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Multiple system atrophy
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=== Clinical === Clinical diagnostic criteria were defined in 1998<ref name="Gilman 94–8">{{cite journal | vauthors = Gilman S, Low PA, Quinn N, Albanese A, Ben-Shlomo Y, Fowler CJ, Kaufmann H, Klockgether T, Lang AE, Lantos PL, Litvan I, Mathias CJ, Oliver E, Robertson D, Schatz I, Wenning GK | display-authors = 6 | title = Consensus statement on the diagnosis of multiple system atrophy | journal = Journal of the Neurological Sciences | volume = 163 | issue = 1 | pages = 94–98 | date = February 1999 | pmid = 10223419 | doi = 10.1016/s0022-510x(98)00304-9 | hdl-access = free | s2cid = 13307970 | hdl = 2027.42/41757 }}</ref> and updated in 2007<ref name="pmid18725592"/> and in 2022.<ref>{{cite journal| vauthors = Wenning GK, Stankovic I, Vignatelli L, Fanciulli A, Calandra-Buonaura G, Seppi K, Palma JA, Meissner WG, Krismer F, Berg D, Cortelli P, Freeman R, Halliday G, Höglinger G, Lang A, Ling H, Litvan I, Low P, Miki Y, Panicker J, Pellecchia MT, Quinn N, Sakakibara R, Stamelou M, Tolosa E, Tsuji S, Warner T, Poewe W, Kaufmann H | title = The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy | journal = Movement Disorders | date = 2022 | volume = 37 | issue = 6 | pages = 1131–48 | pmid = 35445419 | doi = 10.1002/mds.29005 | pmc = 9321158 | hdl = 11585/899814 | hdl-access = free }}</ref> Certain signs and symptoms of MSA also occur with other disorders, such as Parkinson's disease, making the diagnosis more difficult.<ref>{{cite web |url= http://www.mc.vanderbilt.edu/root/vumc.php?site=adc&doc=4791 | work = Vanderbilt Autonomic Dysfunction Center |title=Multiple System Atrophy / Shy Drager Syndrome |access-date = May 29, 2010 }}</ref><ref>{{cite journal | vauthors = Bloomfield SM, Hanna PA, Noor ER, Dalvi AI |date=2018-09-24 | veditors = Benbadis SR | url=http://emedicine.medscape.com/article/1154074-overview#a2|title=multiple system atrophy overview | journal = Medscape | publisher = WebMD LLC }}</ref><ref name="pmid26138942">{{cite journal | vauthors = Koga S, Aoki N, Uitti RJ, van Gerpen JA, Cheshire WP, Josephs KA, Wszolek ZK, Langston JW, Dickson DW | display-authors = 6 | title = When DLB, PD, and PSP masquerade as MSA: an autopsy study of 134 patients | journal = Neurology | volume = 85 | issue = 5 | pages = 404–412 | date = August 2015 | pmid = 26138942 | pmc = 4534078 | doi = 10.1212/WNL.0000000000001807 }}</ref> Features characteristic of OPCA include progressive [[cerebellar ataxia]], leading to clumsiness in body movements, veering from midline when walking, wide-based stance, and falls without signs of [[paralysis]] or weakness.<ref name=Landers>{{cite journal|last=Landers|first=M|author2=Adams M |author3=Acosta K |author4=Fox A. |title=Challenge-oriented gait and balance training in sporadic olivopontocerebellar atrophy: a case study.|journal=J Neurol Phys Ther|year=2009|volume=33|issue=3|pages=160–168|doi=10.1097/npt.0b013e3181b511f4|pmid=19809395|s2cid=24642594|doi-access=free}}</ref><ref name=Berciano>{{cite journal|last=Berciano|first=J|author2=Boesch S |author3=Pérez-Ramos JM |author4=Wenning GK |title=Olivopontocerebellar atrophy: toward a better nosological definition.|journal=Mov. Disord.|year=2006|volume=21|issue=10|pages=1607–13|doi=10.1002/mds.21052|pmid=16874757|s2cid=38376147}}</ref> Clinical presentation can vary greatly between patients, but mostly affects speech, balance and walking.<ref>{{Cite web |url=https://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001765/ |title=Olivopontocerebellar atrophy - PubMed Health |access-date=2017-09-07 |archive-date=2012-11-17 |archive-url=https://web.archive.org/web/20121117071439/http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001765/ |url-status=dead }}</ref> Other possible neurological problems include [[spasmodic dysphonia]], [[hypertonia]], [[hyperreflexia]], rigidity, [[dysarthria]], [[dysphagia]] and neck [[dystonic]] posture.<ref name=Berciano/> [[Dysarthria]] is characterized by increased pauses of irregular duration, impaired coordination of vocal pitch, prolonged syllables and an overall irregular speech rhythm.<ref>{{Cite journal |last1=Hähnel |first1=Tom |last2=Nemitz |first2=Anna |last3=Schön |first3=Katja |last4=Berger |first4=Luise |last5=Vogel |first5=Annemarie |last6=Gruber |first6=Doreen |last7=Schnalke |first7=Nils |last8=Bräuer |first8=Stefan |last9=Falkenburger |first9=Björn H. |last10=Gandor |first10=Florin |date=2025-05-03 |title=Speech Differences between Multiple System Atrophy and Parkinson's Disease |journal=Movement Disorders Clinical Practice |language=en |doi=10.1002/mdc3.70094 |issn=2330-1619|doi-access=free |pmid=40317624 }}</ref> Diagnosis may be based on a thorough medical exam; the presence of signs and symptoms; imaging studies; various laboratory tests; and an evaluation of the family history.<ref>{{Cite web|url=https://rarediseases.info.nih.gov/diseases/7250/olivopontocerebellar-atrophy|title=Olivopontocerebellar atrophy | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program|access-date=19 October 2024|archive-date=21 September 2021|archive-url=https://web.archive.org/web/20210921212514/https://rarediseases.info.nih.gov/diseases/7250/olivopontocerebellar-atrophy|url-status=dead}}</ref>
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