What is PSP?
PSP affects 1:100,000 people
What is PSP?
Progressive Supranuclear Palsy (PSP) is a rare brain disorder affecting about 20,000 Americans. My father was one of those individuals. The disease is characterized by difficulty with gait and balance. Individuals afflicted with the disease also tend to have problems controlling their eye movements and may have mild dementia and mood changes. There is no cure and symptoms worsen as the disease progresses. My extremely simplistic explanation of PSP is that it is a “cousin” of Parkinson’s disease.
What Causes PSP?
PSP symptoms are caused by the deterioration of cells along the brain stem and lesions on the portion of the brain controlling eye movement. The disease itself is not fatal. PSP patients usually die from other causes, such as pneumonia.
The most common early symptoms of PSP include the following:
1. Difficulty with balance. The patient may have an unsteady gait and difficulty walking or unexplained falls.
2. Vision changes. The patient may complain of blurred vision or have difficulty shifting gaze up or down.
3. Slurred Speech and difficulty swallowing (dysphagia). PSP affects motor control including muscles used for speech and swallowing.
4. Mood and behavioral changes. PSP patients may experience symptoms such as depression, outbursts of anger, and memory problems.
Dad’s symptoms began many years before he passed away. I remember him having a slight tremor that would come and go. He took to using a cane because he had a tendency to fall. Dad also would get more agitated in stressful situations and have occasional outbursts of anger. He was also prone to bouts of depression, but we usually attributed those to other causes. Doctors initially diagnosed Dad with Parkinson’s and started him on the traditional Parkinson’s medications. I don’t remember Dad complaining of vision problems early in the disease process; however, as it progressed he did have the distinctive PSP upward gaze.
Key Differences between PSP and Parkinson’s
PSP and Parkinson’s are similar in that they both cause body stiffness, difficulty with movement and gait, and a tendency to fall. Both diseases tend to manifest in late middle-age. Some key differences between the diseases include:
1. PSP patients usually stand straight or with their head tilted back. Parkinson’s patients tend to lean forward.
2. Parkinson’s patients normally fall forward due to the tendency to lean forward. PSP patients, on the other hand, tend to fall backwards due to their upwards gaze.
3. PSP patients are more prone to dysphagia.
4. Abnormal eye movements are more common in PSP than with Parkinson’s
5. Tremors are common with Parkinson’s but rare with PSP. My father did have a tremor of sorts; however, it seemed different to me than the traditional Parkinsonian tremor. Dad would seem to get locked in a repetitive motion. For example, Dad would start rubbing his fingers on the wheels of his wheel-chair and continue until his fingers were raw and bloody. It was like his brain was unable to send the message to his body to stop.
There is no medical treatment for PSPClick thumbnail to view full-size
Treatment for PSP
There is not an effective treatment for PSP at this time. Treatment primarily focuses on non-medical management of gait, vision, and swallowing problems. PSP patients may benefit from various walking aids to prevent falls, special glasses to combat vision problems, and feeding tubes to prevent aspiration related to dysphagia.
Prognosis for PSP Patients.
PSP patients may live for a considerable time after initial diagnosis. It is common for a PSP patient to live with the disease for a decade or more. Patients are prone to fall-related injuries. The majority of PSP patients die from pneumonia related to their difficulty swallowing. It may be necessary for the patient to have a feeding tube to ensure proper nutrition and prevent aspiration.
My father lived with PSP for more than 10 years, passing away March 3, 2003. During that time he also suffered a heart attack and had both legs amputated due to gangrene. He became totally wheelchair-bound. Being in bed or a chair 24/7 did make Dad susceptible to other problems such as skin-breakdowns. Dad eventually became unable to take any food or liquids by mouth and had to have a feeding tube inserted. He also quit speaking altogether due to the difficulty and frustration. It was heartbreaking to see Dad slowly taken away piece by piece. His body was betraying him, but I could tell his mind was still working. For example, he would smile or laugh at appropriate spots while watching TV or smile when we would tell a funny story. I would imagine him trapped inside his body and screaming on the inside to be released.
© 2015 Vicki Holder