Supranuclear palsy, progressive supranuclear palsy

Overview

Progressive supranuclear palsy (PSP), also known as Steele-Richardson-Olszewski syndrome, is a rare disorder that damages specific parts of the brain, especially in areas that control movement, coordination, and thinking. While there is no cure, treatments can help manage the symptoms.

Progressive supranuclear palsy has four types, or phenotypes, which all have similar symptoms but some unique differences, these are Richardson syndrome, PD-like variant (PSP-P), corticobasal syndrome, and pure akinesia and gait freezing. The most common types are Richardson syndrome and PD-like variant (PSP-P), making up 75% of cases.

PSP is a disease that messes with walking, balance, eye movements, and swallowing. It gets worse over time and can cause serious problems like pneumonia and difficulty swallowing. Treatments options include oral medication, movement therapies, eye treatments, among others.

Symptoms

Progressive supranuclear palsy shows different symptoms in each person and tends to start slowly, worsening over time.

Common symptoms include:

• Balance problems while climbing stairs or walking: In the very early stages of the illness, falling, especially falling backward may happen.
• Difficulty in aiming the eyes properly: Some might notice double or blurry vision. They might also have difficulty looking downward with the eyes, and a wide-eyed staring expression.

Other symptoms, which some can resemble dementia and Parkinson’s disease, include:

• Trouble speaking or slurred speech
• REM sleep behavior disorder (RBD)
• Photophobia, or light sensitivity
• Awkward motions and stiffness, particularly in the neck
• Tumbling, particularly falling backward
• Dizziness
• Insomnia
• Difficulty swallowing, potentially leading to gagging or choking
• Challenges with logical thinking, solving problems, and making decisions
• A startled or fearful facial appearance due to tight facial muscles
• Personality changes and shifts in mood, including irritation, despair, and apathy (lack of interest)
• Impulsivity, or displaying sudden laughter or tears without apparent cause
• Feelings of depression and anxiety

If any of the symptoms are observed or persist, consult a healthcare provider for proper diagnosis and treatment.

Causes

Progressive supranuclear palsy (PSP) manifests uniquely in individuals, affecting different regions of the brain with varying severity as it progresses. Eventually, it spreads throughout much of the brain, impacting cells responsible for movement and cognition. While the precise cause of PSP remains uncertain, scientists attribute a role to tau protein. Under normal circumstances, tau assists in maintaining the structure of brain cells, but in PSP, it aggregates into harmful clumps, damaging neurons. Similar tau aggregations are observed in other neurological disorders such as Alzheimer’s disease. Theories regarding the etiology of PSP include random genetic mutations, unidentified infectious agents, or environmental toxins affecting susceptible brain regions. Most instances of PSP are sporadic and show minimal familial inheritance patterns.

Risk factors

The primary risk factor for developing progressive supranuclear palsy is age, with the risk rising notably for individuals aged 60 or older. Additionally, men have a slightly higher likelihood of developing PSP compared to women.