Progressive supranuclear palsy (PSP)

Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is a rare disorder that affects different motor abilities like walking, balance, eye movements and swallowing.

PSP is a condition that gets worse over time as certain parts of the brain that handle movement, coordination and thinking are damaged. As the disease worsens, it can lead to serious issues like pneumonia and trouble swallowing.

What is PSP?

There are two primary characteristics of primary supranuclear palsy: abnormal protein buildup and brain degeneration.

The most distinctive trait of PSP is the buildup of tau proteins in certain brain cells. Tau proteins typically help maintain nerve cell structure, but in PSP, they clump together, causing problems with cell function and eventually killing brain cells.

PSP impacts brain areas like the brainstem and basal ganglia. As nerve cells break down in these regions, it leads to motor and cognitive symptoms, such as those seen in Alzheimer’s disease.

The four stages of PSP

PSP typically has four stages to characterize how far along the disease has progressed. Each stage has its own unique symptoms. These stages include:

• Early stage: Symptoms may look like normal signs of aging, such as trouble walking or vision problems. Some people develop anxiety and decreased desire to interact with others.
• Mid-stage: At this stage, you may need walking aids and vision problems may worsen. Trouble swallowing and speaking are also common.
• Advanced stage: Motor abilities worsen in this stage. You may require a wheelchair and will become more dependent on others for most or all care. Vision and speech are significantly impaired as well. The risks for inhaling food (aspiration) and developing pneumonia are very high.
• End of life: You may likely not be able to eat or drink on your own. The risk of falling, major fracture or an infection is high.

PSP symptoms

PSP symptoms are categorized by the various stages of the disease. As progressive supranuclear palsy progresses to its middle and advanced stages, its symptoms often resemble those of other conditions like Parkinson’s disease, dementia and multiple system atrophy (MSA).

In the later stages of PSP, symptoms may include:

• A fixed or startled facial expression due to rigid facial muscles
• Decreased interest in previously enjoyable activities
• Difficulty swallowing, potentially leading to gagging or choking
• Feelings of dizziness
• Impaired reasoning, problem-solving and decision-making abilities
• Increased frequency of falling, particularly backward
• Impulsive behavior, unexplained laughter or crying
• Onset of depression and anxiety
• Sensitivity to bright light
• Sleep disturbances
• Speech slowness or slurring
• Stiffness, particularly in the neck and clumsy movements.

What are the early symptoms of PSP?

Progressive supranuclear palsy symptoms can be mild in the early stages, sometimes overlooked or confused with other disorders, leading to a potential misdiagnosis.

Early signs might include slight shifts in balance, unsteadiness in walking, trouble with eye movements (particularly in looking up or down) and mild cognitive alterations like executive function difficulties. Some individuals might notice mood or behavioral changes during this phase.

As PSP advances, symptoms become more noticeable and start affecting everyday activities. Walking and balance issues worsen, resulting in more frequent falls.

What is the cause of PSP?

Although the precise cause of progressive supranuclear palsy is still uncertain, its symptoms result from damage to areas of the brain.

Research indicates that people with progressive supranuclear palsy show a buildup of tau protein in the affected brain cells.

Although PSP can sometimes occur within families, a clear genetic link has not been confirmed. Most individuals with this condition have not inherited it.

Diagnosing PSP

Diagnosing progressive supranuclear palsy can be difficult because its symptoms can resemble those of Alzheimer’s or Parkinson’s disease. Due to this, PSP often goes undiagnosed in some people.

To confirm a diagnosis, your doctor may consider PSP rather than other diseases if:

• You don't experience any tremors.
• You frequently fall without a clear explanation.
• Parkinson's disease medications aren't improving your symptoms.
• You have difficulty moving your eyes, especially looking downward.

Your provider may suggest an MRI or a PET scan to confirm the diagnosis. An MRI can reveal any shrinkage in the brain areas affected by PSP. This imaging technique can also help rule out other conditions that have similar symptoms.

A PET scan might be recommended to notice early brain changes not visible on an MRI.

Treating and managing PSP

Since there's no cure for progressive supranuclear palsy, treatment focuses on improving your comfort and managing symptoms. Several approaches can help alleviate PSP symptoms:

• Antidepressants: Some antidepressant medications may provide relief for symptoms like impulsive behavior.
• Botox: Injecting small amounts of this neurotoxin into the muscles around the eyes can block the chemical signals responsible for muscle contractions.
• Palliative care: Palliative care can be administered in hospitals, clinics, nursing facilities, or at home. Its goal is to provide relief from the symptoms of a serious illness, regardless of the disease stage.
• Parkinson's disease medications: These drugs increase levels of a brain chemical that’s essential for smooth muscle movements. However, their effectiveness is often limited and temporary.
• Therapies: Physical therapy, occupational therapy and speech therapy aim to improve quality of life while living with PSP. These therapies may include exercises such as facial exercises, using talking keyboards and gait and balance training, all of which can help manage various PSP symptoms.

To reduce the impact of PSP, you can take certain steps at home, like using eye drops, walking with a walker, and eliminating falling hazards. These measures may help enhance your quality of life while living with PSP.