Dementia-nuchal dystonia

Definition

Progressive supranuclear palsy is a movement disorder caused by damage to certain nerve cells in the brain.

Alternative Names

Causes

Progressive supranuclear palsy is a condition that causes symptoms similar to Parkinson's disease. Symptoms may include a progressive lack of coordination, stiffness of the neck and body, difficulties with eye movement, slow movements, problems with mental function, and difficulty walking that can result in falls.

Progressive supranuclear palsy involves damage to multiple 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 in this condition is unknown. The disease is degenerative (gets worse over time).

People with this condition have deposits in brain tissues that resemble 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

• Repeated falls
• Loss of coordination, unsteady gait (walking pattern)
• Stiffness and rigid movement in the neck, middle of the body, arms, legs
• Difficulty swallowing
• May have a tremor
• Pain or difficulty with bending the neck up or down
• Slow or stiff movements
• Vision difficulty -- unable to look up or down without bending the neck
• Changes in facial expression
• Deeply lined face
• Jaw or face jerks or spasms
• Eyes, pupils different size
• Eye movements, uncontrollable
• Generalized slowness of all movement (bradykinesia)
• Speech difficulties
  • Slow speech
  • Poor enunciation
  • Low voice volume
• Dementia, mild
  • Forgetfulness
  • Slowed thought processes
  • Apathy (indifference)
  • Difficulty manipulating knowledge
• Personality changes (vague and mild)

Exams and Tests

A neurological examination may show Parkinsonian movements with typical stiffness and lack of coordination. Dementia is mild. Eye movements are limited. However, vision, hearing, sensation, and voluntary control of movement remain normal.

Tests may be done to rule out other diseases. An MRI might show shrinking of the brainstem.

Treatment

Treatment is aimed at controlling symptoms. There is no known cure for progressive supranuclear palsy. Levodopa and anticholinergic medications may provide temporary reduction of symptoms.

These are not as effective as in Parkinson's disease, however. Around-the-clock care and monitoring are eventually required because of progressive loss of brain functions.

Outlook (Prognosis)

Treatment sometimes provides temporary reduction of symptoms, but the disorder inevitably progresses. There is a steady decline of brain function, with death commonly occurring in 5 to 7 years.

Possible Complications

• Total inability to control gaze
• Progressive loss of brain functions
• Malnutrition
• Deep vein thrombosis (blood clot in veins)
• Pneumonia
• Side effects from levodopa and anticholinergic medications

When to Contact a Medical Professional

Call your health care provider if frequent falls accompanied by stiff neck/body and vision difficulties occur.

Also, call if progressive supranuclear palsy has been diagnosed and the condition declines to a point when it is not possible to care for the person in the home.

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