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Progressive supranuclear palsy

Definition

Progressive supranuclear palsy is a movement disorder that occurs from damage to certain nerve cells in the brain.

Alternative Names

Dementia-nuchal dystonia; Richardson-Steele-Olszewski syndrome; Palsy - progressive supranuclear

Causes, incidence, and risk factors

Progressive supranuclear palsy is a condition that causes symptoms similar to those of Parkinson's disease.

It 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. Also affected is an area of the brain that controls steadiness when you walk. The frontal lobes of the brain are also affected, leading to personality changes.

The cause of the damage to the brain cells is unknown. The disease gets worse over time.

People with this condition have deposits in brain tissues that look like those found in patients with Alzheimer's disease. There is a loss of tissue in most areas of the brain and in some parts of the spinal cord.

The disorder is most often seen in people over 60 years old, and is somewhat more common in men.

Symptoms

• Changes in expressions of the face
• Deeply lined face
• Difficulty moving the eyes or lack of control over the eyes
• Different size pupils
• Difficulty swallowing
• General slowness of movement (bradykinesia)
• Jaw or face jerks or spasms
• Loss of coordination, unsteady gait (walking pattern)
• Mild-to-moderate dementia
  • Difficulty using knowledge
  • Forgetfulness
  • Indifference (apathy)
  • Slowed thought processes
• Personality changes
• Repeated falls
• Slow or stiff movements
• Speech difficulties
  • Low voice volume
  • Poor ability to speak clearly (enunciate)
  • Slow speech
• Stiffness and rigid movement in the neck, middle of the body, arms, legs
• Tremor
• Vision difficulty -- unable to look up or down without bending the neck

Signs and tests

An exam of the nervous system (neurological examination) may show:

• Dementia that is getting worse
• Limited eye movements, especially up and down movements
• Normal vision, hearing, sensation, and voluntary control of movement
• Stiff and uncoordinated movements like those of Parkinson's disease

The health care provider may do tests to rule out other diseases. Magnetic resonance imaging (MRI) might show shrinking of the brainstem.

Treatment

The goal of treatment is to control symptoms. There is no known cure for progressive supranuclear palsy.

Levodopa or other drugs, such as benztropine or trihexyphenidyl, which block the action of a nervous system chemical called acetylcholine (anticholinergic medications) may temporarily reduce some symptoms, such as rigid limbs or slow movements. However, these medications are usually not as effective as they are for Parkinson's disease.

Many people with this condition will need around-the-clock care and monitoring as they lose brain functions.

Support Groups

Expectations (prognosis)

Treatment sometimes can reduce symptoms temporarily, but the condition will get worse. Brain function will decline over time. Death commonly occurs in 5 to 7 years.

Complications

• Blood clot in veins (deep vein thrombosis) due to limited mobility
• Lack of control over gaze
• Loss of brain functions over time
• Pneumonia due to difficulty swallowing
• Poor nutrition (malnutrition)
• Side effects from medications

Calling your health care provider

Call your health care 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 progressive supranuclear palsy and the condition has declined so much that you can no longer care for the person at home.

Prevention

References

Goetz CG. Textbook of Clinical Neurology. 3rd ed. Philadelphia, Pa: Saunders; 2007.

Lang A. Parkinsonism. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 433.