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Progressive supranuclear palsy (CUI C0038868) Suggest changes to this concept
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Terms & Properties

Concept Unique Identifier (CUI): C0038868

NCI Thesaurus Code: C85028  (see NCI Thesaurus info)

Semantic Type: Disease or Syndrome

NCIt Definition: A rare neurodegenerative disorder characterized by gait and balance difficulties and loss of coordination of eye movements.

RADLEX Definition: A degenerative disease of the central nervous system characterized by balance difficulties; ocular motility disorders (supranuclear ophthalmoplegia); dysarthria; swallowing difficulties; and axial dystonia. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal mesencephalon; subthalamic nucleus; red nucleus; pallidum; dentate nucleus; and vestibular nuclei. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7) [MeSH]

GARD Definition: Progressive supranuclear palsy (PSP) is a movement disorder caused by damage to certain nerve cells in the brain. It affects brain cells that control the movement of the eyes. This leads to serious and permanent problems with balance and the way affected people walk. It usually occurs in middle-aged or elderly people. Symptoms are very different in each person, but may include personality changes; and speech, vision or swallowing problems. Doctors sometimes confuse PSP with Parkinson's disease or Alzheimer's disease. In most cases, the genetic cause of PSP is unknown. Rarely, the disease is known to result from mutations in the MAPT gene. PSP has no effective treatments, but some symptoms may be managed with medication. - this information is from GARD/ORDR/NCATS.

MEDLINEPLUS Definition: 

Progressive supranuclear palsy (PSP) is a rare brain disease. It affects brain cells that control the movement of your eyes. This leads to serious and permanent problems with balance and the way you walk. It usually occurs in middle-aged or elderly people. Symptoms are very different in each person, but may include personality changes, speech, vision and swallowing problems. Doctors sometimes confuse PSP with Parkinson's disease or Alzheimer's disease.

PSP has no cure and no effective treatments. Walking aids, special glasses and certain medicines might help somewhat. Although the disease gets worse over time, it isn't fatal on its own. However, PSP is dangerous because it increases your risk of pneumonia and choking from swallowing problems and injuries from falling.

NIH: National Institute of Neurological Disorders and Stroke

CSP Definition: spastic weakness of the muscles innervated by the cranial nerves, i.e., the muscle of the face, pharynx, and tongue, due to bilateral lesions of the corticospinal tract.

MSH Definition: A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7)

Synonyms & Abbreviations: (see Synonym Details)
Familial progressive supranuclear palsy (type)
NUCHAL DYSTONIA DEMENTIA SYNDROME
Nuchal dystonia-dementia syndrome
Ophthalmoplegia, Progressive Supranuclear
Palsy, Progressive Supranuclear
PALSY, SUPRANUCLEAR, PROGRESSIVE
Progressive supranuclear ophthalmoplegia (disorder)
Progressive supranuclear ophthalmoplegia [Steele-Richardson-Olszewski]
Progressive supranuclear ophthalmoplegia
Progressive Supranuclear Palsies
progressive supranuclear palsy
Progressive supranukleäre Paralyse
PSNP1
PSP
Richardson Syndrome
Richardson's Syndrome
Richardson-Steele-Olszewski syndrome
Steele Richardson Olszewski Disease
STEELE RICHARDSON OLSZEWSKI DIS
Steele Richardson Olszewski Syndrome
Steele-Richardson-Olszewski Disease
STEELE-RICHARDSON-OLSZEWSKI SYNDROME
Supranuclear Ophthalmoplegia, Progressive
Supranuclear Palsies, Progressive
Supranuclear Palsy, Progressive [Disease/Finding]
Supranuclear Palsy, Progressive, 1
Supranuclear palsy, progressive
Syndrome, Richardson's
Syndrome, Steele-Richardson-Olszewski

External Source Codes: 
NCI Thesaurus Code C85028 (see NCI Thesaurus info)

Other Properties: Property Definitions
Name Value Source
ACTIVE 1 SNOMEDCT_US
AQL BL CF CI CL CN CO DG DH DI DT EC EH EM EN EP ET GE HI IM ME MI MO NU PA PC PP PS PX RH RT SU TH UR VE VI MSH
CASE_SIGNIFICANCE_ID 900000000000448009 SNOMEDCT_US
CHARACTERISTIC_TYPE_ID 900000000000011006 SNOMEDCT_US
CTV3ID F1304 SNOMEDCT_US
DATE_CREATED 09/30/2003 MEDLINEPLUS
DATE_CREATED 2013-01-09T00:04:00 GARD
DATE_CREATED 2015-01-22T00:04:00 GARD
DATE_LAST_MODIFIED 2013-01-09T00:04:00 GARD
DC 1 MSH
DEFINITION_STATUS_ID 900000000000074008 SNOMEDCT_US
DID 4000-0300 CSP
DISEASE_IDENTIFIER_ID 33600 GARD
DISEASE_IDENTIFIER_ID 33601 GARD
DISEASE_IDENTIFIER_ID 5493 GARD
DISEASE_IDENTIFIER_ID 5494 GARD
DISEASE_IDENTIFIER_ID 5495 GARD
DISEASE_IDENTIFIER_ID 5496 GARD
DX 19870101 MSH
EFFECTIVE_TIME 20020131 SNOMEDCT_US
GENELOCUS ,1,7,q,2,1,.,1, OMIM
GENESYMBOL DDPAC OMIM
GENESYMBOL MAPT OMIM
GENESYMBOL MSTD OMIM
GENESYMBOL MTBT1 OMIM
HAS_GARD_PAGE true GARD
HN 1987 MSH
IAN DEFAULT ICD10
IDENTIFIER_SOURCE Orp GARD
IDENTIFIER_TYPE_ID 1 GARD
IS_ACTIVE true GARD
IS_RARE true GARD
IS_SPANISH false GARD
LAST_REVIEW_ACTION Modified GARD
LAST_REVIEW_DATE 2014-03-18T00:00:00 GARD
MDA 19860401 MSH
MESH_DEFINITION A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7) NDFRT
MESH_DUI D013494 NDFRT
MESH_NAME Supranuclear Palsy, Progressive NDFRT
MESH_UI M0020849 NDFRT
MIMTYPE 3 OMIM
MIMTYPEMEANING Phenotype description, molecular basis known. OMIM
MIMTYPEVALUE pound OMIM
MMR 20130708 MSH
MN C10.228.140.079.882 MSH
MN C10.228.662.700 MSH
MN C10.292.562.750.500 MSH
MN C10.574.945.500 MSH
MN C10.597.622.447.690 MSH
MN C11.590.472.500 MSH
MN C23.888.592.636.447.690 MSH
MODIFIER_ID 900000000000451002 SNOMEDCT_US
MP_HEALTH_TOPIC_URL https://www.nlm.nih.gov/medlineplus/progressivesupranuclearpalsy.html MEDLINEPLUS
MP_OTHER_LANGUAGE_URL Spanish https://www.nlm.nih.gov/medlineplus/spanish/progressivesupranuclearpalsy.html MEDLINEPLUS
MP_PRIMARY_INSTITUTE_URL National Institute of Neurological Disorders and Stroke http://www.ninds.nih.gov/ MEDLINEPLUS
NDFRT_KIND DISEASE_KIND NDFRT
NUI N0000002863 NDFRT
ORDER_NO 05834 ICD10CM
PM 1987 MSH
PRIMARY_PATH 10036813$10012268$10057167$10029205$Progressive supranuclear palsy$Dementia (excl Alzheimer's type)$Mental impairment disorders$Nervous system disorders MDR
PRIMARY_SOC 10029205 MDR
PT_IN_VERSION 10.0 MDR
PT_IN_VERSION 10.1 MDR
PT_IN_VERSION 11.0 MDR
PT_IN_VERSION 11.1 MDR
PT_IN_VERSION 12.0 MDR
PT_IN_VERSION 12.1 MDR
PT_IN_VERSION 13.0 MDR
PT_IN_VERSION 13.1 MDR
PT_IN_VERSION 14.0 MDR
PT_IN_VERSION 14.1 MDR
PT_IN_VERSION 15.0 MDR
PT_IN_VERSION 15.1 MDR
PT_IN_VERSION 16.0 MDR
PT_IN_VERSION 16.1 MDR
PT_IN_VERSION 17.0 MDR
PT_IN_VERSION 17.1 MDR
PT_IN_VERSION 18.0 MDR
PT_IN_VERSION 18.1 MDR
PT_IN_VERSION 19.0 MDR
PT_IN_VERSION 19.1 MDR
PT_IN_VERSION 20.0 MDR
PT_IN_VERSION 20.1 MDR
PT_IN_VERSION 8.0 MDR
PT_IN_VERSION 8.1 MDR
PT_IN_VERSION 9.0 MDR
PT_IN_VERSION 9.1 MDR
RARE_DISEASE_URL http://rarediseases.info.nih.gov/gard/7471/progressive-supranuclear-palsy/resources/1 GARD
RXAUI 3210345 RXNORM
RXAUI 3217597 RXNORM
RXAUI 3223960 RXNORM
RXAUI 3240253 RXNORM
RXAUI 3240254 RXNORM
RXAUI 3241724 RXNORM
RXAUI 3241725 RXNORM
RXAUI 5039981 RXNORM
RXAUI 5932063 RXNORM
RXAUI 5932175 RXNORM
RXCUI 1022534 RXNORM
SMQ_TERM_ADDVERSION 10.0 MDR
SMQ_TERM_CAT A MDR
SMQ_TERM_LEVEL 4 MDR
SMQ_TERM_LEVEL 5 MDR
SMQ_TERM_LMVERSION 13.1 MDR
SMQ_TERM_SCOPE 2 MDR
SMQ_TERM_STATUS A MDR
SMQ_TERM_WEIGHT 0 MDR
SNOMED_CID 28978003 NDFRT
SUBSET_MEMBER 447562003~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPADVICE~ALWAYS G23.1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 447562003~MAPTARGET~G23.1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~CORRELATIONID~447561005 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPADVICE~ALWAYS G23.1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPCATEGORYID~447637006 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPGROUP~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPPRIORITY~1 SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPRULE~TRUE SNOMEDCT_US
SUBSET_MEMBER 6011000124106~MAPTARGET~G23.1 SNOMEDCT_US
SUBSET_MEMBER 900000000000497000~MAPTARGET~F1304 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000508004~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000548007 SNOMEDCT_US
SUBSET_MEMBER 900000000000509007~ACCEPTABILITYID~900000000000549004 SNOMEDCT_US
TERMUI T039599 MSH
TERMUI T039600 MSH
TERMUI T039601 MSH
TERMUI T364585 MSH
TERMUI T364586 MSH
TERMUI T364587 MSH
TERMUI T811912 MSH
TERMUI T842339 MSH
TERMUI T842340 MSH
TH GHR (2014) MSH
TH NLM (1987) MSH
TH NLM (2000) MSH
TH OMIM (2013) MSH
TH ORD (2010) MSH
TH UNK (19XX) MSH
TYPE_ID 900000000000003001 SNOMEDCT_US
TYPE_ID 900000000000013009 SNOMEDCT_US

Additional Concept Data:  (none)

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