Progressive supranuclear palsy

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

Definition
Progressive supranuclear palsy is a disorder caused by damage to certain nerve cells in the brain, characterized by progressive lack of coordination, stiffness of the neck and trunk, difficulties with eye movement, slow movements, cognitive dysfunction, and difficulty walking that can result in falls.

Causes, incidence, and risk factors

Progressive supranuclear palsy is a disorder characterized by symptoms similar to Parkinson’s disease (including unsteady gait, stiff movements, and mild dementia). Other similar disorders include dysfunction of the cerebellum, multiple system atrophy, and Alzheimer’s disease.

The cause of the damage to the brain cells in this condition is unknown, but the disease is degenerative (gets worse over time). Progressive supranuclear palsy involves damage to multiple cells of the brain, with loss of the myelin sheath (the covering of the nerve cell that speeds nerve impulse conduction) in some nerves and destruction of the entire nerve in other areas.

People with this condition have deposits in brain tissues that resemble the deposits found in Alzheimer’s disease. There is atrophy (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, trunk, 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       o “Masking,” reduced expression       o Deeply lined face       o Jaw or face jerks or spasms       o Eyes, pupils different size       o Eye movements, uncontrollable  
  • Generalized slowness of all movement (bradykinesia) with strength relatively preserved  
  • Speech difficulties       o Slow speech       o Poor enunciation       o Low voice volume  
  • Dementia, mild       o Forgetfulness       o Slowed thought processes       o Apathy (indifference)       o Difficulty manipulating knowledge  
  • Personality changes, vague and mild

Signs and tests

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

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.

Expectations (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.

Complications

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

Calling your health care provider

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.

Johns Hopkins patient information

Last revised: December 7, 2012
by Sharon M. Smith, M.D.

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