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Multiple system atrophy
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==Signs and symptoms== MSA is characterized by the following: '''Autonomic and at least one Motor''' (clinically established MSA criteria 2022)<ref name="pmid10331752">{{cite journal | vauthors = Swan L, Dupont J | title = Multiple system atrophy | journal = Physical Therapy | volume = 79 | issue = 5 | pages = 488–494 | date = May 1999 | pmid = 10331752 | doi = 10.1093/ptj/79.5.488 | doi-access = free }}</ref><ref name="pmid11724918">{{cite journal | vauthors = Burn DJ, Jaros E | title = Multiple system atrophy: cellular and molecular pathology | journal = Molecular Pathology | volume = 54 | issue = 6 | pages = 419–426 | date = December 2001 | pmid = 11724918 | pmc = 1187133 }}</ref> * [[autonomic dysfunction]]: Post-void urinary residual volume ≥100 mL (usually by ultrasound); Unexplained urinary urge incontinence; or Neurogenic orthostatic hypotension (≥20/10 mmHg blood pressure drop) within 3 minutes (usually by head‐up tilt) * [[parkinsonism]] ([[muscle rigidity]] +/ [[tremor]] and slow movement: '''MSA-P''') * [[cerebellar ataxia]] (Poor coordination/unsteady walking: '''MSA-C''') A variant with combined features of MSA and [[dementia with Lewy bodies]] may also exist.{{MEDRS|date=March 2018}}<ref>{{MEDRS|date=March 2018}}{{cite journal | vauthors = Sikorska B, Papierz W, Preusser M, Liberski PP, Budka H | title = Synucleinopathy with features of both multiple system atrophy and dementia with Lewy bodies | journal = Neuropathology and Applied Neurobiology | volume = 33 | issue = 1 | pages = 126–129 | date = February 2007 | pmid = 17239015 | doi = 10.1111/j.1365-2990.2006.00817.x | s2cid = 40186391 }}</ref> There have also been occasional instances of [[frontotemporal lobar degeneration]] associated with MSA.<ref name="pmid25962793">{{cite journal | vauthors = Aoki N, Boyer PJ, Lund C, Lin WL, Koga S, Ross OA, Weiner M, Lipton A, Powers JM, White CL, Dickson DW | display-authors = 6 | title = Atypical multiple system atrophy is a new subtype of frontotemporal lobar degeneration: frontotemporal lobar degeneration associated with α-synuclein | journal = Acta Neuropathologica | volume = 130 | issue = 1 | pages = 93–105 | date = July 2015 | pmid = 25962793 | pmc = 6764097 | doi = 10.1007/s00401-015-1442-z }}</ref> ===Initial presentation=== The most common first sign of MSA is the appearance of an "akinetic-rigid syndrome" (i.e. slowness of initiation of movement resembling [[Parkinson's disease]]) found in 62% at first presentation. Other common signs at onset include problems with balance (cerebellar ataxia) found in 22% at first presentation, followed by genito-urinary symptoms (9%): both men and women often experience urgency, frequency, incomplete bladder emptying, or an inability to pass urine (retention). About 1 in 5 MSA patients experience a fall in their first year of disease.<ref name="Bensimon">{{cite journal | vauthors = Bensimon G, Ludolph A, Agid Y, Vidailhet M, Payan C, Leigh PN | title = Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: the NNIPPS study | journal = Brain | volume = 132 | issue = Pt 1 | pages = 156–171 | date = January 2009 | pmid = 19029129 | pmc = 2638696 | doi = 10.1093/brain/awn291 }}</ref> For men, the first sign can be [[erectile dysfunction]]. Women have also reported reduced genital sensitivity.<ref name="pmid12671951">{{cite journal | vauthors = Oertel WH, Wächter T, Quinn NP, Ulm G, Brandstädter D | title = Reduced genital sensitivity in female patients with multiple system atrophy of parkinsonian type | journal = Movement Disorders | volume = 18 | issue = 4 | pages = 430–432 | date = April 2003 | pmid = 12671951 | doi = 10.1002/mds.10384 | s2cid = 28102026 }}</ref> ===Progression=== As the disease progresses, one of three groups of symptoms predominates. These are:<ref>{{Cite journal |last=Stępnicki |date=2018-08-20 |title=Current Concepts and Treatments of Schizophrenia |journal=Molecules |volume=23 |issue=8 |pages=2087 |doi=10.3390/molecules23082087 |doi-access= free|issn=1420-3049 |pmc=6222385 |pmid=30127324}}</ref> # [[Parkinsonism]] - slow, stiff movement, writing becomes small and spidery<ref>{{cite book |vauthors=Aminoff MJ, Greenberg DA, Simon RP | title = Clinical Neurology | publisher = Lange: McGraw-Hill Medical | edition = 6th | pages = 241–45 |year = 2005 | isbn = 978-0-07-142360-1 |chapter= Chapter 7: Movement disorders}}</ref><ref>{{cite journal | vauthors = Ogawa T, Fujii S, Kuya K, Kitao SI, Shinohara Y, Ishibashi M, Tanabe Y | title = Role of Neuroimaging on Differentiation of Parkinson's Disease and Its Related Diseases | journal = Yonago Acta Medica | volume = 61 | issue = 3 | pages = 145–155 | date = September 2018 | pmid = 30275744 | pmc = 6158357 | doi = 10.33160/yam.2018.09.001 | type = Review | quote = Parkinsonian syndromes are a group of movement disorders characterized by classical motor symptoms such as tremors, bradykinesia, and rigidity. They are most frequently due to primary neurodegenerative disease, resulting in the loss of dopaminergic nerve terminals along the nigrostriatal pathway, similar to idiopathic PD, MSA, PSP, CBD, and DLB. }}</ref> # [[Cerebellar]] dysfunction - difficulty coordinating movement and balance<ref>{{cite book | vauthors = Hodos W |chapter= Evolution of Cerebellum|title=Encyclopedia of Neuroscience|publisher=Springer|date=2009|pages=1240–1243|doi=10.1007/978-3-540-29678-2_3124|isbn=978-3-540-23735-8}}</ref> # [[Autonomic nervous system]] dysfunction - impaired automatic body functions, including one, some, or all of the following:<ref>{{DorlandsDict|nine/000950637|Autonomic nervous system}}</ref> ::* postural or [[orthostatic hypotension]], resulting in [[dizziness]] or [[fainting]] upon standing up<ref>{{cite web |url=https://www.lecturio.com/concepts/hypotension/| title=Hypotension|website=The Lecturio Medical Concept Library |access-date= 27 July 2021}}</ref> ::* [[urinary incontinence]] or [[urinary retention]]<ref>{{cite book | vauthors = Ackley B | title = Nursing diagnosis handbook : an evidence-based guide to planning care |edition=9th| publisher = Mosby | location = Maryland Heights, Mo | year = 2010 | isbn = 9780323071505 }}</ref><ref>{{cite journal| vauthors = Richard C, Amarenco G, Palma JA, Kaufmann H, Drapier S, Gamé X, Brucker B, Peyronnet B | title = Early bladder dysfunction in multiple system atrophy: who seek shall find | journal = Clin Auton Res | date = 2019 | volume = 29 | issue = 6 | pages = 625–6 | pmid = 1705345 | doi = 10.1007/s10286-019-00648-2 }}</ref><ref>{{cite journal| vauthors = Sakakibara R, Panicker J, Finazzi-Agro E, Iacovelli V, Bruschini H, Subcomittee PD,NUPC,ICS | title = A guideline for the management of bladder dysfunction in Parkinson's disease and other gait disorders | journal = Neurourol Urodyn | date = 2016 | volume = 35 | issue = 5 | pages = 551–63 | pmid = 25810035 | doi = 10.1002/nau.22764 }}</ref> ::* [[impotence]]<ref>{{cite book | vauthors = Cunningham GR, Rosen RC | chapter = Overview of male sexual dysfunction. | title = UpToDate | veditors = Martin KA | publisher = UpToDate | location = Waltham, MA | date = 2018 }}</ref> ::* [[constipation]]<ref>{{cite web |url=https://www.lecturio.com/concepts/constipation/| title=Constipation|website=The Lecturio Medical Concept Library |access-date= 27 July 2021}}</ref> ::* [[vocal cord]] paralysis ::* [[dry mouth]] and skin ::* trouble regulating [[body temperature]] due to [[sweating deficiency]] in all parts of the body ::* loud snoring, [[abnormal breathing]] or inspiratory [[stridor]] during sleep ::* other sleep disorders including [[sleep apnea]], [[Rapid eye movement sleep behavior disorder|REM behavior disorder]]<ref>{{cite journal | vauthors = Gilman S, Koeppe RA, Chervin RD, Consens FB, Little R, An H, Junck L, Heumann M | display-authors = 6 | title = REM sleep behavior disorder is related to striatal monoaminergic deficit in MSA | journal = Neurology | volume = 61 | issue = 1 | pages = 29–34 | date = July 2003 | pmid = 12847152 | doi = 10.1212/01.wnl.0000073745.68744.94 | s2cid = 9538306 }}</ref> ::* [[diplopia|double vision]]<ref name="NIH">{{cite web |url=https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Multiple-System-Atrophy |title=What is multiple system atrophy? |author=<!--Not stated--> |website=NIH |access-date=November 25, 2018 }}</ref> ::*[[fasciculations|muscle twitches]]<ref name="NIH"/> ::* Cognitive impairment<ref name="pmid20576697">{{cite journal|author3-link=Bernhard Landwehrmeyer | vauthors = Brown RG, Lacomblez L, Landwehrmeyer BG, Bak T, Uttner I, Dubois B, Agid Y, Ludolph A, Bensimon G, Payan C, Leigh NP | display-authors = 6 | title = Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy | journal = Brain | volume = 133 | issue = Pt 8 | pages = 2382–2393 | date = August 2010 | pmid = 20576697 | doi = 10.1093/brain/awq158 | doi-access = free }}</ref>
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