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Generalized bulbospinal muscular atrophy

MedGen UID:
1842387
Concept ID:
C5680795
Disease or Syndrome
Orphanet: ORPHA206710

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVGeneralized bulbospinal muscular atrophy

Professional guidelines

PubMed

Gregg AR, Aarabi M, Klugman S, Leach NT, Bashford MT, Goldwaser T, Chen E, Sparks TN, Reddi HV, Rajkovic A, Dungan JS; ACMG Professional Practice and Guidelines Committee
Genet Med 2021 Oct;23(10):1793-1806. Epub 2021 Jul 20 doi: 10.1038/s41436-021-01203-z. PMID: 34285390Free PMC Article
Ye Z, Reintam Blaser A, Lytvyn L, Wang Y, Guyatt GH, Mikita JS, Roberts J, Agoritsas T, Bertschy S, Boroli F, Camsooksai J, Du B, Heen AF, Lu J, Mella JM, Vandvik PO, Wise R, Zheng Y, Liu L, Siemieniuk RAC
BMJ 2020 Jan 6;368:l6722. doi: 10.1136/bmj.l6722. PMID: 31907223
Rinaldi C, Wood MJA
Nat Rev Neurol 2018 Jan;14(1):9-21. Epub 2017 Dec 1 doi: 10.1038/nrneurol.2017.148. PMID: 29192260

Recent clinical studies

Etiology

Zambon AA, Pini V, Bosco L, Falzone YM, Munot P, Muntoni F, Previtali SC
Brain 2023 Mar 1;146(3):806-822. doi: 10.1093/brain/awac452. PMID: 36445400Free PMC Article
Blair HA
CNS Drugs 2022 Sep;36(9):995-1005. Epub 2022 Aug 12 doi: 10.1007/s40263-022-00941-1. PMID: 35960489
Paik J
CNS Drugs 2022 Apr;36(4):401-410. Epub 2022 Mar 13 doi: 10.1007/s40263-022-00910-8. PMID: 35284988
Fan HC, Ho LI, Chi CS, Chen SJ, Peng GS, Chan TM, Lin SZ, Harn HJ
Cell Transplant 2014;23(4-5):441-58. doi: 10.3727/096368914X678454. PMID: 24816443
D'Amico A, Mercuri E, Tiziano FD, Bertini E
Orphanet J Rare Dis 2011 Nov 2;6:71. doi: 10.1186/1750-1172-6-71. PMID: 22047105Free PMC Article

Diagnosis

Rathore G, Kang PB
Pediatr Neurol 2023 Dec;149:1-14. Epub 2023 Aug 29 doi: 10.1016/j.pediatrneurol.2023.08.034. PMID: 37757659
Younger DS
Handb Clin Neurol 2023;195:401-423. doi: 10.1016/B978-0-323-98818-6.00011-X. PMID: 37562880
Paik J
CNS Drugs 2022 Apr;36(4):401-410. Epub 2022 Mar 13 doi: 10.1007/s40263-022-00910-8. PMID: 35284988
D'Amico A, Mercuri E, Tiziano FD, Bertini E
Orphanet J Rare Dis 2011 Nov 2;6:71. doi: 10.1186/1750-1172-6-71. PMID: 22047105Free PMC Article
Lunn MR, Wang CH
Lancet 2008 Jun 21;371(9630):2120-33. doi: 10.1016/S0140-6736(08)60921-6. PMID: 18572081

Therapy

Egli M, Manoharan M
Nucleic Acids Res 2023 Apr 11;51(6):2529-2573. doi: 10.1093/nar/gkad067. PMID: 36881759Free PMC Article
Paik J
CNS Drugs 2022 Apr;36(4):401-410. Epub 2022 Mar 13 doi: 10.1007/s40263-022-00910-8. PMID: 35284988
De Vivo DC, Bertini E, Swoboda KJ, Hwu WL, Crawford TO, Finkel RS, Kirschner J, Kuntz NL, Parsons JA, Ryan MM, Butterfield RJ, Topaloglu H, Ben-Omran T, Sansone VA, Jong YJ, Shu F, Staropoli JF, Kerr D, Sandrock AW, Stebbins C, Petrillo M, Braley G, Johnson K, Foster R, Gheuens S, Bhan I, Reyna SP, Fradette S, Farwell W; NURTURE Study Group
Neuromuscul Disord 2019 Nov;29(11):842-856. Epub 2019 Sep 12 doi: 10.1016/j.nmd.2019.09.007. PMID: 31704158Free PMC Article
Rinaldi C, Wood MJA
Nat Rev Neurol 2018 Jan;14(1):9-21. Epub 2017 Dec 1 doi: 10.1038/nrneurol.2017.148. PMID: 29192260
Lunn MR, Wang CH
Lancet 2008 Jun 21;371(9630):2120-33. doi: 10.1016/S0140-6736(08)60921-6. PMID: 18572081

Prognosis

Blair HA
CNS Drugs 2022 Sep;36(9):995-1005. Epub 2022 Aug 12 doi: 10.1007/s40263-022-00941-1. PMID: 35960489
Morton SU, Christodoulou J, Costain G, Muntoni F, Wakeling E, Wojcik MH, French CE, Szuto A, Dowling JJ, Cohn RD, Raymond FL, Darras BT, Williams DA, Lunke S, Stark Z, Rowitch DH, Agrawal PB
JAMA Neurol 2022 Apr 1;79(4):405-413. doi: 10.1001/jamaneurol.2022.0067. PMID: 35254387Free PMC Article
De Vivo DC, Bertini E, Swoboda KJ, Hwu WL, Crawford TO, Finkel RS, Kirschner J, Kuntz NL, Parsons JA, Ryan MM, Butterfield RJ, Topaloglu H, Ben-Omran T, Sansone VA, Jong YJ, Shu F, Staropoli JF, Kerr D, Sandrock AW, Stebbins C, Petrillo M, Braley G, Johnson K, Foster R, Gheuens S, Bhan I, Reyna SP, Fradette S, Farwell W; NURTURE Study Group
Neuromuscul Disord 2019 Nov;29(11):842-856. Epub 2019 Sep 12 doi: 10.1016/j.nmd.2019.09.007. PMID: 31704158Free PMC Article
Kolb SJ, Coffey CS, Yankey JW, Krosschell K, Arnold WD, Rutkove SB, Swoboda KJ, Reyna SP, Sakonju A, Darras BT, Shell R, Kuntz N, Castro D, Parsons J, Connolly AM, Chiriboga CA, McDonald C, Burnette WB, Werner K, Thangarajh M, Shieh PB, Finanger E, Cudkowicz ME, McGovern MM, McNeil DE, Finkel R, Iannaccone ST, Kaye E, Kingsley A, Renusch SR, McGovern VL, Wang X, Zaworski PG, Prior TW, Burghes AHM, Bartlett A, Kissel JT; NeuroNEXT Clinical Trial Network on behalf of the NN101 SMA Biomarker Investigators
Ann Neurol 2017 Dec;82(6):883-891. Epub 2017 Dec 8 doi: 10.1002/ana.25101. PMID: 29149772Free PMC Article
D'Amico A, Mercuri E, Tiziano FD, Bertini E
Orphanet J Rare Dis 2011 Nov 2;6:71. doi: 10.1186/1750-1172-6-71. PMID: 22047105Free PMC Article

Clinical prediction guides

Crawford TO, Swoboda KJ, De Vivo DC, Bertini E, Hwu WL, Finkel RS, Kirschner J, Kuntz NL, Nazario AN, Parsons JA, Pechmann A, Ryan MM, Butterfield RJ, Topaloglu H, Ben-Omran T, Sansone VA, Jong YJ, Shu F, Zhu C, Raynaud S, Lago TR, Paradis AD, Foster R, Chin R, Berger Z; NURTURE Study Group
Muscle Nerve 2023 Aug;68(2):157-170. Epub 2023 Jul 6 doi: 10.1002/mus.27853. PMID: 37409780
Guglieri M, Bushby K, McDermott MP, Hart KA, Tawil R, Martens WB, Herr BE, McColl E, Speed C, Wilkinson J, Kirschner J, King WM, Eagle M, Brown MW, Willis T, Griggs RC; FOR-DMD Investigators of the Muscle Study Group, Straub V, van Ruiten H, Childs AM, Ciafaloni E, Shieh PB, Spinty S, Maggi L, Baranello G, Butterfield RJ, Horrocks IA, Roper H, Alhaswani Z, Flanigan KM, Kuntz NL, Manzur A, Darras BT, Kang PB, Morrison L, Krzesniak-Swinarska M, Mah JK, Mongini TE, Ricci F, von der Hagen M, Finkel RS, O'Reardon K, Wicklund M, Kumar A, McDonald CM, Han JJ, Joyce N, Henricson EK, Schara-Schmidt U, Gangfuss A, Wilichowski E, Barohn RJ, Statland JM, Campbell C, Vita G, Vita GL, Howard JF Jr, Hughes I, McMillan HJ, Pegoraro E, Bello L, Burnette WB, Thangarajh M, Chang T
JAMA 2022 Apr 19;327(15):1456-1468. doi: 10.1001/jama.2022.4315. PMID: 35381069Free PMC Article
Morton SU, Christodoulou J, Costain G, Muntoni F, Wakeling E, Wojcik MH, French CE, Szuto A, Dowling JJ, Cohn RD, Raymond FL, Darras BT, Williams DA, Lunke S, Stark Z, Rowitch DH, Agrawal PB
JAMA Neurol 2022 Apr 1;79(4):405-413. doi: 10.1001/jamaneurol.2022.0067. PMID: 35254387Free PMC Article
Day JW, Mendell JR, Mercuri E, Finkel RS, Strauss KA, Kleyn A, Tauscher-Wisniewski S, Tukov FF, Reyna SP, Chand DH
Drug Saf 2021 Oct;44(10):1109-1119. Epub 2021 Aug 12 doi: 10.1007/s40264-021-01107-6. PMID: 34383289Free PMC Article
Kolb SJ, Coffey CS, Yankey JW, Krosschell K, Arnold WD, Rutkove SB, Swoboda KJ, Reyna SP, Sakonju A, Darras BT, Shell R, Kuntz N, Castro D, Parsons J, Connolly AM, Chiriboga CA, McDonald C, Burnette WB, Werner K, Thangarajh M, Shieh PB, Finanger E, Cudkowicz ME, McGovern MM, McNeil DE, Finkel R, Iannaccone ST, Kaye E, Kingsley A, Renusch SR, McGovern VL, Wang X, Zaworski PG, Prior TW, Burghes AHM, Bartlett A, Kissel JT; NeuroNEXT Clinical Trial Network on behalf of the NN101 SMA Biomarker Investigators
Ann Neurol 2017 Dec;82(6):883-891. Epub 2017 Dec 8 doi: 10.1002/ana.25101. PMID: 29149772Free PMC Article

Recent systematic reviews

Aponte Ribero V, Martí Y, Batson S, Mitchell S, Gorni K, Gusset N, Oskoui M, Servais L, Sutherland CS
Neurology 2023 Nov 21;101(21):e2103-e2113. Epub 2023 Oct 9 doi: 10.1212/WNL.0000000000207878. PMID: 37813581Free PMC Article
Al-Taie A, Köseoğlu A
Paediatr Respir Rev 2023 Dec;48:65-71. Epub 2023 Jul 13 doi: 10.1016/j.prrv.2023.06.004. PMID: 37563072
Paracha N, Hudson P, Mitchell S, Sutherland CS
Pharmacoeconomics 2022 Apr;40(Suppl 1):69-89. Epub 2021 Oct 18 doi: 10.1007/s40273-021-01095-6. PMID: 34658008Free PMC Article
Wadman RI, van der Pol WL, Bosboom WM, Asselman FL, van den Berg LH, Iannaccone ST, Vrancken AF
Cochrane Database Syst Rev 2020 Jan 6;1(1):CD006282. doi: 10.1002/14651858.CD006282.pub5. PMID: 32006461Free PMC Article
Wadman RI, Bosboom WM, van der Pol WL, van den Berg LH, Wokke JH, Iannaccone ST, Vrancken AF
Cochrane Database Syst Rev 2012 Apr 18;(4):CD006282. doi: 10.1002/14651858.CD006282.pub4. PMID: 22513940

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