Progressive Supranuclear Palsy

Summary about Disease


Progressive Supranuclear Palsy (PSP) is a rare brain disorder that affects movement, balance, eye movements, and thinking. It's a progressive condition, meaning the symptoms gradually worsen over time. PSP is caused by damage to nerve cells in the brain, specifically in areas that control movement and coordination. It is often misdiagnosed initially as Parkinson's disease because they share some similar symptoms.

Symptoms


Symptoms of PSP vary from person to person but often include:

Balance problems: Frequent falls, stiffness, and difficulty walking.

Eye movement problems: Difficulty looking up or down voluntarily. This can lead to blurry vision or double vision.

Stiffness and rigidity: Particularly in the neck and upper body.

Slowed movement: Similar to Parkinson's disease (bradykinesia).

Speech problems: Slurred or slow speech (dysarthria).

Swallowing difficulties: (Dysphagia) potentially leading to choking or aspiration pneumonia.

Changes in mood and behavior: Irritability, apathy, depression, or impulsivity.

Cognitive impairment: Problems with planning, decision-making, and problem-solving.

"Masked" facial expression: Reduced facial expressions, making it difficult to convey emotions.

Causes


The exact cause of PSP is unknown. Researchers believe it results from the gradual deterioration of nerve cells in specific areas of the brain that control movement, coordination, thinking, and other important functions. A protein called tau accumulates abnormally in these brain cells. It is not considered to be directly inherited or caused by environmental factors. There is no known gene that directly causes PSP.

Medicine Used


There is no cure for PSP, and no medications can stop or reverse the underlying brain damage. Treatment focuses on managing symptoms and improving quality of life.

Medications for Parkinson's disease: Such as levodopa, may provide some relief from stiffness and slowness in some individuals, but they are usually less effective than in Parkinson's disease.

Antidepressants: To manage depression, anxiety, or irritability.

Botulinum toxin (Botox): Injections can help with involuntary eye closure (blepharospasm).

Other medications: To address specific symptoms, such as sleep disturbances or bladder control problems.

Is Communicable


No, Progressive Supranuclear Palsy is not a communicable disease. It is not contagious and cannot be spread from person to person.

Precautions


There are no specific precautions to prevent PSP, as the cause is unknown. However, general safety measures are important for those living with PSP:

Fall prevention: Removing hazards in the home, using assistive devices (walkers, canes), and wearing appropriate footwear.

Swallowing precautions: Working with a speech therapist to learn safe swallowing techniques and modifying food textures as needed.

Communication strategies: Exploring alternative communication methods if speech becomes significantly impaired.

Regular exercise: Maintain mobility and strength as much as possible with physical therapy.

Protect the eyes: Due to difficulties with vertical eye movements, protect your eyes from sun and wind.

How long does an outbreak last?


PSP is not an infectious disease and therefore does not have outbreaks. It is a progressive neurological disorder that gradually worsens over time. There is no "outbreak" period.

How is it diagnosed?


Diagnosing PSP can be challenging, especially in the early stages, as it can resemble other conditions, such as Parkinson's disease. Diagnosis typically involves:

Neurological examination: Assessing motor skills, balance, eye movements, reflexes, and cognitive function.

Medical history: Reviewing the individual's symptoms and medical history.

Brain imaging: MRI scans can help rule out other conditions and may show specific patterns suggestive of PSP, such as atrophy in certain brain regions.

Eye movement testing: Assessing the range and speed of eye movements.

DaTscan: (Dopamine transporter scan) can help differentiate PSP from Parkinson's disease, as DaTscan results are typically normal in PSP.

Clinical criteria: Doctors use established diagnostic criteria to determine if a person meets the criteria for PSP.

Timeline of Symptoms


The progression of PSP varies from person to person, but generally follows a pattern:

Early stages: Balance problems, frequent falls, stiffness, subtle changes in personality or cognition, and difficulty with eye movements may appear.

Middle stages: Symptoms become more pronounced. Eye movement problems worsen, leading to difficulty looking up or down. Speech and swallowing difficulties develop. Cognitive impairment becomes more noticeable.

Late stages: Severe stiffness, difficulty walking or standing, significant speech and swallowing problems, and marked cognitive decline are common. People may require assistance with all activities of daily living.

Important Considerations


Early diagnosis is important: While there is no cure, early diagnosis allows individuals and families to plan for the future and access supportive care.

Multidisciplinary care: A team of healthcare professionals, including neurologists, physical therapists, speech therapists, occupational therapists, and social workers, can provide comprehensive care.

Support groups: Joining a support group can provide emotional support, practical advice, and a sense of community for individuals and families affected by PSP.

Advance care planning: Discussing end-of-life wishes and making advance care planning decisions is important.

Research: Supporting research efforts can help improve understanding of PSP and potentially lead to new treatments in the future.

Aspiration Pneumonia: A major concern is aspiration pneumonia because of the progressive difficulty in swallowing. Be mindful of any signs.