Progressive supranuclear palsy Health Article

Definition

Progressive supranuclear palsy is a movement disorder caused by 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. The covering of certain nerve cells (myelin sheath) is destroyed. Entire nerves may be damaged in some areas. The cause of the damage to the brain cells is unknown. The disease gets worse over time (degenerative).

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.

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
• Different size pupils
• Difficulty swallowing
• General slowness of movement (bradykinesia)
• Jaw or face jerks or spasms
• Loss of coordination, unsteady gait (walking pattern)
• Mild dementia
  • Difficulty using knowledge
  • Forgetfulness
  • Indifference (apathy)
  • Slowed thought processes
• Pain or difficulty with bending the neck up or down
• 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
• Uncontrollable eye movements
• 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:

• Limited eye movements
• Mild dementia
• 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.