Supranuclear palsy, progressive supranuclear palsy
Make an Appointment
Talk to DoctorOverview
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.
Diagnosis
The diagnosis of progressive supranuclear palsy often relies on symptoms and imaging tests to examine the brain. There is not a specific test for PSP.
To aid in the diagnosis, these tests may be required:
- Magnetic Resonance Imaging (MRI): This is usually recommended to rule out other potential causes like Parkinson’s disease or stroke. MRI can detect specific brain shrinkage patterns associated with certain conditions.
- Positron emission tomography (PET) scan: Early brain changes that might not be visible on an MRI can be detected by this test.
Generally, diagnosing progressive supranuclear palsy can be challenging because its symptoms overlap with Parkinson’s disease. However, PSP might be suspected if a person lack tremors, experience frequent unexplained falls, have little or no response to Parkinson’s medications, or struggle with moving the eyes downward.
Treatment
Treatment for progressive supranuclear palsy primarily aims to alleviate symptoms and improve the patient’s quality of life. This approach includes various interventions such as oral medications, eye therapies, and movement exercises. Participation in clinical trials for PSP could be an avenue worth considering. Researchers are actively developing treatments for PSP, some of which focus on preventing the formation of tau or facilitating its breakdown.
Common treatment options include:
- Oral medications: Several medications are available to manage symptoms of progressive supranuclear palsy:
- Antiparkinsonian medications: Although these medications have a limited and brief effect typically 2 to 3 years, they can provide temporary relief for PSP symptoms like balance issues, stiff muscles, slow movements, and tremors. Commonly prescribed medications, typically used for Parkinson’s disease, include levodopa, levodopa with anticholinergic agents, and amantadine.
- Antidepressants: Antidepressants such as fluoxetine, amitriptyline, and imipramine may be prescribed to address clinical depression associated with PSP. These may only slightly improve symptoms like impulsive behavior.
- OnabotulinumtoxinA (Botox): Botox helps reduce eyelid spasms by blocking the chemical impulses that cause muscles to contract. It could provide temporary relief by relaxing the muscles around the eyes.
- Special eyeglasses with prism lenses: These allows a person to see below their direct line of sight without needing to move their eyes downward, providing a solution for vision issues related to the condition.
- Movement therapies: Several therapies can assist in alleviating certain symptoms of progressive supranuclear palsy:
- Speech therapy: The therapy targets improving speech and swallowing difficulties, providing support in communication and nutrition.
- Occupational therapy: This involves working with healthcare professionals to enhance daily task performance, focusing on standing, sitting, movement techniques, and using adaptive tools safely.
- Physical therapy: Treatment aims to address pain, stiffness, and mobility challenges, aiding individuals in managing movement difficulties effectively.
