What is Progressive Supranuclear Palsy (PSP)?
Progressive supranuclear palsy (PSP) is a rare, progressive neurodegenerative brain disorder classified as an atypical parkinsonian syndrome. It involves the gradual buildup of a protein called tau in brain cells, leading to their damage and death in areas that control movement, balance, eye control, and cognition. Unlike Parkinson’s disease, PSP progresses more rapidly and does not respond well to typical Parkinson’s medications.
PSP affects about 1 in 100,000 people, and along with Guillain-Barré Syndrome (GBS) seems to be increasing in occurrence, typically starting in the mid- to late-60s (though it can begin as early as the 40s), and is slightly more common in men than women.
Causes and Risk Factors
The exact cause of PSP is unknown, but it is linked to abnormal tau protein accumulation (a “tauopathy”), similar to conditions like Alzheimer’s or frontotemporal dementia. Genetic factors play a minor role—most cases are sporadic, not inherited, though rare mutations in the MAPT gene can contribute. Environmental factors, such as exposure to pesticides, have been speculated but not confirmed. There’s no strong family history in most cases, so the risk to relatives is low.
Symptoms
PSP symptoms worsen over time and can be grouped into motor, ocular, and cognitive/behavioral categories. Early signs often mimic Parkinson’s but include more pronounced balance issues and eye movement problems.
| Category | Common Symptoms | Notes |
|---|---|---|
| Motor (Movement) | – Loss of balance and frequent unexplained falls (often backward) – Stiff posture with neck extension (leaning backward) – Slowed movements (bradykinesia) – Difficulty walking (broad-based gait) – Muscle rigidity | Falls can occur within the first year; unlike Parkinson’s, there’s no forward stooping. |
| Ocular (Eye-Related) | – Vertical gaze palsy: Difficulty looking up or down (supranuclear refers to brain control above eye nuclei) – Slowed eye movements (saccades) – Blurred or double vision – Light sensitivity (photophobia) and reduced blinking | This is a hallmark feature; horizontal eye movements are usually spared initially. |
| Cognitive/Behavioral | – Slurred speech (dysarthria) – Swallowing difficulties (dysphagia), leading to choking risk – Emotional changes: Apathy, irritability, or impulsivity – Mild cognitive impairment, like planning issues or memory lapses | Speech and swallowing are more severely affected than in Parkinson’s. |
Diagnosis
There’s no single test for PSP; diagnosis relies on clinical evaluation, ruling out other conditions (e.g., Parkinson’s, Alzheimer’s), and imaging like MRI to show brain atrophy in the midbrain. Eye movement exams are key for confirmation. Early stages are often misdiagnosed due to symptom overlap.
Treatment and Management
PSP has no cure, and symptoms progress steadily, with average survival of 6–8 years from onset (death often from complications like pneumonia from aspiration). Treatment focuses on symptom relief and quality of life:
- Medications: Levodopa for movement (limited benefit); antidepressants for mood; Botox for eye or neck stiffness.
- Therapies: Physical therapy for balance/falls prevention; speech therapy for swallowing/speech; occupational therapy for daily activities.
- Supportive Care: Sunglasses for photophobia; assistive devices like walkers; palliative care in later stages.
Research into tau-targeting therapies is ongoing, but none are approved yet.
From first symptoms, generally a PSP patient only has about 10 years to live. Instead of fighting the system and trying to prove you don’t have PSP, create an environment you will be happy and comfortable in whether you have PSP or not.
Financially, Socially, Ergonomically, Functionally
No steps inside nor outside your home; you will at some point need wheelchair access throughout your home and to a van out by the street.
A shower a wheelchair can be rolled into.
A toilet with sufficient room to use various types of assistance equipment.
Doors are a hinderance.
No carpets anywhere. Carpets greatly make mobility difficult.
Anti-Fall protection. PSP delays sensory responses. Muscles don’t respond fast enough to maintain balance consistently. Injuries from falls can complicate care significantly. But at the same time you will need to walk as much as practical to stave off muscle atrophy.
Muscle weakness is a progressive problem.
A suction device when eating can help quickly ease the discomfort of not being able to swallow foods and liquids.
Caregivers should not be allowed to lift you, or struggle to prevent you from falling. Your awkward fall can permanently harm their back. They should learn to only help you fall gracefully without slamming to the floor.
Once on the floor, a sling is placed under you by rolling you back and forth. A hoist (hoyer) is then used to mechanically pick you up and place you in a chair or on a bed. Where you can regain a safe and comfortable posture.
Caregiver Support
We were considering moving to the Philippines and rent a house. Live-in Caregivers are about $150/month and you need 3 of them for 24/7 care. They need rooms to sleep and care for themselves. Philippine law requires providing them with educational support. Not expensive but it takes some of their time. Philippine Caregivers we hired were exceptional.
Do not make any exception. Do not allow any family of caregivers to visit or provide support. Family comes first in the Philippines, and you may come to support 20 family menbers living in your home. None of them capable of maintaining detailed cleanliness nor home repairs you have come to expect.
Pay too much, and they get lazy and perpetually look for ways to take advantage of you; human nature. Pay just enough and they are grateful and protect their income.
If you live on social security and do not have significant family support, you may not have another choice. You are becoming paralyzed. Act as soon as practical. It takes efforts to get established.
Nursing Homes require significant research as they can take all of your last 10 years of earnings. No matter who is caring for the patient. And nursing home caregivers and administrators can make life uncomfortable. But if you leave, they can refuse taking you back without ANY refund.
Temporary nursing homes can cost $1500/day. Research your choices before being forced to make a last minute decision.
California Programs potentially providing assistance
Sacramento Transit has a door to door handicapped service for $5 per trip. You need to setup in advance and it takes a few weeks of processing.
Medical Transport costs a minimum of $60 one-way
Useful Durable Medical Equipment (DME)
Be frugal. Caregivers are your most significant expense.
Be careful about automatic refill services. We had ordered a type of diaper, but it didn’t work well. We had 8 cartons before we could get company to stop sending us this waste.
https://pspcbdfoundation.org/psp/
PSP Support Groups
Cellphone Group Support
https://www.smartpatients.com/communities/psp-and-cbd
https://www.psp.org/ineedsupport/supportgroups