Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Progressive supranuclear palsy


Other Names for this Disease

  • Familial progressive supranuclear palsy (type)
  • PSP
  • Steele-Richardson-Olszewski Syndrome
  • Supranuclear palsy, progressive
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Symptoms

Newline Maker

What are the signs and symptoms of progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) displays a wide range of symptoms. The pattern of signs and symptoms can be quite different among affected people. The most frequent first symptom of is a loss of balance while walking. Affected people may have unexplained falls, or a stiffness and awkwardness in gait. Sometimes the falls are described 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 regular activities); increased irritability; cantankerousness; and forgetfulness. Affected people may suddenly laugh or cry for no apparent reason; they may also be apathetic, or have occasional angry outbursts.[1] 

As the disease progresses, most people will begin to develop a blurring of vision and problems controlling eye movement. Eye problems usually offer the first definitive clue that PSP is the diagnosis. Those with PSP have trouble shifting their gaze downward, and/or 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 affected person 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 people with PSP.[1]

Last updated: 8/28/2014

The Human Phenotype Ontology provides the following list of signs and symptoms for Progressive supranuclear palsy. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Frontal release signs 45%
Tremor 30%
Limb dystonia 18%
Adult onset -
Akinesia -
Apathy -
Autosomal dominant inheritance -
Axial dystonia -
Blurred vision -
Bradykinesia -
Diplopia -
Dysarthria -
Dysphagia -
Eyelid apraxia -
Falls -
Frontolimbic dementia -
Gait imbalance -
Gliosis -
Granulovacuolar degeneration -
Irritability -
Memory impairment -
Mutism -
Neurofibrillary tangles -
Neuronal loss in central nervous system -
Parkinsonism -
Photophobia -
Retrocollis -
Rigidity -
Supranuclear gaze palsy -

Last updated: 11/3/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


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.


Other Names for this Disease
  • Familial progressive supranuclear palsy (type)
  • PSP
  • Steele-Richardson-Olszewski Syndrome
  • Supranuclear palsy, progressive
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.