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Progressive supranuclear palsy
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{{Short description|Medical condition of the brain}} {{cs1 config|name-list-style=vanc|display-authors=6}} {{Infobox medical condition (new) | name = Progressive supranuclear palsy | synonyms = Steele–Richardson–Olszewski syndrome, frontotemporal dementia with parkinsonism | image = Steele-olszewski-richardson disease.jpg | caption = MRI demonstrating the hummingbird sign of supranuclear palsy due to atrophy of the midbrain | pronounce = | field = [[Neurology]] | width = 280px | symptoms = Impaired balance, [[Hypokinesia|slowed movements]], [[Ophthalmoparesis|difficulty moving eyes]], [[dementia]] | complications = | onset = 60–70 years | duration = | types = | causes = Unknown | risks = | diagnosis = | differential = [[Parkinson's disease]], [[corticobasal degeneration]], [[FTDP-17]], [[Alzheimer's disease]] | prevention = | treatment = Medication, physical therapy, occupational therapy | medication = [[L-DOPA|Levodopa]], [[amantadine]] | prognosis = Fatal (usually 7–10 years after diagnosis) | frequency = 6 per 100,000 | deaths = }} '''Progressive supranuclear palsy''' ('''PSP''') is a late-onset [[neurodegenerative disease]] involving the gradual deterioration and death of specific volumes of the [[brain]], linked to 4-repeat tau pathology.<ref name="Golbe20142">{{cite journal |vauthors=Golbe LI |date=April 2014 |title=Progressive supranuclear palsy |journal=Seminars in Neurology |volume=34 |issue=2 |pages=151–9 |doi=10.1055/s-0034-1381736 |pmid=24963674 |doi-access=free}}</ref><ref name="ICD112">{{cite web |title=ICD-11 - Mortality and Morbidity Statistics |url=https://icd.who.int/browse11/l-m/en#/http://id.who.int/icd/entity/1493396558 |website=icd.who.int}}</ref><ref name=":0">{{Cite journal |last1=Boxer |first1=Adam L |last2=Yu |first2=Jin-Tai |last3=Golbe |first3=Lawrence I |last4=Litvan |first4=Irene |last5=Lang |first5=Anthony E |last6=Höglinger |first6=Günter U |date=July 2017 |title=Advances in progressive supranuclear palsy: new diagnostic criteria, biomarkers, and therapeutic approaches |journal=The Lancet Neurology |language=en |volume=16 |issue=7 |pages=552–563 |doi=10.1016/S1474-4422(17)30157-6|pmid=28653647 |pmc=5802400 }}</ref> The condition leads to symptoms including [[Balance disorder|loss of balance]], [[Hypokinesia|slowing of movement]], [[Ophthalmoparesis|difficulty moving the eyes]], and [[cognitive impairment]].<ref name="Golbe20142" /> PSP may be mistaken for other types of neurodegeneration such as [[Parkinson's disease]], [[frontotemporal dementia]] and [[Alzheimer's disease]]. It is the second most common tauopathy behind [[Alzheimer's disease]]. The cause of the condition is uncertain, but involves the accumulation of [[tau protein]] within the brain. Medications such as [[L-DOPA|levodopa]] and [[amantadine]] may be useful in some cases.<ref name="Golbe20142" /> PSP was first officially described by Richardson, Steele, and Olszewski in 1963 as a form of progressive parkinsonism.<ref name=":3">{{Cite journal |last1=Lopez |first1=G. |last2=Bayulkem |first2=K. |last3=Hallett |first3=M. |date=October 2016 |title=Progressive supranuclear palsy (PSP): Richardson syndrome and other PSP variants |journal=Acta Neurologica Scandinavica |language=en |volume=134 |issue=4 |pages=242–249 |doi=10.1111/ane.12546|pmid=27070344 |pmc=7292631 }}</ref> However, the earliest known case presenting clinical features consistent with PSP, along with pathological confirmation, was reported in France in 1951.<ref name=":13">{{Cite journal |last1=Brusa |first1=Adolfo |last2=Stoehr |first2=Rolf |last3=Pramstaller |first3=Peter P. |date=March 2004 |title=Progressive supranuclear palsy: New disease or variant of postencephalitic parkinsonism? |url=https://movementdisorders.onlinelibrary.wiley.com/doi/10.1002/mds.10699 |journal=Movement Disorders |language=en |volume=19 |issue=3 |pages=247–252 |doi=10.1002/mds.10699 |pmid=15022178 |issn=0885-3185}}</ref> Originally thought to be a more general type of atypical parkinsonism, PSP is now linked to distinct clinical phenotypes including '''PSP-Richardson's syndrome (PSP-RS)''', which is the most common sub-type of the disease.<ref name=":6">{{Cite journal |last1=Coughlin |first1=David G. |last2=Litvan |first2=Irene |date=April 2020 |title=Progressive supranuclear palsy: Advances in diagnosis and management |journal=Parkinsonism & Related Disorders |language=en |volume=73 |pages=105–116 |doi=10.1016/j.parkreldis.2020.04.014|pmid=32487421 |pmc=7462164 }}</ref> As PSP advances to a fully symptomatic stage, many PSP subtypes eventually exhibit the clinical characteristics of PSP-RS.<ref name=":0" /> PSP, encompassing all its phenotypes, has a prevalence of 18 per 100,000, whereas PSP-RS affects approximately 5 to 7 per 100,000 individuals.<ref name="Golbe20142" /><ref name=":0" /> The first symptoms typically occur at 60–70 years of age. [[Male|Males]] are slightly more likely to be affected than [[Female|females.]]<ref name="Golbe20142" /> No association has been found between PSP and any particular race, location, or occupation.<ref name="Golbe20142" />
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