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Myotonia with warm-up phenomenon

MedGen UID:
871108
Concept ID:
C4025575
Disease or Syndrome
HPO: HP:0003740

Definition

Myotonia that occurs after a period of rest and decreases with continuing exercise. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMyotonia with warm-up phenomenon

Conditions with this feature

Congenital myotonia, autosomal recessive form
MedGen UID:
155852
Concept ID:
C0751360
Disease or Syndrome
Myotonia congenita is characterized by muscle stiffness present from childhood; all striated muscle groups including the extrinsic eye muscles, facial muscles, and tongue may be involved. Stiffness is relieved by repeated contractions of the muscle (the "warm-up" phenomenon). Muscles are usually hypertrophic. Whereas autosomal recessive (AR) myotonia congenita is often associated with more severe manifestations (such as progressive minor distal weakness and attacks of transient weakness brought on by movement after rest), autosomal dominant (AD) myotonia congenita is not. The age of onset varies: in AD myotonia congenita onset is usually in infancy or early childhood; in AR myotonia congenita the average age of onset is slightly older. In both AR and AD myotonia congenita onset may be as late as the third or fourth decade of life.
Congenital myotonia, autosomal dominant form
MedGen UID:
422446
Concept ID:
C2936781
Disease or Syndrome
Myotonia congenita is characterized by muscle stiffness present from childhood; all striated muscle groups including the extrinsic eye muscles, facial muscles, and tongue may be involved. Stiffness is relieved by repeated contractions of the muscle (the "warm-up" phenomenon). Muscles are usually hypertrophic. Whereas autosomal recessive (AR) myotonia congenita is often associated with more severe manifestations (such as progressive minor distal weakness and attacks of transient weakness brought on by movement after rest), autosomal dominant (AD) myotonia congenita is not. The age of onset varies: in AD myotonia congenita onset is usually in infancy or early childhood; in AR myotonia congenita the average age of onset is slightly older. In both AR and AD myotonia congenita onset may be as late as the third or fourth decade of life.

Recent clinical studies

Etiology

He Y, Qiu Y, Xiong Y, Shen Y, Jiang K, Yi H, Huang P, Zhu Y, Zhu M, Zhou M, Hong D, Tan D
Channels (Austin) 2024 Dec;18(1):2349823. Epub 2024 May 8 doi: 10.1080/19336950.2024.2349823. PMID: 38720415Free PMC Article
Li Y, Li M, Wang Z, Yang F, Wang H, Bai X, Sun B, Chen S, Huang X
Channels (Austin) 2022 Dec;16(1):35-46. doi: 10.1080/19336950.2022.2041292. PMID: 35170402Free PMC Article
Milla CP, De Castro CP, Gómez-González C, Martínez-Montero P, Pascual Pascual SI, Molano Mateos J
J Genet 2019 Sep;98 PMID: 31544778
Al-Ghamdi F, Darras BT, Ghosh PS
Pediatr Neurol 2017 May;70:26-33. Epub 2017 Feb 16 doi: 10.1016/j.pediatrneurol.2017.02.006. PMID: 28325641
Toth A, Lovadi E, Komoly S, Schwarcz A, Orsi G, Perlaki G, Bogner P, Sebok A, Kovacs N, Pal E, Janszky J
Acta Neurol Scand 2015 Jul;132(1):65-72. Epub 2015 Jan 28 doi: 10.1111/ane.12360. PMID: 25630356

Diagnosis

Kanbayashi T, Kobayashi S, Hatanaka Y, Shimizu J, Sonoo M
Intern Med 2023 Nov 15;62(22):3397-3400. Epub 2023 Apr 7 doi: 10.2169/internalmedicine.1545-22. PMID: 37032082Free PMC Article
Yuan JH, Higuchi Y, Hashiguchi A, Ando M, Yoshimura A, Nakamura T, Sakiyama Y, Takashima H
J Neurol 2022 Dec;269(12):6406-6415. Epub 2022 Jul 30 doi: 10.1007/s00415-022-11305-6. PMID: 35907044
Li Y, Li M, Wang Z, Yang F, Wang H, Bai X, Sun B, Chen S, Huang X
Channels (Austin) 2022 Dec;16(1):35-46. doi: 10.1080/19336950.2022.2041292. PMID: 35170402Free PMC Article
Milla CP, De Castro CP, Gómez-González C, Martínez-Montero P, Pascual Pascual SI, Molano Mateos J
J Genet 2019 Sep;98 PMID: 31544778
Al-Ghamdi F, Darras BT, Ghosh PS
Pediatr Neurol 2017 May;70:26-33. Epub 2017 Feb 16 doi: 10.1016/j.pediatrneurol.2017.02.006. PMID: 28325641

Therapy

Horlings CG, Drost G, Bloem BR, Trip J, Pieterse AJ, van Engelen BG, Allum JH
J Neurol Neurosurg Psychiatry 2009 Feb;80(2):207-12. Epub 2008 Jun 11 doi: 10.1136/jnnp.2008.143982. PMID: 18550628
Dupré N, Chrestian N, Bouchard JP, Rossignol E, Brunet D, Sternberg D, Brais B, Mathieu J, Puymirat J
Neuromuscul Disord 2009 May;19(5):330-4. Epub 2008 Mar 11 doi: 10.1016/j.nmd.2008.01.007. PMID: 18337100
Logigian EL, Blood CL, Dilek N, Martens WB, Moxley RT 4th, Wiegner AW, Thornton CA, Moxley RT 3rd
Muscle Nerve 2005 Jul;32(1):35-42. doi: 10.1002/mus.20339. PMID: 15880468
Nitz J, Burns Y, Wuthapanich N, Jackson R
Physiother Res Int 1999;4(1):1-11. doi: 10.1002/pri.1999.4.1.1. PMID: 10368835

Prognosis

Elaraby NM, Ahmed HA, Dawoud H, Ashaat NA, Azmy A, Galal ER, Elhusseny Y, Awady HE, Metwally AM, Ashaat EA
Mol Biol Rep 2024 Jun 15;51(1):766. doi: 10.1007/s11033-024-09646-8. PMID: 38877370

Clinical prediction guides

Elaraby NM, Ahmed HA, Dawoud H, Ashaat NA, Azmy A, Galal ER, Elhusseny Y, Awady HE, Metwally AM, Ashaat EA
Mol Biol Rep 2024 Jun 15;51(1):766. doi: 10.1007/s11033-024-09646-8. PMID: 38877370
Li Y, Li M, Wang Z, Yang F, Wang H, Bai X, Sun B, Chen S, Huang X
Channels (Austin) 2022 Dec;16(1):35-46. doi: 10.1080/19336950.2022.2041292. PMID: 35170402Free PMC Article
Trip J, Drost G, Ginjaar HB, Nieman FH, van der Kooi AJ, de Visser M, van Engelen BG, Faber CG
J Neurol Neurosurg Psychiatry 2009 Jun;80(6):647-52. Epub 2009 Feb 11 doi: 10.1136/jnnp.2008.162396. PMID: 19211598

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