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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 a mixture of major and minor genetic determinants possibly together with environmental factors. Diseases inherited in this manner are termed complex diseases.
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
Pharmacologic treatment of orthostatic hypotension.
Park JW, Okamoto LE, Shibao CA, Biaggioni I
Auton Neurosci 2020 Dec;229:102721. Epub 2020 Aug 28 doi: 10.1016/j.autneu.2020.102721. PMID: 32979782Free PMC Article
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

Recent clinical studies

Etiology

Neurofilament light levels predict clinical progression and death in multiple system atrophy.
Chelban V, Nikram E, Perez-Soriano A, Wilke C, Foubert-Samier A, Vijiaratnam N, Guo T, Jabbari E, Olufodun S, Gonzalez M, Senkevich K, Laurens B, Péran P, Rascol O, Le Traon AP, Todd EG, Costantini AA, Alikhwan S, Tariq A, Ng BL, Muñoz E, Painous C, Compta Y, Junque C, Segura B, Zhelcheska K, Wellington H, Schöls L, Jaunmuktane Z, Kobylecki C, Church A, Hu MTM, Rowe JB, Leigh PN, Massey L, Burn DJ, Pavese N, Foltynie T, Pchelina S, Wood N, Heslegrave AJ, Zetterberg H, Bocchetta M, Rohrer JD, Marti MJ, Synofzik M, Morris HR, Meissner WG, Houlden H
Brain 2022 Dec 19;145(12):4398-4408. doi: 10.1093/brain/awac253. PMID: 35903017Free PMC Article
Cerebrospinal Fluid Metabolome in Parkinson's Disease and Multiple System Atrophy.
Kwon DH, Hwang JS, Kim SG, Jang YE, Shin TH, Lee G
Int J Mol Sci 2022 Feb 7;23(3) doi: 10.3390/ijms23031879. PMID: 35163800Free PMC Article
Repetitive transcranial magnetic stimulation of the cerebellum improves ataxia and cerebello-fronto plasticity in multiple system atrophy: a randomized, double-blind, sham-controlled and TMS-EEG study.
Song P, Li S, Wang S, Wei H, Lin H, Wang Y
Aging (Albany NY) 2020 Oct 21;12(20):20611-20622. doi: 10.18632/aging.103946. PMID: 33085647Free PMC Article
Synucleinopathies.
Coon EA, Singer W
Continuum (Minneap Minn) 2020 Feb;26(1):72-92. doi: 10.1212/CON.0000000000000819. PMID: 31996623Free 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

Diagnosis

Multiple System Atrophy.
Claassen DO
Continuum (Minneap Minn) 2022 Oct 1;28(5):1350-1363. doi: 10.1212/CON.0000000000001154. PMID: 36222769
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
Diagnosis of multiple system atrophy.
Palma JA, Norcliffe-Kaufmann L, Kaufmann H
Auton Neurosci 2018 May;211:15-25. Epub 2017 Oct 23 doi: 10.1016/j.autneu.2017.10.007. PMID: 29111419Free 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

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
Mesenchymal Stromal Cell Therapies for Neurodegenerative Diseases.
Staff NP, Jones DT, Singer W
Mayo Clin Proc 2019 May;94(5):892-905. doi: 10.1016/j.mayocp.2019.01.001. PMID: 31054608Free 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

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: Recent Developments and Future Perspectives.
Meissner WG, Fernagut PO, Dehay B, Péran P, Traon AP, Foubert-Samier A, Lopez Cuina M, Bezard E, Tison F, Rascol O
Mov Disord 2019 Nov;34(11):1629-1642. Epub 2019 Nov 6 doi: 10.1002/mds.27894. PMID: 31692132
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
The neuropathology of the adult cerebellum.
Koeppen AH
Handb Clin Neurol 2018;154:129-149. doi: 10.1016/B978-0-444-63956-1.00008-4. PMID: 29903436Free PMC Article
Parkinsonism.
Keener AM, Bordelon YM
Semin Neurol 2016 Aug;36(4):330-4. Epub 2016 Sep 19 doi: 10.1055/s-0036-1585097. PMID: 27643900

Clinical prediction guides

Cerebrospinal Fluid Metabolome in Parkinson's Disease and Multiple System Atrophy.
Kwon DH, Hwang JS, Kim SG, Jang YE, Shin TH, Lee G
Int J Mol Sci 2022 Feb 7;23(3) doi: 10.3390/ijms23031879. PMID: 35163800Free PMC Article
Diagnostic value of cerebrospinal fluid alpha-synuclein seed quantification in synucleinopathies.
Poggiolini I, Gupta V, Lawton M, Lee S, El-Turabi A, Querejeta-Coma A, Trenkwalder C, Sixel-Döring F, Foubert-Samier A, Pavy-Le Traon A, Plazzi G, Biscarini F, Montplaisir J, Gagnon JF, Postuma RB, Antelmi E, Meissner WG, Mollenhauer B, Ben-Shlomo Y, Hu MT, Parkkinen L
Brain 2022 Apr 18;145(2):584-595. doi: 10.1093/brain/awab431. PMID: 34894214Free 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
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
Rare Neurodegenerative Diseases: Clinical and Genetic Update.
Matilla-Dueñas A, Corral-Juan M, Rodríguez-Palmero Seuma A, Vilas D, Ispierto L, Morais S, Sequeiros J, Alonso I, Volpini V, Serrano-Munuera C, Pintos-Morell G, Álvarez R, Sánchez I
Adv Exp Med Biol 2017;1031:443-496. doi: 10.1007/978-3-319-67144-4_25. PMID: 29214587

Recent systematic reviews

Effects of Palliative Care for Progressive Neurologic Diseases: A Systematic Review and Meta-Analysis.
Chan LML, Yan OY, Lee JJJ, Lam WWT, Lin CC, Auyeung M, Bloem BR, Kwok JYY
J Am Med Dir Assoc 2023 Feb;24(2):171-184. Epub 2022 Dec 5 doi: 10.1016/j.jamda.2022.11.001. PMID: 36481217
Diagnostic value of α-synuclein seeding amplification assays in α-synucleinopathies: A systematic review and meta-analysis.
Yoo D, Bang JI, Ahn C, Nyaga VN, Kim YE, Kang MJ, Ahn TB
Parkinsonism Relat Disord 2022 Nov;104:99-109. Epub 2022 Oct 19 doi: 10.1016/j.parkreldis.2022.10.007. PMID: 36289019
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
Deep brain stimulation for atypical parkinsonism: A systematic review on efficacy and safety.
Artusi CA, Rinaldi D, Balestrino R, Lopiano L
Parkinsonism Relat Disord 2022 Mar;96:109-118. Epub 2022 Mar 9 doi: 10.1016/j.parkreldis.2022.03.002. PMID: 35288028
Stridor in multiple system atrophy: Consensus statement on diagnosis, prognosis, and treatment.
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

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