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Diseases

Genetic and Rare Diseases Information Center (GARD)

Progressive supranuclear palsy


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

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What are the signs and symptoms of Progressive supranuclear palsy?

Progressive supranuclear palsy (PSP) causes a wide range of symptoms that differ among affected people. Most commonly, the first symptom is losing balance while walking, which may cause unexplained falls. Other common early symptoms include changes in personality, mood and behavior; irritability; and forgetfulness.[1]

As PSP progresses, most people develop blurring of vision and problems controlling eye movement. This can lead to involuntarily closing the eyes; prolonged or infrequent blinking; or difficulty opening the eyes. Some people have trouble maintaining eye contact during a conversation.[1]

Other issues develop as the disease advances, including slow movement (bradykinesia), clumsiness, and stiffness. These problems worsen with time, with most affected people ultimately needing a wheelchair.[2] Weakening of the muscles in the mouth, tongue and throat lead to slurred speech and difficulty swallowing. This often causes aspiration pneumonia, the most common cause of death in people with PSP.[1]

Last updated: 1/28/2016

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: 7/1/2016

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. NINDS. November 5, 2015; http://www.ninds.nih.gov/disorders/psp/detail_psp.htm.
  2. Progressive Supranuclear Palsy. Genetics Home Reference. May, 2015; http://ghr.nlm.nih.gov/condition/progressive-supranuclear-palsy.


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