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Multiple system atrophy
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====Old terminology==== {{Infobox medical condition (new) | name = Olivopontocerebellar atrophy | image = Gray707.png | caption = Sagittal section through right cerebellar hemisphere. The right olive has also been cut sagittally. | field = [[Neurology]] | synonyms = Multiple system atrophy – cerebellar subtype<ref>{{cite web |title=Multiple system atrophy – cerebellar subtype: MedlinePlus Medical Encyclopedia |url=https://medlineplus.gov/ency/article/000758.htm |website=medlineplus.gov |access-date=24 May 2019 |language=en}}</ref> }} Historically, many terms were used to refer to this disorder, based on the predominant systems presented. These terms were discontinued by consensus in 1996 and replaced with MSA and its subtypes,<ref name="pmid8628505">{{cite journal | author = The Consensus Committee of the American Autonomic Society and the American Academy of Neurology | title = Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. | journal = Neurology | volume = 46 | issue = 5 | pages = 1470 | date = May 1996 | pmid = 8628505 | doi = 10.1212/wnl.46.5.1470 | s2cid = 219212717 }}</ref> but awareness of these older terms and their definitions is helpful to understanding the relevant literature prior to 1996. These include [[striatonigral degeneration]] (SND), '''olivopontocerebellar atrophy''' (OPCA), and [[Shy–Drager syndrome]].<ref name="Ahmed-2012">{{cite journal | vauthors = Ahmed Z, Asi YT, Sailer A, Lees AJ, Houlden H, Revesz T, Holton JL | title = The neuropathology, pathophysiology and genetics of multiple system atrophy | journal = Neuropathology and Applied Neurobiology | volume = 38 | issue = 1 | pages = 4–24 | date = February 2012 | pmid = 22074330 | doi = 10.1111/j.1365-2990.2011.01234.x | s2cid = 22901422 }}</ref> A table describing the characteristics and modern names of these conditions follows: {| class="wikitable" ! Historical Name !! Characteristics !! Modern name and abbreviation |- | Striatonigral degeneration || predominating [[Parkinson's disease|Parkinson's]]-like symptoms || MSA-P, "p" = parkinsonian subtype |- | Sporadic olivopontocerebellar atrophy (OPCA) || characterized by progressive ataxia (an inability to coordinate voluntary muscular movements) of the gait and arms and [[dysarthria]] (difficulty in articulating words) || MSA-C, "c" = cerebellar dysfunction subtype |- | Shy-Drager syndrome|| characterized by Parkinsonism plus a more pronounced failure of the [[autonomic nervous system]].<ref name="Shy GM, Drager GA 1960 511–27">{{cite journal | vauthors = Shy GM, Drager GA | title = A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study | journal = Archives of Neurology | volume = 2 | issue = 5 | pages = 511–527 | date = May 1960 | pmid = 14446364 | doi = 10.1001/archneur.1960.03840110025004 }}</ref> || No modern equivalent – this terminology fell out of favour<ref name="pmid8644992">{{cite journal | vauthors = Schatz IJ | title = Farewell to the "Shy-Drager syndrome" | journal = Annals of Internal Medicine | volume = 125 | issue = 1 | pages = 74–75 | date = July 1996 | pmid = 8644992 | doi = 10.7326/0003-4819-125-1-199607010-00012 | s2cid = 8594266 }}</ref> and was not specified in the 2007 consensus paper.<ref name="pmid18725592"/> The earlier consensus of 1998<ref name="Gilman 94–8"/> referred to MSA-A, "a" = autonomic dysfunction subtype but this subtype is no longer used. |} The term ''olivopontocerebellar atrophy'' was originally coined by [[Joseph Jules Dejerine]] and [[André Antoine Henri Thomas|André Thomas]].<ref>{{WhoNamedIt|synd|1903}} - "Dejerine-Thomas atrophy"</ref><ref>J. J. Dejerine, A. Thomas. L’atrophie olivo-ponto-cérébelleuse. Nouvelle iconographie de la Salpêtrière, Paris, 1900, 13: 330-370. 1912, 25: 223-250.</ref> It was subdivided as: {| class="wikitable" ! Number ! [[OMIM]] ! Alt. name ! Inheritance |- | '''OPCA type 2''' | {{OMIM|258300||none}} |Fickler<ref>Fickler, A. Klinische und pathologisch-anatomische Beitraege zu den Erkrankungen des Kleinhirns. Dtsch. Z. Nervenheilk. 41: 306-375, 1911.</ref>-Winkler<ref>Winkler, C. A case of olivo-pontine cerebellar atrophy and our conceptions of neo- and palaio-cerebellum. Schweiz. Arch. Neurol. Psychiat. 13: 684-702, 1923.</ref> type OPCA | [[autosomal recessive]] |- | '''OPCA type 5''' | {{OMIM|164700||none}} | OPCA with [[dementia]] and [[extrapyramidal signs]] | [[autosomal dominant]] |} Non-hereditary diseases formerly categorized as olivopontocerebellar atrophy have were reclassified as forms of MSA<ref>[https://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=mesh&cmd=search&term=multiple+system+atrophy MeSH Result<!-- Bot generated title -->]</ref> as well as to four hereditary types, that have been currently reclassified as four different forms of [[spinocerebellar ataxia]]: {| class="wikitable" ! Hereditary OPCA type !! OPCA name !! [[spinocerebellar ataxia|SCA]] # !! Gene !! [[OMIM]] |- | OPCA type 1 || "Menzel type OPCA" || SCA1 || [[ATXN1]] || {{OMIM|164400||none}} |- | OPCA type 2, [[autosomal dominant]] || "Holguin type OPCA" || SCA2|| [[ATXN2]] || {{OMIM|183090||none}} |- | OPCA type 3 || "OPCA with retinal degeneration" || SCA7 || [[ATXN7]] || {{OMIM|164500||none}} |- | OPCA type 4 || "Schut-Haymaker type OPCA"|| SCA1 || [[ATXN1]] || {{OMIM|164400||none}} |}
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