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Multiple system atrophy(MSA)

MedGen UID:
98276
•Concept ID:
C0393571
•
Pathologic Function
Synonyms: MSA; Multiple system atrophy (MSA) with orthostatic hypotension; Multiple system atrophy with autonomic failure; Orthostatic hypotension, bladder and bowel incontinence, anhidrosis, iris atrophy, amyotrophy, ataxia, rigidity and tremor; Shy-Drager syndrome
SNOMED CT: Multiple system atrophy (230297002); MSA - Multiple system atrophy (230297002); Shy-Drager syndrome (230297002)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
•Concept ID:
C0600599
•
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Non-Mendelian inheritance (Orphanet)
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0007803
Orphanet: ORPHA102

Definition

Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by autonomic failure (cardiovascular and/or urinary), parkinsonism, cerebellar impairment and corticospinal signs with a median survival of 6-9 years. [from ORDO]

Professional guidelines

PubMed

The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy.
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
Mov Disord 2022 Jun;37(6):1131-1148. Epub 2022 Apr 21 doi: 10.1002/mds.29005. PMID: 35445419Free PMC Article
Autonomic dysfunction: Diagnosis and management.
Rafanelli M, Walsh K, Hamdan MH, Buyan-Dent L
Handb Clin Neurol 2019;167:123-137. doi: 10.1016/B978-0-12-804766-8.00008-X. PMID: 31753129
The Differential Diagnosis and Treatment of Atypical Parkinsonism.
Levin J, Kurz A, Arzberger T, Giese A, Höglinger GU
Dtsch Arztebl Int 2016 Feb 5;113(5):61-9. doi: 10.3238/arztebl.2016.0061. PMID: 26900156Free PMC Article

Curated

UK NICE Clinical guideline (CG148), Urinary incontinence in neurological disease: assessment and management, 2023

Recent clinical studies

Etiology

Synucleinopathies.
Lamotte G, Singer W
Handb Clin Neurol 2023;196:175-202. doi: 10.1016/B978-0-323-98817-9.00032-6. PMID: 37620069
Neuropathology of autonomic dysfunction in synucleinopathies.
Coon EA, Cutsforth-Gregory JK, Benarroch EE
Mov Disord 2018 Mar;33(3):349-358. Epub 2018 Jan 3 doi: 10.1002/mds.27186. PMID: 29297596
Multiple system atrophy: Building a global community - 30years of advocacy efforts.
Bower PG
Auton Neurosci 2018 May;211:39-42. Epub 2017 Dec 16 doi: 10.1016/j.autneu.2017.12.006. PMID: 29269241
Multiple system atrophy: clinicopathological characteristics in Japanese patients.
Ozawa T, Onodera O
Proc Jpn Acad Ser B Phys Biol Sci 2017;93(5):251-258. doi: 10.2183/pjab.93.016. PMID: 28496050Free PMC Article
Multiple system atrophy.
Wenning GK, Geser F
Rev Neurol (Paris) 2003 May;159(5 Pt 2):3S31-8. PMID: 12773886

Diagnosis

Inflammation in multiple system atrophy.
Leńska-Mieciek M, Madetko-Alster N, Alster P, Królicki L, Fiszer U, Koziorowski D
Front Immunol 2023;14:1214677. Epub 2023 Jun 22 doi: 10.3389/fimmu.2023.1214677. PMID: 37426656Free PMC Article
Multiple system atrophy.
Goh YY, Saunders E, Pavey S, Rushton E, Quinn N, Houlden H, Chelban V
Pract Neurol 2023 Jun;23(3):208-221. Epub 2023 Mar 16 doi: 10.1136/pn-2020-002797. PMID: 36927875Free PMC Article
Multiple system atrophy.
Poewe W, Stankovic I, Halliday G, Meissner WG, Wenning GK, Pellecchia MT, Seppi K, Palma JA, Kaufmann H
Nat Rev Dis Primers 2022 Aug 25;8(1):56. doi: 10.1038/s41572-022-00382-6. PMID: 36008429
Multiple system atrophy.
Fanciulli A, Stankovic I, Krismer F, Seppi K, Levin J, Wenning GK
Int Rev Neurobiol 2019;149:137-192. Epub 2019 Nov 21 doi: 10.1016/bs.irn.2019.10.004. PMID: 31779811
Multiple-system atrophy.
Fanciulli A, Wenning GK
N Engl J Med 2015 Jan 15;372(3):249-63. doi: 10.1056/NEJMra1311488. PMID: 25587949

Therapy

Mitochondrial Dysfunction and Neurodegenerative Disorders: Role of Nutritional Supplementation.
Mantle D, Hargreaves IP
Int J Mol Sci 2022 Oct 20;23(20) doi: 10.3390/ijms232012603. PMID: 36293457Free PMC Article
Risk and predictors of dementia and parkinsonism in idiopathic REM sleep behaviour disorder: a multicentre study.
Postuma RB, Iranzo A, Hu M, Högl B, Boeve BF, Manni R, Oertel WH, Arnulf I, Ferini-Strambi L, Puligheddu M, Antelmi E, Cochen De Cock V, Arnaldi D, Mollenhauer B, Videnovic A, Sonka K, Jung KY, Kunz D, Dauvilliers Y, Provini F, Lewis SJ, Buskova J, Pavlova M, Heidbreder A, Montplaisir JY, Santamaria J, Barber TR, Stefani A, St Louis EK, Terzaghi M, Janzen A, Leu-Semenescu S, Plazzi G, Nobili F, Sixel-Doering F, Dusek P, Bes F, Cortelli P, Ehgoetz Martens K, Gagnon JF, Gaig C, Zucconi M, Trenkwalder C, Gan-Or Z, Lo C, Rolinski M, Mahlknecht P, Holzknecht E, Boeve AR, Teigen LN, Toscano G, Mayer G, Morbelli S, Dawson B, Pelletier A
Brain 2019 Mar 1;142(3):744-759. doi: 10.1093/brain/awz030. PMID: 30789229Free PMC Article
(18)F-FDG PET in Parkinsonism: Differential Diagnosis and Evaluation of Cognitive Impairment.
Meyer PT, Frings L, Rücker G, Hellwig S
J Nucl Med 2017 Dec;58(12):1888-1898. Epub 2017 Sep 14 doi: 10.2967/jnumed.116.186403. PMID: 28912150
Neuroinflammation in Synucleinopathies.
Lim S, Chun Y, Lee JS, Lee SJ
Brain Pathol 2016 May;26(3):404-9. doi: 10.1111/bpa.12371. PMID: 26940152Free PMC Article
Multiple-system atrophy.
Fanciulli A, Wenning GK
N Engl J Med 2015 Jan 15;372(3):249-63. doi: 10.1056/NEJMra1311488. PMID: 25587949

Prognosis

Cutaneous α-Synuclein Signatures in Patients With Multiple System Atrophy and Parkinson Disease.
Gibbons C, Wang N, Rajan S, Kern D, Palma JA, Kaufmann H, Freeman R
Neurology 2023 Apr 11;100(15):e1529-e1539. Epub 2023 Jan 19 doi: 10.1212/WNL.0000000000206772. PMID: 36657992Free PMC Article
Multiple system atrophy.
Poewe W, Stankovic I, Halliday G, Meissner WG, Wenning GK, Pellecchia MT, Seppi K, Palma JA, Kaufmann H
Nat Rev Dis Primers 2022 Aug 25;8(1):56. doi: 10.1038/s41572-022-00382-6. PMID: 36008429
Magnetic resonance imaging in dementia.
Raposo Rodríguez L, Tovar Salazar DJ, Fernández García N, Pastor Hernández L, Fernández Guinea Ó
Radiologia (Engl Ed) 2018 Nov-Dec;60(6):476-484. Epub 2018 Jun 11 doi: 10.1016/j.rx.2018.04.003. PMID: 29903629
Alpha-synucleinopathies.
Alafuzoff I, Hartikainen P
Handb Clin Neurol 2017;145:339-353. doi: 10.1016/B978-0-12-802395-2.00024-9. PMID: 28987181
α-Synucleinopathy phenotypes.
McCann H, Stevens CH, Cartwright H, Halliday GM
Parkinsonism Relat Disord 2014 Jan;20 Suppl 1:S62-7. doi: 10.1016/S1353-8020(13)70017-8. PMID: 24262191

Clinical prediction guides

An update on multiple system atrophy.
Stankovic I, Kuijpers M, Kaufmann H
Curr Opin Neurol 2024 Aug 1;37(4):400-408. Epub 2024 Jun 3 doi: 10.1097/WCO.0000000000001285. PMID: 38828714Free PMC Article
Progressive supranuclear palsy, multiple system atrophy and corticobasal degeneration.
Fabbrini G, Fabbrini A, Suppa A
Handb Clin Neurol 2019;165:155-177. doi: 10.1016/B978-0-444-64012-3.00009-5. PMID: 31727210
Alpha-synucleinopathies.
Alafuzoff I, Hartikainen P
Handb Clin Neurol 2017;145:339-353. doi: 10.1016/B978-0-12-802395-2.00024-9. PMID: 28987181
Multiple system atrophy.
Peeraully T
Semin Neurol 2014 Apr;34(2):174-81. Epub 2014 Jun 25 doi: 10.1055/s-0034-1381737. PMID: 24963676
Multiple system atrophy of the cerebellar type: clinical state of the art.
Lin DJ, Hermann KL, Schmahmann JD
Mov Disord 2014 Mar;29(3):294-304. Epub 2014 Feb 24 doi: 10.1002/mds.25847. PMID: 24615754

Recent systematic reviews

The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy.
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
Mov Disord 2022 Jun;37(6):1131-1148. Epub 2022 Apr 21 doi: 10.1002/mds.29005. PMID: 35445419Free PMC Article
Nocebo effect in multiple system atrophy: systematic review and meta-analysis of placebo-controlled clinical trials.
Wang ZX, Zhang NN, Zhao HX, Song J
Neurol Sci 2022 Feb;43(2):899-905. Epub 2022 Jan 1 doi: 10.1007/s10072-021-05758-2. PMID: 34973075Free PMC Article
Cerebrospinal Fluid Biomarkers in Multiple System Atrophy Relative to Parkinson's Disease: A Meta-Analysis.
Xie D, Feng L, Huang H, Zhao Q, Ning P, Shen Q, Lu H, Xu F, Xu Y
Behav Neurol 2021;2021:5559383. Epub 2021 May 31 doi: 10.1155/2021/5559383. PMID: 34158872Free PMC Article
Diagnostic utility of fluid biomarkers in multiple system atrophy: a systematic review and meta-analysis.
Cong S, Xiang C, Wang H, Cong S
J Neurol 2021 Aug;268(8):2703-2712. Epub 2020 Mar 11 doi: 10.1007/s00415-020-09781-9. PMID: 32162061
Multiple System Atrophy (MSA) and smoking: a meta-analysis and mechanistic insights.
Tseng FS, Deng X, Ong YL, Li HH, Tan EK
Aging (Albany NY) 2020 Nov 7;12(21):21959-21970. doi: 10.18632/aging.104021. PMID: 33161394Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
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      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NICE, 2023
      UK NICE Clinical guideline (CG148), Urinary incontinence in neurological disease: assessment and management, 2023

    Reviews

    Related information

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      Clinical tests in GTR
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      Related Medical Subject Headings
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