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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
Symptomatic Care in Multiple System Atrophy: State of the Art.
Grossauer A, Sidoroff V, Heim B, Seppi K
Cerebellum 2023 Jun;22(3):433-446. Epub 2022 May 17 doi: 10.1007/s12311-022-01411-6. PMID: 35581488Free PMC Article
Multiple system atrophy is not caused by C9orf72 hexanucleotide repeat expansions.
Scholz SW, Majounie E, Revesz T, Holton JL, Okun MS, Houlden H, Singleton AB
Neurobiol Aging 2015 Feb;36(2):1223.e1-2. Epub 2014 Sep 6 doi: 10.1016/j.neurobiolaging.2014.08.033. PMID: 25281017Free PMC Article
Cardiovascular autonomic dysfunction in patients with movement disorders.
Walter BL
Cleve Clin J Med 2008 Mar;75 Suppl 2:S54-8. doi: 10.3949/ccjm.75.suppl_2.s54. PMID: 18540148
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
Imaging synucleinopathies.
Brooks DJ, Tambasco N
Mov Disord 2016 Jun;31(6):814-29. Epub 2016 Feb 16 doi: 10.1002/mds.26547. PMID: 26879635
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

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
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
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

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
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
SPECT Molecular Imaging in Atypical Parkinsonism.
Brumberg J, Isaias IU
Int Rev Neurobiol 2018;142:37-65. Epub 2018 Aug 29 doi: 10.1016/bs.irn.2018.08.006. PMID: 30409259
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

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
Orthostatic Hypotension in Multiple System Atrophy: Related Factors and Disease Prognosis.
Jiang Q, Zhang L, Lin J, Wei Q, Li C, Hou Y, Ou R, Liu K, Yang T, Xiao Y, Zhao B, Wu Y, Shang H
J Parkinsons Dis 2023;13(8):1313-1320. doi: 10.3233/JPD-230095. PMID: 38143372Free PMC Article
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: a clinical and neuropathological perspective.
Ubhi K, Low P, Masliah E
Trends Neurosci 2011 Nov;34(11):581-90. Epub 2011 Sep 29 doi: 10.1016/j.tins.2011.08.003. PMID: 21962754Free PMC Article

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
Infectious Agents as Potential Drivers of α-Synucleinopathies.
Linard M, Ravier A, Mougué L, Grgurina I, Boutillier AL, Foubert-Samier A, Blanc F, Helmer C
Mov Disord 2022 Mar;37(3):464-477. Epub 2022 Jan 18 doi: 10.1002/mds.28925. PMID: 35040520
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
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
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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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