Progressive supranuclear palsy
Dementia - nuchal dystonia; Richardson-Steele-Olszewski syndrome; Palsy - progressive supranuclearProgressive supranuclear palsy (PSP) is a movement disorder that occurs from damage to certain nerve cells in the brain.
Causes
PSP is a condition that causes symptoms similar to those of Parkinson disease.
Parkinson disease
Parkinson disease results from certain brain cells dying. These cells help control movement and coordination. The disease leads to shaking (tremors...
Read Article Now Book Mark ArticleIt involves damage to many cells of the brain. Many areas are affected, including the part of the brainstem where cells that control eye movement are located. The area of the brain that controls steadiness when you walk is also affected. The frontal lobes of the brain are also affected, leading to personality changes.
The cause of the damage to the brain cells is unknown. PSP gets worse over time.
People with PSP have deposits in brain tissues that look like those found in people with Alzheimer disease. There is a loss of tissue in most areas of the brain and in some parts of the spinal cord.
Alzheimer disease
Dementia is a loss of brain function that occurs with certain diseases. Alzheimer disease (AD) is the most common form of dementia. It affects memo...
Read Article Now Book Mark ArticleThe disorder is most often seen in people over 60 years old, and is somewhat more common in men.
Symptoms
Symptoms may include any of the following:
- Loss of balance, repeated falls
- Lunging forward when moving, or fast walking
- Bumping into objects or people
- Changes in expressions of the face
- Deeply lined face
- Eye and vision problems such as different sized pupils, difficulty moving the eyes (supranuclear ophthalmoplegia), lack of control over the eyes, problems keeping the eyes open
Different sized pupils
Anisocoria is unequal pupil size. The pupil is the black part in the center of the eye. It gets larger in dim light and smaller in bright light....
Read Article Now Book Mark ArticleSupranuclear ophthalmoplegia
Supranuclear ophthalmoplegia is a condition that affects the movement of the eyes.
Read Article Now Book Mark Article - Difficulty swallowing
- Tremors, jaw or face jerks or spasms
- Mild-to-moderate dementia
Dementia
Dementia is a loss of brain function that occurs with certain diseases. It affects one or more brain functions such as memory, thinking, language, j...
Read Article Now Book Mark Article - Personality changes
- Slow or stiff movements
- Speech difficulties, such as low voice volume, not able to say words clearly, slow speech
- Stiffness and rigid movement in the neck, middle of the body, arms, and legs
Exams and Tests
An exam of the nervous system (neurologic exam) may show:
- Dementia that is getting worse
- Difficulty walking
- Limited eye movements, especially up and down movements
- Inability to keep eyes open
- Normal vision, hearing, feeling, and control of movement
- Stiff and uncoordinated movements like those of Parkinson disease
Your health care provider may do the following tests to rule out other diseases:
- MRI might show shrinking of the brainstem (hummingbird sign)
MRI
A head MRI (magnetic resonance imaging) is an imaging test that uses powerful magnets and radio waves to create pictures of the brain and surrounding...
Read Article Now Book Mark Article - PET scan of the brain will show changes in the front of the brain
PET scan of the brain
A brain positron emission tomography (PET) scan is an imaging test of the brain. It uses a radioactive substance called a tracer to look for disease...
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Treatment
The goal of treatment is to control symptoms. There is no known cure for PSP.
Medicines such as levodopa (combined with carbidopa) may be tried. These medicines raise the level of a brain chemical called dopamine. Dopamine is involved in the control of movement. The medicines may reduce some symptoms, such as rigid limbs or slow movements for a time. But they are usually not as effective as they are for Parkinson disease.
Many people with PSP will eventually need around-the-clock care and monitoring as they lose brain functions.
Outlook (Prognosis)
Treatment sometimes can reduce symptoms for a while, but the condition will get worse. Brain function will decline over time. Death commonly occurs in 5 to 7 years.
Newer medicines are being studied to treat this condition.
Possible Complications
Complications of PSP include:
- Blood clot in veins (deep vein thrombosis) due to limited movement
Deep vein thrombosis
Deep vein thrombosis (DVT) is a condition that occurs when a blood clot forms in a vein deep inside a part of the body. DVT mainly affects the large...
Read Article Now Book Mark Article - Injury from falling
- Lack of control over vision
- Loss of brain functions over time
- Pneumonia due to trouble swallowing
Pneumonia
Pneumonia is a breathing (respiratory) condition in which there is an infection of the lung. This article covers community-acquired pneumonia (CAP). ...
Read Article Now Book Mark Article - Poor nutrition (malnutrition)
- Side effects from medicines
When to Contact a Medical Professional
Contact your provider if you often fall, and if you have a stiff neck/body, and vision problems.
Also, call if a loved one has been diagnosed with PSP and the condition has declined so much that you can no longer care for the person at home.
References
Jankovic J. Parkinson disease and other movement disorders. In: Jankovic J, Mazziotta JC, Pomeroy SL, Newman NJ, eds. Bradley and Daroff's Neurology in Clinical Practice. 8th ed. Philadelphia, PA: Elsevier;2022:chap 96.
National Institute of Neurological Disorders and Stroke website. Progressive supranuclear palsy (PSP). www.ninds.nih.gov/health-information/disorders/progressive-supranuclear-palsy-psp. Updated November 28, 2023. Accessed June 28, 2024.
Rowe JB, Holland N, Rittman T. Progressive supranuclear palsy: diagnosis and management. Pract Neurol. 2021;21(5):376-383. PMID: 34215700 pubmed.ncbi.nlm.nih.gov/34215700/.
Brain structures - illustration
The structures of the brain include the brainstem, consisting of the spinal cord, the medulla oblongata, the pons and the midbrain; the cerebellum; the cerebrum (one half, or hemisphere shown), and the diencephalon.
Brain structures
illustration
Review Date: 6/13/2024
Reviewed By: Joseph V. Campellone, MD, Department of Neurology, Cooper Medical School at Rowan University, Camden, NJ. Review provided by VeriMed Healthcare Network. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.