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Fatigable weakness of bulbar muscles

MedGen UID:
868199
Concept ID:
C4022591
Finding
HPO: HP:0030192

Definition

A type of weakness of the bulbar muscles (muscles of the mouth and throat responsible for speech and swallowing) that occurs after a muscle group is used and lessens if the muscle group has some rest. That is, there is diminution of strength with repetitive muscle actions. [from HPO]

Professional guidelines

PubMed

Melzer N, Ruck T, Fuhr P, Gold R, Hohlfeld R, Marx A, Melms A, Tackenberg B, Schalke B, Schneider-Gold C, Zimprich F, Meuth SG, Wiendl H
J Neurol 2016 Aug;263(8):1473-94. Epub 2016 Feb 17 doi: 10.1007/s00415-016-8045-z. PMID: 26886206Free PMC Article

Recent clinical studies

Etiology

Dunaway Young S, McGrattan K, Johnson E, van der Heul M, Duong T, Bakke M, Werlauff U, Pasternak A, Cattaneo C, Hoffman K, Fanelli L, Breaks A, Allison K, Baranello G, Finkel R, Coratti G, Lofra RM
J Neuromuscul Dis 2023;10(4):639-652. doi: 10.3233/JND-221672. PMID: 37212069Free PMC Article
Finsterer J
Orphanet J Rare Dis 2019 Feb 26;14(1):57. doi: 10.1186/s13023-019-1025-5. PMID: 30808424Free PMC Article
Souza PV, Batistella GN, Lino VC, Pinto WB, Annes M, Oliveira AS
Arq Neuropsiquiatr 2016 Sep;74(9):750-760. doi: 10.1590/0004-282X20160106. PMID: 27706425
Melzer N, Ruck T, Fuhr P, Gold R, Hohlfeld R, Marx A, Melms A, Tackenberg B, Schalke B, Schneider-Gold C, Zimprich F, Meuth SG, Wiendl H
J Neurol 2016 Aug;263(8):1473-94. Epub 2016 Feb 17 doi: 10.1007/s00415-016-8045-z. PMID: 26886206Free PMC Article
Juel VC, Massey JM
Orphanet J Rare Dis 2007 Nov 6;2:44. doi: 10.1186/1750-1172-2-44. PMID: 17986328Free PMC Article

Diagnosis

Estephan EP, Baima JPS, Zambon AA
Arq Neuropsiquiatr 2022 May;80(5 Suppl 1):257-265. doi: 10.1590/0004-282X-ANP-2022-S105. PMID: 35976295Free PMC Article
Finsterer J
Orphanet J Rare Dis 2019 Feb 26;14(1):57. doi: 10.1186/s13023-019-1025-5. PMID: 30808424Free PMC Article
Hehir MK, Silvestri NJ
Neurol Clin 2018 May;36(2):253-260. doi: 10.1016/j.ncl.2018.01.002. PMID: 29655448
Melzer N, Ruck T, Fuhr P, Gold R, Hohlfeld R, Marx A, Melms A, Tackenberg B, Schalke B, Schneider-Gold C, Zimprich F, Meuth SG, Wiendl H
J Neurol 2016 Aug;263(8):1473-94. Epub 2016 Feb 17 doi: 10.1007/s00415-016-8045-z. PMID: 26886206Free PMC Article
Gwathmey KG, Burns TM
Semin Neurol 2015 Aug;35(4):327-39. Epub 2015 Oct 6 doi: 10.1055/s-0035-1558975. PMID: 26502757

Therapy

Finsterer J
Orphanet J Rare Dis 2019 Feb 26;14(1):57. doi: 10.1186/s13023-019-1025-5. PMID: 30808424Free PMC Article
Birnbaum S, Sharshar T, Eymard B, Theaudin M, Portero P, Hogrel JY
BMC Neurol 2018 Sep 18;18(1):145. doi: 10.1186/s12883-018-1150-0. PMID: 30227849Free PMC Article
Jagathesan T, O'Brien MD
Aerosp Med Hum Perform 2017 Jan 1;88(1):30-33. doi: 10.3357/AMHP.4724.2017. PMID: 28061919
Gwathmey KG, Burns TM
Semin Neurol 2015 Aug;35(4):327-39. Epub 2015 Oct 6 doi: 10.1055/s-0035-1558975. PMID: 26502757
Silvestri NJ, Wolfe GI
Semin Neurol 2012 Jul;32(3):215-26. Epub 2012 Nov 1 doi: 10.1055/s-0032-1329200. PMID: 23117946

Prognosis

Thebault S, Gibbs E, Bourque P, McKim D, Rakhra K, Breiner A, Frykman H, Warman-Chardon J
Neuromuscul Disord 2021 Dec;31(12):1279-1281. Epub 2021 Sep 6 doi: 10.1016/j.nmd.2021.08.014. PMID: 34690051
Lucchini M, Bortolani S, Monforte M, Papacci M, Ricci E, Mirabella M, Tasca G
Neurol Neuroimmunol Neuroinflamm 2021 Jul;8(4) Epub 2021 May 19 doi: 10.1212/NXI.0000000000001016. PMID: 34011678Free PMC Article
Francini-Pesenti F, Vitturi N, Tresso S, Sorarù G
Rev Neurol (Paris) 2020 Dec;176(10):780-787. Epub 2020 Jul 4 doi: 10.1016/j.neurol.2020.03.020. PMID: 32631678
Hehir MK, Silvestri NJ
Neurol Clin 2018 May;36(2):253-260. doi: 10.1016/j.ncl.2018.01.002. PMID: 29655448
Juel VC, Massey JM
Orphanet J Rare Dis 2007 Nov 6;2:44. doi: 10.1186/1750-1172-2-44. PMID: 17986328Free PMC Article

Clinical prediction guides

Vakrakou AG, Karachaliou E, Chroni E, Zouvelou V, Tzanetakos D, Salakou S, Papadopoulou M, Tzartos S, Voumvourakis K, Kilidireas C, Giannopoulos S, Tsivgoulis G, Tzartos J
Front Immunol 2023;14:1212757. Epub 2023 Jul 26 doi: 10.3389/fimmu.2023.1212757. PMID: 37564637Free PMC Article
Aguirre F, Fernández RN, Arrejoría RM, Manin A, Cores VE, Sivori M, Villa AM
Neurologia (Engl Ed) 2023 Jul-Aug;38(6):405-411. Epub 2022 Jul 14 doi: 10.1016/j.nrleng.2020.09.006. PMID: 35842128
Cleanthous S, Mork AC, Regnault A, Cano S, Kaminski HJ, Morel T
Orphanet J Rare Dis 2021 Oct 30;16(1):457. doi: 10.1186/s13023-021-02064-0. PMID: 34717694Free PMC Article

Recent systematic reviews

Finsterer J
Orphanet J Rare Dis 2019 Feb 26;14(1):57. doi: 10.1186/s13023-019-1025-5. PMID: 30808424Free PMC Article

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