in any way we can.

    Sincerely,
    Dr. McFarland

  3. Lynn Parkis

    I am surprised to find that UofF has so much information and support for PSP and CBS — as, when my husband presented the very symptoms mentioned here, no one he saw at SHANDS ever mentioned Parkinsonism, PSP or CBS. All we heard was, “It’s not MY specialty. Good bye.” Perhaps the educational information mentioned above should be sent to all Neuro doctors so that patients like my husband don’t lose valuable months without constant therapy, while trying to find someone who can diagnose this condition. A doctor at Baltimore’s Johns Hopkins reviewed the same information we gave SHANDS, and gave us a diagnosis, so that we can now seek therapy that may slow my husband’s digression. I resent those months lost, as they may shorten the time I have left with my husband. PLEASE, share information between “Specialties” so that others don’t go the same route we’ve had.

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