Progressive supranuclear palsy

Progressive supranuclear palsy (PSP) is a rare brain condition that affects body movements such as walking, balance, and eye function. PSP is caused by damage to nerve cells in areas of the brain that control and coordinate movement. This damage results in gradual weakness or paralysis of the muscles controlled by this region, including eye muscles.

Progressive supranuclear palsy is one of several atypical parkinsonism disorders. Early symptoms of PSP are often mistaken for Parkinson's disease, but symptoms of PSP progress more quickly. Most symptoms affect control of muscle movement or motor control, while other non-motor symptoms may affect mood or behavior.

Motor symptoms of PSP may include:

• Stiff or slow movements in the limbs, trunk (axial rigidity), or neck
• Problems with walking, balance, clumsiness, and frequent falls
• Tendency to lean or lunge forward to avoid falling backward
• Problems with moving the eyes, looking down, keeping eyes open, and sensitivity to bright light
• Changes in facial expressions/deeply lined face
• Tremors or muscle spasms (especially in the jaw or face)
• Difficulty speaking/swallowing

Non-motor symptoms of PSP may include:

• Behavioral changes, including impulsivity or poor judgment
• Dizziness
• Dementia (mild-to-moderate memory loss, difficulty with reasoning, making decisions, and solving problems)
• Personality or mood changes (depression, anxiety, irritability, laughing or crying without reason)
• Sensitivity to bright light
• Sleep disturbances (insomnia, rapid eye movement sleep behavior disorder)

What are the stages of progressive supranuclear palsy?

Doctors track the progression of PSP in four stages:

1. Early stage usually occurs within the first year after disease onset. These symptoms may look like normal changes associated with aging. At this stage, people may have trouble with balance and walking. Visual problems may impact the ability to read. Voice changes start to occur with reduced speaking volume. Some people experience mood and behavior changes, including apathy, anxiety, and decreased interaction with other people.
2. Mid-stage PSP usually occurs between the second and third years with the condition. People with PSP often need walking aids and experience more vision problems. Speaking and swallowing may become more difficult.
3. Advanced stage PSP usually occurs between years three through six. People with PSP have significant problems with walking, muscle stiffness, and pain, often requiring a wheelchair for mobility. They depend on others for most or all care aspects. Vision, speech, and swallowing are significantly impaired. The risks for inhaling food (aspiration) and developing pneumonia are very high.
4. End-of-life PSP typically spans six to eight weeks. Reduced consciousness and marked deterioration in the ability to eat or drink resulting in rapid weight loss occur during this stage. A fall, major fracture, or infection may also occur during this period.

People with progressive supranuclear palsy have higher amounts of a protein called tau in the brain. Higher levels of tau cause nerve cells to die. This protein also occurs in other conditions characterized by nerve damage in the brain, such as Alzheimer’s disease. Researchers aren’t sure what causes higher tau levels.

Progressive supranuclear palsy is difficult to diagnose, especially in early stages. No standard laboratory tests or imaging studies can confirm this brain condition.

Doctors typically rule out all other possibilities in order to diagnose PSP. Early PSP symptoms mimic those of Parkinson’s disease or other nerve conditions, but these symptoms often do not respond well to standard Parkinson’s disease treatments.

Movement specialists at Penn Medicine have experience and skill diagnosing PSP. We can detect the subtle differences between this condition and other movement disorders to make an accurate diagnosis.

There is no cure for PSP, but treatments can help manage symptoms and improve quality of life. The CurePSP Foundation has designated Penn Medicine as a PSP Center of Care CurePSP - Centers of Care in recognition of our specialized treatment options, research, and support for people with PSP.

Penn's Movement Disorders Center offers all leading therapies for PSP, including:

• Eye treatments: Botulinum toxin injections may improve vision by reducing excessive eye closing and abnormal eye movements. Eye drops and artificial tears can treat dry eyes due to decreased blinking. Wraparound sunglasses can help people with PSP who are sensitive to bright light.
• Oral medications: Some medications used to treat Parkinson's disease may help treat slow movements, stiffness, and balance problems, but they often have short-lived effects.
• Rehabilitative therapies: Physical therapy may help prevent falls and improve balance. Occupational therapy may help improve the ability to perform daily tasks. Speech therapy may provide targeted treatments for swallowing and speech difficulties to reduce risk of aspiration and improve communication.
• Palliative care: This specialized care provides medical, social, and emotional support for people with PSP and their caregivers as symptoms progress. Palliative care enables people with PSP to live more comfortably.