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Progressive muscular atrophy

MedGen UID:
214634
Concept ID:
C0917981
Disease or Syndrome
Synonyms: Atrophies, Progressive Muscular; Atrophy, Progressive Muscular; Muscular Atrophies, Progressive; Muscular Atrophy, Progressive; Myelopathic Muscular Atrophy, Progressive; Progressive Muscular Atrophies; Progressive Muscular Atrophy; Progressive Myelopathic Muscular Atrophy
SNOMED CT: PMA - Progressive muscular atrophy (88923002); Progressive muscular atrophy (88923002); Duchenne-Aran muscular atrophy (88923002); Pure progressive muscular atrophy (88923002)

Definition

A rare, milder form of amyotrophic lateral sclerosis. It is characterized by a slowly progressive clinical course. Signs and symptoms include muscle weakness, atrophy, and fasciculation. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProgressive muscular atrophy

Professional guidelines

PubMed

Watanabe S, Sekiguchi K, Noda Y, Matsumoto R
J Clin Neuromuscul Dis 2022 Jun 1;23(4):175-182. doi: 10.1097/CND.0000000000000401. PMID: 35608640Free PMC Article
Pugdahl K, Camdessanché JP, Cengiz B, de Carvalho M, Liguori R, Rossatto C, Oliveira Santos M, Vacchiano V, Johnsen B
Clin Neurophysiol 2021 Dec;132(12):3183-3189. Epub 2021 Sep 8 doi: 10.1016/j.clinph.2021.08.014. PMID: 34544646
Cooper-Knock J, Shaw PJ, Kirby J
Acta Neuropathol 2014 Mar;127(3):333-45. Epub 2014 Feb 4 doi: 10.1007/s00401-014-1251-9. PMID: 24493408Free PMC Article

Recent clinical studies

Etiology

Maranzano A, Verde F, Colombo E, Poletti B, Doretti A, Bonetti R, Gagliardi D, Meneri M, Maderna L, Messina S, Corti S, Morelli C, Silani V, Ticozzi N
Brain 2023 Oct 3;146(10):4105-4116. doi: 10.1093/brain/awad129. PMID: 37075222Free PMC Article
Zhang X, Yang K, Le W
Adv Exp Med Biol 2020;1207:53-74. doi: 10.1007/978-981-15-4272-5_3. PMID: 32671738
Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
Juntas Morales R, Pageot N, Taieb G, Camu W
Rev Neurol (Paris) 2017 May;173(5):308-319. Epub 2017 Apr 26 doi: 10.1016/j.neurol.2017.03.015. PMID: 28456383
Patterson MC
Handb Clin Neurol 2013;113:1707-8. doi: 10.1016/B978-0-444-59565-2.00039-3. PMID: 23622392

Diagnosis

Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
Liewluck T, Saperstein DS
Neurol Clin 2015 Nov;33(4):761-73. doi: 10.1016/j.ncl.2015.07.005. PMID: 26515620
Patterson MC
Handb Clin Neurol 2013;113:1707-8. doi: 10.1016/B978-0-444-59565-2.00039-3. PMID: 23622392
Rowland LP
Muscle Nerve 2010 Feb;41(2):161-5. doi: 10.1002/mus.21565. PMID: 20082312
Belsh JM
Neurology 1999;53(8 Suppl 5):S26-30; discussion S35-6. PMID: 10560634

Therapy

Gould RL, McDermott CJ, Thompson BJ, Rawlinson CV, Bursnall M, Bradburn M, Kumar P, Turton EJ, White DA, Serfaty MA, Graham CD, McCracken LM, Goldstein LH, Al-Chalabi A, Orrell RW, Williams T, Noad R, Baker I, Faull C, Lambert T, Chhetri SK, Ealing J, Hanratty A, Radunovic A, Gunawardana N, Meadows G, Gorrie GH, Young T, Lawrence V, Cooper C, Shaw PJ, Howard RJ; COMMEND Collaboration Group
Lancet 2024 Jun 1;403(10442):2381-2394. Epub 2024 May 9 doi: 10.1016/S0140-6736(24)00533-6. PMID: 38735299
de Wit J, Vervoort SCJM, van Eerden E, van den Berg LH, Visser-Meily JMA, Beelen A, Schröder CD
BMC Psychol 2019 Jun 15;7(1):35. doi: 10.1186/s40359-019-0308-x. PMID: 31202270Free PMC Article
Tard C, Defebvre L, Moreau C, Devos D, Danel-Brunaud V
Rev Neurol (Paris) 2017 May;173(5):263-272. Epub 2017 May 4 doi: 10.1016/j.neurol.2017.03.029. PMID: 28477850
Liewluck T, Saperstein DS
Neurol Clin 2015 Nov;33(4):761-73. doi: 10.1016/j.ncl.2015.07.005. PMID: 26515620
Meamar R, Nasr-Esfahani MH, Mousavi SA, Basiri K
J Clin Neurosci 2013 Dec;20(12):1659-63. Epub 2013 Oct 19 doi: 10.1016/j.jocn.2013.04.024. PMID: 24148693

Prognosis

Maranzano A, Verde F, Colombo E, Poletti B, Doretti A, Bonetti R, Gagliardi D, Meneri M, Maderna L, Messina S, Corti S, Morelli C, Silani V, Ticozzi N
Brain 2023 Oct 3;146(10):4105-4116. doi: 10.1093/brain/awad129. PMID: 37075222Free PMC Article
Moreno-Gambín MI, Tembl JI, Mazón M, Cañada-Martínez AJ, Martí-Bonmatí L, Sevilla T, Vázquez-Costa JF
J Neurol 2022 Mar;269(3):1631-1640. Epub 2021 Aug 11 doi: 10.1007/s00415-021-10729-w. PMID: 34379200Free PMC Article
Zappa G, LoMauro A, Baranello G, Cavallo E, Corti P, Mastella C, Costantino MA
J Neurodev Disord 2021 Feb 2;13(1):9. doi: 10.1186/s11689-021-09355-4. PMID: 33530934Free PMC Article
Tard C, Defebvre L, Moreau C, Devos D, Danel-Brunaud V
Rev Neurol (Paris) 2017 May;173(5):263-272. Epub 2017 May 4 doi: 10.1016/j.neurol.2017.03.029. PMID: 28477850
Visser J, van den Berg-Vos RM, Franssen H, van den Berg LH, Wokke JH, de Jong JM, Holman R, de Haan RJ, de Visser M
Arch Neurol 2007 Apr;64(4):522-8. doi: 10.1001/archneur.64.4.522. PMID: 17420313

Clinical prediction guides

Maranzano A, Verde F, Colombo E, Poletti B, Doretti A, Bonetti R, Gagliardi D, Meneri M, Maderna L, Messina S, Corti S, Morelli C, Silani V, Ticozzi N
Brain 2023 Oct 3;146(10):4105-4116. doi: 10.1093/brain/awad129. PMID: 37075222Free PMC Article
Walusinski O
Rev Neurol (Paris) 2022 Apr;178(4):298-305. Epub 2021 Sep 23 doi: 10.1016/j.neurol.2021.06.011. PMID: 34565621
Moreno-Gambín MI, Tembl JI, Mazón M, Cañada-Martínez AJ, Martí-Bonmatí L, Sevilla T, Vázquez-Costa JF
J Neurol 2022 Mar;269(3):1631-1640. Epub 2021 Aug 11 doi: 10.1007/s00415-021-10729-w. PMID: 34379200Free PMC Article
Pinto WBVR, Debona R, Nunes PP, Assis ACD, Lopes CG, Bortholin T, Dias RB, Naylor FGM, Chieia MAT, Souza PVS, Oliveira ASB
Rev Neurol (Paris) 2019 Apr;175(4):221-232. Epub 2019 Mar 4 doi: 10.1016/j.neurol.2018.04.016. PMID: 30846210
Kira J
Intern Med 2002 Mar;41(3):169-74. doi: 10.2169/internalmedicine.41.169. PMID: 11929175

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