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Proximal Hereditary Motor Neuropathy Type I

MedGen UID:
21913
Concept ID:
C0043116
Disease or Syndrome
Synonym: HMN (Hereditary Motor Neuropathy) Proximal Type I

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVProximal Hereditary Motor Neuropathy Type I
Follow this link to review classifications for Proximal Hereditary Motor Neuropathy Type I in Orphanet.

Professional guidelines

PubMed

Beloribi-Djefaflia S, Attarian S
Rev Neurol (Paris) 2023 Jan-Feb;179(1-2):35-48. Epub 2022 Dec 30 doi: 10.1016/j.neurol.2022.11.006. PMID: 36588067
Yiu EM, Bray P, Baets J, Baker SK, Barisic N, de Valle K, Estilow T, Farrar MA, Finkel RS, Haberlová J, Kennedy RA, Moroni I, Nicholson GA, Ramchandren S, Reilly MM, Rose K, Shy ME, Siskind CE, Yum SW, Menezes MP, Ryan MM, Burns J
J Neurol Neurosurg Psychiatry 2022 May;93(5):530-538. Epub 2022 Feb 9 doi: 10.1136/jnnp-2021-328483. PMID: 35140138
Stino AM, Naddaf E, Dyck PJ, Dyck PJB
Muscle Nerve 2021 Feb;63(2):157-169. Epub 2020 Sep 11 doi: 10.1002/mus.27046. PMID: 32914902

Recent clinical studies

Etiology

Tazir M, Nouioua S
Rev Neurol (Paris) 2024 Dec;180(10):1031-1036. Epub 2024 May 3 doi: 10.1016/j.neurol.2023.09.005. PMID: 38702287
Record CJ, Skorupinska M, Laura M, Rossor AM, Pareyson D, Pisciotta C, Feely SME, Lloyd TE, Horvath R, Sadjadi R, Herrmann DN, Li J, Walk D, Yum SW, Lewis RA, Day J, Burns J, Finkel RS, Saporta MA, Ramchandren S, Weiss MD, Acsadi G, Fridman V, Muntoni F, Poh R, Polke JM, Zuchner S, Shy ME, Scherer SS, Reilly MM; Inherited Neuropathies Consortium—Rare Disease Clinical Research Network
Brain 2023 Oct 3;146(10):4336-4349. doi: 10.1093/brain/awad187. PMID: 37284795Free PMC Article
Frasquet M, Sevilla T
Curr Opin Neurol 2022 Oct 1;35(5):562-570. Epub 2022 Aug 8 doi: 10.1097/WCO.0000000000001087. PMID: 35942667
Eggermann K, Gess B, Häusler M, Weis J, Hahn A, Kurth I
Dtsch Arztebl Int 2018 Feb 9;115(6):91-97. doi: 10.3238/arztebl.2018.0091. PMID: 29478438Free PMC Article
Fink JK
Acta Neuropathol 2013 Sep;126(3):307-28. Epub 2013 Jul 30 doi: 10.1007/s00401-013-1115-8. PMID: 23897027Free PMC Article

Diagnosis

Tazir M, Nouioua S
Rev Neurol (Paris) 2024 Dec;180(10):1031-1036. Epub 2024 May 3 doi: 10.1016/j.neurol.2023.09.005. PMID: 38702287
Frasquet M, Sevilla T
Curr Opin Neurol 2022 Oct 1;35(5):562-570. Epub 2022 Aug 8 doi: 10.1097/WCO.0000000000001087. PMID: 35942667
Eggermann K, Gess B, Häusler M, Weis J, Hahn A, Kurth I
Dtsch Arztebl Int 2018 Feb 9;115(6):91-97. doi: 10.3238/arztebl.2018.0091. PMID: 29478438Free PMC Article
Li J
Semin Neurol 2012 Jul;32(3):204-14. Epub 2012 Nov 1 doi: 10.1055/s-0032-1329198. PMID: 23117945Free PMC Article
Kleopa KA, Scherer SS
Neurol Clin 2002 Aug;20(3):679-709. doi: 10.1016/s0733-8619(01)00016-0. PMID: 12432826

Therapy

Chen X, Dong T, Hu Y, De Pace R, Mattera R, Eberhardt K, Ziegler M, Pirovolakis T, Sahin M, Bonifacino JS, Ebrahimi-Fakhari D, Gray SJ
J Clin Invest 2023 May 15;133(10) doi: 10.1172/JCI164575. PMID: 36951961Free PMC Article
Haqq AM, Chung WK, Dollfus H, Haws RM, Martos-Moreno GÁ, Poitou C, Yanovski JA, Mittleman RS, Yuan G, Forsythe E, Clément K, Argente J
Lancet Diabetes Endocrinol 2022 Dec;10(12):859-868. Epub 2022 Nov 7 doi: 10.1016/S2213-8587(22)00277-7. PMID: 36356613Free PMC Article
Stino AM, Naddaf E, Dyck PJ, Dyck PJB
Muscle Nerve 2021 Feb;63(2):157-169. Epub 2020 Sep 11 doi: 10.1002/mus.27046. PMID: 32914902
Morena J, Gupta A, Hoyle JC
Int J Mol Sci 2019 Jul 12;20(14) doi: 10.3390/ijms20143419. PMID: 31336816Free PMC Article
Pareyson D, Marchesi C
Lancet Neurol 2009 Jul;8(7):654-67. doi: 10.1016/S1474-4422(09)70110-3. PMID: 19539237

Prognosis

Sivera Mascaró R, García Sobrino T, Horga Hernández A, Pelayo Negro AL, Alonso Jiménez A, Antelo Pose A, Calabria Gallego MD, Casasnovas C, Cemillán Fernández CA, Esteban Pérez J, Fenollar Cortés M, Frasquet Carrera M, Gallano Petit MP, Giménez Muñoz A, Gutiérrez Gutiérrez G, Gutiérrez Martínez A, Juntas Morales R, Ciano-Petersen NL, Martínez Ulloa PL, Mederer Hengstl S, Millet Sancho E, Navacerrada Barrero FJ, Navarrete Faubel FE, Pardo Fernández J, Pascual Pascual SI, Pérez Lucas J, Pino Mínguez J, Rabasa Pérez M, Sánchez González M, Sotoca J, Rodríguez Santiago B, Rojas García R, Turon-Sans J, Vicent Carsí V, Sevilla Mantecón T
Neurologia (Engl Ed) 2025 Apr;40(3):290-305. Epub 2024 Mar 1 doi: 10.1016/j.nrleng.2024.02.008. PMID: 38431252
Dong H, Qin B, Zhang H, Lei L, Wu S
Biomolecules 2024 Sep 9;14(9) doi: 10.3390/biom14091138. PMID: 39334903Free PMC Article
Méreaux JL, Banneau G, Papin M, Coarelli G, Valter R, Raymond L, Kol B, Ariste O, Parodi L, Tissier L, Mairey M, Ait Said S, Gautier C, Guillaud-Bataille M; French SPATAX clinical network, Forlani S, de la Grange P, Brice A, Vazza G, Durr A, Leguern E, Stevanin G
Brain 2022 Apr 29;145(3):1029-1037. doi: 10.1093/brain/awab386. PMID: 34983064
Klein CJ
Continuum (Minneap Minn) 2020 Oct;26(5):1224-1256. doi: 10.1212/CON.0000000000000927. PMID: 33003000
Pearson TS, Pons R, Ghaoui R, Sue CM
Mov Disord 2019 May;34(5):625-636. Epub 2019 Mar 26 doi: 10.1002/mds.27655. PMID: 30913345

Clinical prediction guides

Tazir M, Nouioua S
Rev Neurol (Paris) 2024 Dec;180(10):1031-1036. Epub 2024 May 3 doi: 10.1016/j.neurol.2023.09.005. PMID: 38702287
Record CJ, Skorupinska M, Laura M, Rossor AM, Pareyson D, Pisciotta C, Feely SME, Lloyd TE, Horvath R, Sadjadi R, Herrmann DN, Li J, Walk D, Yum SW, Lewis RA, Day J, Burns J, Finkel RS, Saporta MA, Ramchandren S, Weiss MD, Acsadi G, Fridman V, Muntoni F, Poh R, Polke JM, Zuchner S, Shy ME, Scherer SS, Reilly MM; Inherited Neuropathies Consortium—Rare Disease Clinical Research Network
Brain 2023 Oct 3;146(10):4336-4349. doi: 10.1093/brain/awad187. PMID: 37284795Free PMC Article
Pipis M, Feely SME, Polke JM, Skorupinska M, Perez L, Shy RR, Laura M, Morrow JM, Moroni I, Pisciotta C, Taroni F, Vujovic D, Lloyd TE, Acsadi G, Yum SW, Lewis RA, Finkel RS, Herrmann DN, Day JW, Li J, Saporta M, Sadjadi R, Walk D, Burns J, Muntoni F, Ramchandren S, Horvath R, Johnson NE, Züchner S, Pareyson D, Scherer SS, Rossor AM, Shy ME, Reilly MM; Inherited Neuropathies Consortium - Rare Disease Clinical Research Network (INC-RDCRN)
Brain 2020 Dec 1;143(12):3589-3602. doi: 10.1093/brain/awaa323. PMID: 33415332Free PMC Article
Klein CJ
Continuum (Minneap Minn) 2020 Oct;26(5):1224-1256. doi: 10.1212/CON.0000000000000927. PMID: 33003000
Rinaldi R, Patel A
PM R 2013 May;5(5 Suppl):S63-73. Epub 2013 Mar 28 doi: 10.1016/j.pmrj.2013.03.028. PMID: 23542772

Recent systematic reviews

Cesaroni CA, Caiazza L, Pisanò G, Gnazzo M, Sigona G, Rizzi S, Pantani A, Frattini D, Fusco C
Genes (Basel) 2025 Oct 29;16(11) doi: 10.3390/genes16111279. PMID: 41300731Free PMC Article
Conde RM, Senem I, Dos Santos M, de Lima Osório F, Marques Júnior W
J Peripher Nerv Syst 2023 Jun;28(2):169-178. Epub 2023 Apr 24 doi: 10.1111/jns.12548. PMID: 37060329
Finsterer J, Iglseder S, Wanschitz J, Topakian R, Löscher WN, Grisold W
Acta Neurol Scand 2019 Feb;139(2):92-105. Epub 2018 Oct 23 doi: 10.1111/ane.13035. PMID: 30295933
Corrado B, Ciardi G, Bargigli C
Medicine (Baltimore) 2016 Apr;95(17):e3278. doi: 10.1097/MD.0000000000003278. PMID: 27124017Free PMC Article
Ruano L, Melo C, Silva MC, Coutinho P
Neuroepidemiology 2014;42(3):174-83. Epub 2014 Mar 5 doi: 10.1159/000358801. PMID: 24603320

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