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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.
 
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

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
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
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

Lamotte G, Singer W
Handb Clin Neurol 2023;196:175-202. doi: 10.1016/B978-0-323-98817-9.00032-6. PMID: 37620069
Rusz J, Tykalová T, Salerno G, Bancone S, Scarpelli J, Pellecchia MT
J Neurol 2019 Jun;266(6):1394-1404. Epub 2019 Mar 11 doi: 10.1007/s00415-019-09271-7. PMID: 30859316
Krismer F, Wenning GK
Nat Rev Neurol 2017 Apr;13(4):232-243. Epub 2017 Mar 17 doi: 10.1038/nrneurol.2017.26. PMID: 28303913
Ogawa T, Sakakibara R, Kuno S, Ishizuka O, Kitta T, Yoshimura N
Nat Rev Urol 2017 Feb;14(2):79-89. Epub 2016 Dec 13 doi: 10.1038/nrurol.2016.254. PMID: 27958390
Stanzani-Maserati M, Gallassi R, Calandra-Buonaura G, Alessandria M, Oppi F, Poda R, Sambati L, Provini F, Cortelli P
Eur Neurol 2014;72(5-6):349-59. Epub 2014 Oct 14 doi: 10.1159/000364903. PMID: 25322750

Diagnosis

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
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
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
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
Fanciulli A, Wenning GK
N Engl J Med 2015 Jan 15;372(3):249-63. doi: 10.1056/NEJMra1311488. PMID: 25587949

Therapy

Mantle D, Hargreaves IP
Int J Mol Sci 2022 Oct 20;23(20) doi: 10.3390/ijms232012603. PMID: 36293457Free PMC Article
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
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
Krismer F, Wenning GK
Nat Rev Neurol 2017 Apr;13(4):232-243. Epub 2017 Mar 17 doi: 10.1038/nrneurol.2017.26. PMID: 28303913
Fanciulli A, Wenning GK
N Engl J Med 2015 Jan 15;372(3):249-63. doi: 10.1056/NEJMra1311488. PMID: 25587949

Prognosis

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
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
Calandra-Buonaura G, Alfonsi E, Vignatelli L, Benarroch EE, Giannini G, Iranzo A, Low PA, Martinelli P, Provini F, Quinn N, Tolosa E, Wenning GK, Abbruzzese G, Bower P, Antonini A, Bhatia KP, Bonavita J, Pellecchia MT, Pizzorni N, Tison F, Ghorayeb I, Meissner WG, Ozawa T, Pacchetti C, Pozzi NG, Vicini C, Schindler A, Cortelli P, Kaufmann H
Parkinsonism Relat Disord 2021 May;86:124-132. Epub 2021 Mar 30 doi: 10.1016/j.parkreldis.2021.03.027. PMID: 33839029
De Pablo-Fernández E, Lees AJ, Holton JL, Warner TT
JAMA Neurol 2019 Apr 1;76(4):470-479. doi: 10.1001/jamaneurol.2018.4377. PMID: 30640364Free PMC Article
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

Clinical prediction guides

Zhang L, Cao B, Hou Y, Gu X, Wei QQ, Ou R, Zhao B, Song W, Shang H
Neurology 2022 Jan 4;98(1):e73-e82. Epub 2021 Oct 18 doi: 10.1212/WNL.0000000000012968. PMID: 34663646
Lin JY, Zhang LY, Cao B, Wei QQ, Ou RW, Hou YB, Liu KC, Xu XR, Jiang Z, Gu XJ, Liu J, Shang HF
Chin Med J (Engl) 2020 Nov 23;134(6):690-698. doi: 10.1097/CM9.0000000000001211. PMID: 33234871Free PMC Article
Eschlböck S, Delazer M, Krismer F, Bodner T, Fanciulli A, Heim B, Heras Garvin A, Kaindlstorfer C, Karner E, Mair K, Rabensteiner C, Raccagni C, Seppi K, Poewe W, Wenning GK
Ann Clin Transl Neurol 2020 Feb;7(2):219-228. Epub 2020 Feb 7 doi: 10.1002/acn3.50987. PMID: 32031752Free PMC Article
Alafuzoff I, Hartikainen P
Handb Clin Neurol 2017;145:339-353. doi: 10.1016/B978-0-12-802395-2.00024-9. PMID: 28987181
Peeraully T
Semin Neurol 2014 Apr;34(2):174-81. Epub 2014 Jun 25 doi: 10.1055/s-0034-1381737. PMID: 24963676

Recent systematic reviews

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
Ma X, Wang Y, Wang N, Zhang R
J Neurol 2022 Mar;269(3):1272-1281. Epub 2021 Jul 10 doi: 10.1007/s00415-021-10703-6. PMID: 34245345
Wittke C, Petkovic S, Dobricic V, Schaake S; MDS‐endorsed PSP Study Group, Respondek G, Weissbach A, Madoev H, Trinh J, Vollstedt EJ, Kuhnke N, Lohmann K, Dulovic Mahlow M, Marras C, König IR, Stamelou M, Bonifati V, Lill CM, Kasten M, Huppertz HJ, Höglinger G, Klein C
Mov Disord 2021 Jul;36(7):1499-1510. Epub 2021 Mar 19 doi: 10.1002/mds.28517. PMID: 34396589Free PMC Article
Cortelli P, Calandra-Buonaura G, Benarroch EE, Giannini G, Iranzo A, Low PA, Martinelli P, Provini F, Quinn N, Tolosa E, Wenning GK, Abbruzzese G, Bower P, Alfonsi E, Ghorayeb I, Ozawa T, Pacchetti C, Pozzi NG, Vicini C, Antonini A, Bhatia KP, Bonavita J, Kaufmann H, Pellecchia MT, Pizzorni N, Schindler A, Tison F, Vignatelli L, Meissner WG
Neurology 2019 Oct 1;93(14):630-639. doi: 10.1212/WNL.0000000000008208. PMID: 31570638Free PMC Article
Respondek G, Kurz C, Arzberger T, Compta Y, Englund E, Ferguson LW, Gelpi E, Giese A, Irwin DJ, Meissner WG, Nilsson C, Pantelyat A, Rajput A, van Swieten JC, Troakes C, Josephs KA, Lang AE, Mollenhauer B, Müller U, Whitwell JL, Antonini A, Bhatia KP, Bordelon Y, Corvol JC, Colosimo C, Dodel R, Grossman M, Kassubek J, Krismer F, Levin J, Lorenzl S, Morris H, Nestor P, Oertel WH, Rabinovici GD, Rowe JB, van Eimeren T, Wenning GK, Boxer A, Golbe LI, Litvan I, Stamelou M, Höglinger GU; Movement Disorder Society-Endorsed PSP Study Group
Mov Disord 2017 Jul;32(7):995-1005. Epub 2017 May 13 doi: 10.1002/mds.27034. PMID: 28500752Free PMC Article

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

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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

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