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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Progressive supranuclear palsy


Other Names for this Disease
  • Familial progressive supranuclear palsy (type)
  • PSP
  • Steele-Richardson-Olszewski Syndrome
  • Supranuclear palsy, progressive
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Symptoms


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What are the signs and symptoms of progressive supranuclear palsy?

Progressive supranuclear palsy displays a wide range of symptoms. The pattern of signs and symptoms can be quite different from person to person. The most frequent first symptom of is a loss of balance while walking. Affected individuals may experience unexplained falls or a stiffness and awkwardness in gait. Sometimes the falls are described by the person experiencing them as attacks of dizziness. This often prompts suspicion of an inner ear problem.[1]

Other common early symptoms are changes in personality such as a loss of interest in ordinary pleasurable activities or increased irritability, cantankerousness, and forgetfulness. Affected individuals may suddenly laugh or cry for no apparent reason; they may also be apathetic, or have occasional angry outbursts, also for no apparent reason.[1] 

As the disease progresses, most people will begin to develop a blurring of vision and problems controlling eye movement. In fact, eye problems usually offer the first definitive clue that progressive supranuclear palsy is the proper diagnosis. Those with progressive supranuclear palsy have trouble voluntarily shifting their gaze downward, and also can have trouble controlling their eyelids. This can lead to involuntary closing of the eyes, prolonged or infrequent blinking, or difficulty in opening the eyes.[1] Another common visual problem is an inability to maintain eye contact during a conversation. This can give the mistaken impression that the patient is hostile or uninterested.[1]

Weakened movements of the mouth, tongue and throat can lead to slurred speech and difficulty swallowing. The inability of throat muscles to create a watertight seal outside the person's lungs often results in aspiration pneumonia - the most common cause of death in those with progressive supranuclear palsy.[1]

In rare cases, some affected individuals will notice shaking of the hands.[1]
Last updated: 8/18/2011

References
  1. Progressive Supranuclear Palsy Fact Sheet. National Institute for Neurological Disorders and Stroke (NINDS). August 16, 2011; http://www.ninds.nih.gov/disorders/psp/detail_psp.htm. Accessed 8/18/2011.