{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T06:10:40Z","timestamp":1788588640934,"version":"build-2803163510"},"reference-count":53,"publisher":"Springer Science and Business Media LLC","issue":"5","license":[{"start":{"date-parts":[[2025,8,2]],"date-time":"2025-08-02T00:00:00Z","timestamp":1754092800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"},{"start":{"date-parts":[[2025,8,2]],"date-time":"2025-08-02T00:00:00Z","timestamp":1754092800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Neurol Ther"],"published-print":{"date-parts":[[2025,10]]},"DOI":"10.1007\/s40120-025-00804-z","type":"journal-article","created":{"date-parts":[[2025,8,2]],"date-time":"2025-08-02T10:34:18Z","timestamp":1754130858000},"page":"2249-2260","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Assessing the Safety and Efficacy of Lamotrigine as Anti-myotonic Agent in Myotonic Dystrophy Type\u00a01 (DM1): A Longitudinal, Open-Label, Pilot Study"],"prefix":"10.1007","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-2303-1180","authenticated-orcid":false,"given":"Barbara","family":"Risi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nesaiba","family":"Ait Allali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2060-0279","authenticated-orcid":false,"given":"Stefano","family":"Cotti Piccinelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0007-4113-3562","authenticated-orcid":false,"given":"Filomena","family":"Caria","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-0465-5262","authenticated-orcid":false,"given":"Simona","family":"Damioli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3456-3149","authenticated-orcid":false,"given":"Beatrice","family":"Labella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8178-3112","authenticated-orcid":false,"given":"Enrica","family":"Bertella","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giorgia","family":"Giovanelli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesca","family":"Garofali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppina","family":"Margollicci","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Carugati","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-7224-8213","authenticated-orcid":false,"given":"Lucia","family":"Ferullo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-2732-5270","authenticated-orcid":false,"given":"Emanuele","family":"Olivieri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4912-818X","authenticated-orcid":false,"given":"Loris","family":"Poli","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0119-3639","authenticated-orcid":false,"given":"Alessandro","family":"Padovani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2852-7512","authenticated-orcid":false,"given":"Massimiliano","family":"Filosto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,8,2]]},"reference":[{"key":"804_CR1","doi-asserted-by":"publisher","first-page":"594","DOI":"10.1016\/j.bbadis.2014.05.019","volume":"1852","author":"G Meola","year":"2015","unstructured":"Meola G, Cardani R. Myotonic dystrophies: an update on clinical aspects, genetic, pathology, and molecular pathomechanisms. Biochim Biophys Acta. 2015;1852:594\u2013606. https:\/\/doi.org\/10.1016\/j.bbadis.2014.05.019.","journal-title":"Biochim Biophys Acta"},{"key":"804_CR2","doi-asserted-by":"publisher","first-page":"799","DOI":"10.1016\/0092-8674(92)90154-5","volume":"68","author":"JD Brook","year":"1992","unstructured":"Brook JD, McCurrach ME, Harley HG, et al. Molecular basis of myotonic dystrophy: expansion of a trinucleotide (CTG) repeat at the 3\u2032 end of a transcript encoding a protein kinase family member. Cell. 1992;68:799\u2013808. https:\/\/doi.org\/10.1016\/0092-8674(92)90154-5.","journal-title":"Cell"},{"key":"804_CR3","doi-asserted-by":"publisher","first-page":"1256","DOI":"10.1126\/science.1546326","volume":"255","author":"YH Fu","year":"1992","unstructured":"Fu YH, Pizzuti A, Fenwick RG, et al. An unstable triplet repeat in a gene related to myotonic muscular dystrophy. Science. 1992;255:1256\u20138. https:\/\/doi.org\/10.1126\/science.1546326.","journal-title":"Science"},{"key":"804_CR4","doi-asserted-by":"publisher","unstructured":"The International Myotonic Dystrophy Consortium (IDMC). New nomenclature and DNA testing guidelines for myotonic dystrophy type\u00a01 (DM1). Neurology. 2000;54:1218\u20131221. https:\/\/doi.org\/10.1212\/WNL.54.6.1218.","DOI":"10.1212\/WNL.54.6.1218"},{"key":"804_CR5","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1002\/mus.20339","volume":"32","author":"EL Logigian","year":"2005","unstructured":"Logigian EL, Blood CL, Dilek N, et al. Quantitative analysis of the \u201cwarm-up\u201d phenomenon in myotonic dystrophy type\u00a01. Muscle Nerve. 2005;32:35\u201342. https:\/\/doi.org\/10.1002\/mus.20339.","journal-title":"Muscle Nerve"},{"key":"804_CR6","doi-asserted-by":"publisher","first-page":"80","DOI":"10.1002\/ana.410140113","volume":"14","author":"EW Streib","year":"1983","unstructured":"Streib EW, Sun SF. Distribution of electrical myotonia in myotonic muscular dystrophy. Ann Neurol. 1983;14:80\u20132. https:\/\/doi.org\/10.1002\/ana.410140113.","journal-title":"Ann Neurol"},{"key":"804_CR7","doi-asserted-by":"publisher","first-page":"2379","DOI":"10.1172\/JCI117931","volume":"95","author":"JP Mounsey","year":"1995","unstructured":"Mounsey JP, Xu P, John JE, et al. Modulation of skeletal muscle sodium channels by human myotonin protein kinase. J Clin Invest. 1995;95:2379\u201384. https:\/\/doi.org\/10.1172\/JCI117931.","journal-title":"J Clin Invest"},{"key":"804_CR8","doi-asserted-by":"publisher","first-page":"12785","DOI":"10.1073\/pnas.91.26.12785","volume":"91","author":"N Yang","year":"1994","unstructured":"Yang N, Ji S, Zhou M, et al. Sodium channel mutations in paramyotonia congenita exhibit similar biophysical phenotypes in vitro. Proc Natl Acad Sci U S A. 1994;91:12785\u20139. https:\/\/doi.org\/10.1073\/pnas.91.26.12785.","journal-title":"Proc Natl Acad Sci U S A"},{"key":"804_CR9","doi-asserted-by":"publisher","first-page":"772","DOI":"10.1046\/j.1523-1755.2000.00914.x","volume":"57","author":"SC Cannon","year":"2000","unstructured":"Cannon SC. Spectrum of sodium channel disturbances in the nondystrophic myotonias and periodic paralyses. Kidney Int. 2000;57:772\u20139. https:\/\/doi.org\/10.1046\/j.1523-1755.2000.00914.x.","journal-title":"Kidney Int"},{"key":"804_CR10","doi-asserted-by":"publisher","first-page":"325","DOI":"10.1038\/ng0796-325","volume":"13","author":"S Reddy","year":"1996","unstructured":"Reddy S, Smith DBJ, Rich MM, et al. Mice lacking the myotonic dystrophy protein kinase develop a late onset progressive myopathy. Nat Genet. 1996;13:325\u201335. https:\/\/doi.org\/10.1038\/ng0796-325.","journal-title":"Nat Genet"},{"key":"804_CR11","doi-asserted-by":"publisher","first-page":"316","DOI":"10.1038\/ng0796-316","volume":"13","author":"G Jansen","year":"1996","unstructured":"Jansen G, Groenen PJTA, B\u00e4chner D, et al. Abnormal myotonic dystrophy protein kinase levels produce only mild myopathy in mice. Nat Genet. 1996;13:316\u201324. https:\/\/doi.org\/10.1038\/ng0796-316.","journal-title":"Nat Genet"},{"key":"804_CR12","doi-asserted-by":"publisher","DOI":"10.3389\/fphys.2014.00378","author":"G St\u00f6lting","year":"2014","unstructured":"St\u00f6lting G, Fischer M, Fahlke C. CLC channel function and dysfunction in health and disease. Front Physiol. 2014. https:\/\/doi.org\/10.3389\/fphys.2014.00378.","journal-title":"Front Physiol"},{"key":"804_CR13","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1113\/jphysiol.1969.sp008930","volume":"204","author":"SH Bryant","year":"1969","unstructured":"Bryant SH. Cable properties of external intercostal muscle fibres from myotonic and nonmyotonic goats. J Physiol. 1969;204:539\u201350. https:\/\/doi.org\/10.1113\/jphysiol.1969.sp008930.","journal-title":"J Physiol"},{"key":"804_CR14","doi-asserted-by":"publisher","first-page":"89","DOI":"10.1085\/jgp.50.1.89","volume":"50","author":"RJ Lipicky","year":"1966","unstructured":"Lipicky RJ, Bryant SH. Sodium, potassium, and chloride fluxes in intercostal muscle from normal goats and goats with hereditary myotonia. J Gen Physiol. 1966;50:89\u2013111. https:\/\/doi.org\/10.1085\/jgp.50.1.89.","journal-title":"J Gen Physiol"},{"key":"804_CR15","doi-asserted-by":"publisher","first-page":"2091","DOI":"10.1172\/JCI106703","volume":"50","author":"RJ Lipicky","year":"1971","unstructured":"Lipicky RJ, Bryant SH, Salmon JH. Cable parameters, sodium, potassium, chloride, and water content, and potassium efflux in isolated external intercostal muscle of normal volunteers and patients with myotonia congenita. J Clin Invest. 1971;50:2091\u2013103. https:\/\/doi.org\/10.1172\/JCI106703.","journal-title":"J Clin Invest"},{"key":"804_CR16","doi-asserted-by":"publisher","first-page":"1281","DOI":"10.1042\/BST0371281","volume":"37","author":"JE Lee","year":"2009","unstructured":"Lee JE, Cooper TA. Pathogenic mechanisms of myotonic dystrophy. Biochem Soc Trans. 2009;37:1281\u20136. https:\/\/doi.org\/10.1042\/BST0371281.","journal-title":"Biochem Soc Trans"},{"key":"804_CR17","doi-asserted-by":"publisher","first-page":"79","DOI":"10.1085\/jgp.200609635","volume":"129","author":"JD Lueck","year":"2007","unstructured":"Lueck JD, Mankodi A, Swanson MS, et al. Muscle chloride channel dysfunction in two mouse models of myotonic dystrophy. J Gen Physiol. 2007;129:79\u201394. https:\/\/doi.org\/10.1085\/jgp.200609635.","journal-title":"J Gen Physiol"},{"key":"804_CR18","doi-asserted-by":"publisher","first-page":"35","DOI":"10.1016\/S1097-2765(02)00563-4","volume":"10","author":"A Mankodi","year":"2002","unstructured":"Mankodi A, Takahashi MP, Jiang H, et al. Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy. Mol Cell. 2002;10:35\u201344. https:\/\/doi.org\/10.1016\/S1097-2765(02)00563-4.","journal-title":"Mol Cell"},{"key":"804_CR19","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1016\/S1097-2765(02)00572-5","volume":"10","author":"N Charlet-B","year":"2002","unstructured":"Charlet-B N, Savkur RS, Singh G, et al. Loss of the muscle-specific chloride channel in type\u00a01 myotonic dystrophy due to misregulated alternative splicing. Mol Cell. 2002;10:45\u201353. https:\/\/doi.org\/10.1016\/S1097-2765(02)00572-5.","journal-title":"Mol Cell"},{"key":"804_CR20","doi-asserted-by":"publisher","first-page":"762","DOI":"10.1002\/mus.880140811","volume":"14","author":"C Franke","year":"1991","unstructured":"Franke C, Iaizzo PA, Hatt H, et al. Altered Na+ channel activity and reduced Cl\u2212 conductance cause hyperexcitability in recessive generalized myotonia (Becker). Muscle Nerve. 1991;14:762\u201370. https:\/\/doi.org\/10.1002\/mus.880140811.","journal-title":"Muscle Nerve"},{"key":"804_CR21","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1016\/0960-8966(91)90042-Q","volume":"1","author":"PA Iaizzo","year":"1991","unstructured":"Iaizzo PA, Franke C, Hatt H, et al. Altered sodium channel behaviour causes myotonia in dominantly inherited myotonia congenita. Neuromuscul Disord. 1991;1:47\u201353. https:\/\/doi.org\/10.1016\/0960-8966(91)90042-Q.","journal-title":"Neuromuscul Disord"},{"key":"804_CR22","unstructured":"European Medicines Agency. Mexiletine (Namuscla) 2018. https:\/\/www.ema.europa.eu\/en\/medicines\/human\/EPAR\/namuscla. Accessed 21 April 2025."},{"key":"804_CR23","unstructured":"NHS England. Standard contract for diagnostic service for rare neuromuscular disorders 2013. https:\/\/www.england.nhs.uk\/wp-content\/uploads\/2018\/08\/Diagnostic-service-for-rare-neuromuscular-disorders-all-ages.pdf. Accessed 21 April 2025."},{"key":"804_CR24","first-page":"98","volume":"27","author":"F Lehmann-Horn","year":"2008","unstructured":"Lehmann-Horn F, Jurkat-Rott K, R\u00fcdel R, Centre UM. Diagnostics and therapy of muscle channelopathies\u2014guidelines of the Ulm Muscle Centre. Acta Myol. 2008;27:98\u2013113.","journal-title":"Acta Myol"},{"key":"804_CR25","doi-asserted-by":"publisher","first-page":"1441","DOI":"10.1212\/WNL.0b013e3181dc1a3a","volume":"74","author":"EL Logigian","year":"2010","unstructured":"Logigian EL, Martens WB, Moxley RT, et al. Mexiletine is an effective antimyotonia treatment in myotonic dystrophy type\u00a01. Neurology. 2010;74:1441\u20138. https:\/\/doi.org\/10.1212\/WNL.0b013e3181dc1a3a.","journal-title":"Neurology"},{"key":"804_CR26","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000011002","author":"C Heatwole","year":"2021","unstructured":"Heatwole C, Luebbe E, Rosero S, et al. Mexiletine in myotonic dystrophy type\u00a01. Neurology. 2021. https:\/\/doi.org\/10.1212\/WNL.0000000000011002.","journal-title":"Neurology"},{"key":"804_CR27","doi-asserted-by":"publisher","first-page":"58","DOI":"10.1016\/S0002-8703(76)80435-8","volume":"91","author":"RG Talbot","year":"1976","unstructured":"Talbot RG, Julian DG, Prescott LF. Long-term treatment of ventricular arrhythmias with oral mexiletine. Am Heart J. 1976;91:58\u201365. https:\/\/doi.org\/10.1016\/S0002-8703(76)80435-8.","journal-title":"Am Heart J"},{"key":"804_CR28","doi-asserted-by":"publisher","first-page":"288","DOI":"10.1093\/cvr\/13.5.288","volume":"13","author":"I Yamaguchi","year":"1979","unstructured":"Yamaguchi I, Singh BN, Mandel WJ. Electrophysiological actions of mexiletine on isolated rabbit atria and canine ventricular muscle and Purkinje fibres. Cardiovasc Res. 1979;13:288\u201396. https:\/\/doi.org\/10.1093\/cvr\/13.5.288.","journal-title":"Cardiovasc Res"},{"key":"804_CR29","first-page":"899","volume-title":"McGraw-Hill education","author":"L Laurence","year":"2005","unstructured":"Laurence L, Brunton BCK. The pharmacological basis of therapeutics. In: McGraw-Hill education. 14th ed. New York: Goodman and Gillman\u2019s; 2005. p. 899\u2013932.","edition":"14"},{"key":"804_CR30","doi-asserted-by":"publisher","first-page":"1944","DOI":"10.1016\/j.hrthm.2020.05.043","volume":"17","author":"R Vio","year":"2020","unstructured":"Vio R, Zorzi A, Bello L, et al. Evaluation of mexiletine effect on conduction delay and bradyarrhythmic complications in patients with myotonic dystrophy type\u00a01 over long-term follow-up. Heart Rhythm. 2020;17:1944\u201350. https:\/\/doi.org\/10.1016\/j.hrthm.2020.05.043.","journal-title":"Heart Rhythm"},{"key":"804_CR31","doi-asserted-by":"publisher","DOI":"10.1212\/CPJ.0000000000001073","author":"C Mousele","year":"2021","unstructured":"Mousele C, Matthews E, Pitceathly RDS, et al. Long-term safety and efficacy of mexiletine in myotonic dystrophy types 1 and 2. Neurol Clin Pract. 2021. https:\/\/doi.org\/10.1212\/CPJ.0000000000001073.","journal-title":"Neurol Clin Pract"},{"key":"804_CR32","doi-asserted-by":"publisher","first-page":"450","DOI":"10.1016\/j.acvd.2024.03.001","volume":"117","author":"K Wahbi","year":"2024","unstructured":"Wahbi K, Bassez G, Duchateau J, et al. Expert opinion on mexiletine treatment in adult patients with myotonic dystrophy. Arch Cardiovasc Dis. 2024;117:450\u20136. https:\/\/doi.org\/10.1016\/j.acvd.2024.03.001.","journal-title":"Arch Cardiovasc Dis"},{"key":"804_CR33","doi-asserted-by":"publisher","first-page":"1702","DOI":"10.1002\/acn3.51667","volume":"9","author":"S Yamada","year":"2022","unstructured":"Yamada S, Hashizume A, Hijikata Y, et al. Mexiletine in spinal and bulbar muscular atrophy: a randomized controlled trial. Ann Clin Transl Neurol. 2022;9:1702\u201314. https:\/\/doi.org\/10.1002\/acn3.51667.","journal-title":"Ann Clin Transl Neurol"},{"key":"804_CR34","doi-asserted-by":"publisher","first-page":"1474","DOI":"10.1212\/WNL.0000000000002507","volume":"86","author":"MD Weiss","year":"2016","unstructured":"Weiss MD, Macklin EA, Simmons Z, et al. A randomized trial of mexiletine in ALS: safety and effects on muscle cramps and progression. Neurology. 2016;86:1474\u201381. https:\/\/doi.org\/10.1212\/WNL.0000000000002507.","journal-title":"Neurology"},{"key":"804_CR35","doi-asserted-by":"publisher","first-page":"1594","DOI":"10.2337\/diacare.20.10.1594","volume":"20","author":"P Oskarsson","year":"1997","unstructured":"Oskarsson P, Ljunggren JG, Lins PE. Efficacy and safety of mexiletine in the treatment of painful diabetic neuropathy. The Mexiletine Study Group. Diabetes Care. 1997;20:1594\u20137. https:\/\/doi.org\/10.2337\/diacare.20.10.1594.","journal-title":"Diabetes Care"},{"key":"804_CR36","doi-asserted-by":"publisher","first-page":"627","DOI":"10.1016\/s0002-9149(80)80015-4","volume":"45","author":"JJ Heger","year":"1980","unstructured":"Heger JJ, Nattel S, Rinkenberger RL, Zipes DP. Mexiletine therapy in 15 patients with drug-resistant ventricular tachycardia. Am J Cardiol. 1980;45:627\u201332. https:\/\/doi.org\/10.1016\/s0002-9149(80)80015-4.","journal-title":"Am J Cardiol"},{"key":"804_CR37","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/j.1875-9114.1986.tb03442.x","volume":"6","author":"PE Fenster","year":"1986","unstructured":"Fenster PE, Comess KA. Pharmacology and clinical use of mexiletine. Pharmacotherapy. 1986;6:1\u20137. https:\/\/doi.org\/10.1002\/j.1875-9114.1986.tb03442.x.","journal-title":"Pharmacotherapy"},{"key":"804_CR38","doi-asserted-by":"publisher","first-page":"1531","DOI":"10.1001\/jamaneurol.2015.2338","volume":"72","author":"KJ Suetterlin","year":"2015","unstructured":"Suetterlin KJ, Bugiardini E, Kaski JP, et al. Long-term safety and efficacy of mexiletine for patients with skeletal muscle channelopathies. JAMA Neurol. 2015;72:1531. https:\/\/doi.org\/10.1001\/jamaneurol.2015.2338.","journal-title":"JAMA Neurol"},{"key":"804_CR39","doi-asserted-by":"publisher","DOI":"10.1002\/14651858.CD004762.pub2","author":"J Trip","year":"2006","unstructured":"Trip J, Drost GG, van Engelen BG, Faber CG. Drug treatment for myotonia. Cochrane Database Syst Rev. 2006. https:\/\/doi.org\/10.1002\/14651858.CD004762.pub2.","journal-title":"Cochrane Database Syst Rev"},{"key":"804_CR40","doi-asserted-by":"publisher","first-page":"136","DOI":"10.1002\/mus.25452","volume":"56","author":"M Skov","year":"2017","unstructured":"Skov M, de Paoli FV, Nielsen OB, Pedersen TH. The anti-convulsants lacosamide, lamotrigine, and rufinamide reduce myotonia in isolated human and rat skeletal muscle. Muscle Nerve. 2017;56:136\u201342. https:\/\/doi.org\/10.1002\/mus.25452.","journal-title":"Muscle Nerve"},{"key":"804_CR41","doi-asserted-by":"publisher","first-page":"203","DOI":"10.1254\/jphs.13116SC","volume":"123","author":"Y Nakatani","year":"2013","unstructured":"Nakatani Y, Masuko H, Amano T. The effect of lamotrigine on Nav1.4 voltage-gated sodium channels. J Pharmacol Sci. 2013;123:203\u20136. https:\/\/doi.org\/10.1254\/jphs.13116SC.","journal-title":"J Pharmacol Sci"},{"key":"804_CR42","doi-asserted-by":"publisher","first-page":"857","DOI":"10.1111\/j.1528-1157.1996.tb00038.x","volume":"37","author":"F Matsuo","year":"1996","unstructured":"Matsuo F, Gay P, Madsen J, et al. Lamotrigine high-dose tolerability and safety in patients with epilepsy: a double-blind, placebo-controlled, eleven-week study. Epilepsia. 1996;37:857\u201362. https:\/\/doi.org\/10.1111\/j.1528-1157.1996.tb00038.x.","journal-title":"Epilepsia"},{"key":"804_CR43","doi-asserted-by":"publisher","first-page":"1025","DOI":"10.1080\/14737175.2022.2155141","volume":"22","author":"N Zhang","year":"2022","unstructured":"Zhang N, Yang D, Niu C, et al. Comparison of the retention rate, safety, and efficacy of two different titration protocols for lamotrigine in newly diagnosed epilepsy patients. Expert Rev Neurother. 2022;22:1025\u20139. https:\/\/doi.org\/10.1080\/14737175.2022.2155141.","journal-title":"Expert Rev Neurother"},{"key":"804_CR44","doi-asserted-by":"publisher","first-page":"897","DOI":"10.2147\/TCRM.S131021","volume":"13","author":"N Brickel","year":"2017","unstructured":"Brickel N, Shaikh MHS, Kirkham A, et al. Collaboration in pharmacovigilance: lamotrigine and fatal severe cutaneous adverse reactions \u2013 a review of spontaneous reports. Ther Clin Risk Manag. 2017;13:897\u2013903. https:\/\/doi.org\/10.2147\/TCRM.S131021.","journal-title":"Ther Clin Risk Manag"},{"key":"804_CR45","doi-asserted-by":"publisher","first-page":"2371","DOI":"10.1111\/epi.17339","volume":"63","author":"J Christensen","year":"2022","unstructured":"Christensen J, Trabjerg BB, Dreier JW. Cardiac morbidity and mortality associated with the use of lamotrigine. Epilepsia. 2022;63:2371\u201380. https:\/\/doi.org\/10.1111\/epi.17339.","journal-title":"Epilepsia"},{"key":"804_CR46","doi-asserted-by":"publisher","first-page":"2295","DOI":"10.1093\/brain\/awx192","volume":"140","author":"G Andersen","year":"2017","unstructured":"Andersen G, Hedermann G, Witting N, et al. The antimyotonic effect of lamotrigine in non-dystrophic myotonias: a double-blind randomized study. Brain. 2017;140:2295\u2013305. https:\/\/doi.org\/10.1093\/brain\/awx192.","journal-title":"Brain"},{"key":"804_CR47","doi-asserted-by":"publisher","first-page":"1004","DOI":"10.1016\/S1474-4422(24)00320-X","volume":"23","author":"V Vivekanandam","year":"2024","unstructured":"Vivekanandam V, Skorupinska I, Jayaseelan DL, et al. Mexiletine versus lamotrigine in non-dystrophic myotonias: a randomised, double-blind, head-to-head, crossover, non-inferiority, phase\u00a03 trial. Lancet Neurol. 2024;23:1004\u201312. https:\/\/doi.org\/10.1016\/S1474-4422(24)00320-X.","journal-title":"Lancet Neurol"},{"key":"804_CR48","doi-asserted-by":"publisher","first-page":"610","DOI":"10.1016\/j.nmd.2005.07.002","volume":"15","author":"E Hammar\u00e9n","year":"2005","unstructured":"Hammar\u00e9n E, Kjellby-Wendt G, Lindberg C. Quantification of mobility impairment and self-assessment of stiffness in patients with myotonia congenita by the physiotherapist. Neuromuscul Disord. 2005;15:610\u20137. https:\/\/doi.org\/10.1016\/j.nmd.2005.07.002.","journal-title":"Neuromuscul Disord"},{"key":"804_CR49","doi-asserted-by":"publisher","first-page":"711","DOI":"10.1177\/1352458517690824","volume":"23","author":"P Feys","year":"2017","unstructured":"Feys P, Lamers I, Francis G, et al. The nine-hole peg test as a manual dexterity performance measure for multiple sclerosis. Mult Scler J. 2017;23:711\u201320. https:\/\/doi.org\/10.1177\/1352458517690824.","journal-title":"Mult Scler J"},{"key":"804_CR50","doi-asserted-by":"publisher","first-page":"20220046","DOI":"10.2490\/prm.20220046","volume":"7","author":"N Watanabe","year":"2022","unstructured":"Watanabe N, Otaka Y, Kumagai M, et al. Reliability of the modified nine hole peg test in healthy adults and individuals with hemiparetic stroke. Prog Rehabil Med. 2022;7:20220046. https:\/\/doi.org\/10.2490\/prm.20220046.","journal-title":"Prog Rehabil Med"},{"key":"804_CR51","doi-asserted-by":"publisher","first-page":"336","DOI":"10.1212\/wnl.56.3.336","volume":"56","author":"J Mathieu","year":"2001","unstructured":"Mathieu J, Boivin H, Meunier D, et al. Assessment of a disease-specific muscular impairment rating scale in myotonic dystrophy. Neurology. 2001;56:336\u201340. https:\/\/doi.org\/10.1212\/wnl.56.3.336.","journal-title":"Neurology"},{"key":"804_CR52","doi-asserted-by":"publisher","DOI":"10.1085\/jgp.201912526","author":"X Wang","year":"2020","unstructured":"Wang X, Burke SRA, Talmadge RJ, et al. Depressed neuromuscular transmission causes weakness in mice lacking BK potassium channels. J Gen Physiol. 2020. https:\/\/doi.org\/10.1085\/jgp.201912526.","journal-title":"J Gen Physiol"},{"key":"804_CR53","doi-asserted-by":"publisher","first-page":"1956","DOI":"10.1038\/s41467-023-37619-1","volume":"14","author":"N Hu","year":"2023","unstructured":"Hu N, Kim E, Antoury L, Wheeler TM. Correction of Clcn1 alternative splicing reverses muscle fiber type transition in mice with myotonic dystrophy. Nat Commun. 2023;14:1956. https:\/\/doi.org\/10.1038\/s41467-023-37619-1.","journal-title":"Nat Commun"}],"container-title":["Neurology and Therapy"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s40120-025-00804-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s40120-025-00804-z\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s40120-025-00804-z.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,20]],"date-time":"2025-09-20T11:22:26Z","timestamp":1758367346000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s40120-025-00804-z"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,8,2]]},"references-count":53,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2025,10]]}},"alternative-id":["804"],"URL":"https:\/\/doi.org\/10.1007\/s40120-025-00804-z","relation":{},"ISSN":["2193-8253","2193-6536"],"issn-type":[{"value":"2193-8253","type":"print"},{"value":"2193-6536","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,8,2]]},"assertion":[{"value":"16 May 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"21 July 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 August 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Barbara Risi, Nesaiba Ait Allali, Stefano Cotti Piccinelli, Filomena Caria, Simona Damioli, Beatrice Labella, Enrica Bertella, Giorgia Giovanelli, Francesca Garofali, Giuseppina Margollicci, Roberto Carugati, Lucia Ferullo, Emanuele Olivieri, Loris Poli, Alessandro Padovani, Massimiliano Filosto have nothing to disclose.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of Interest"}},{"value":"This study was conducted in accordance with the principles of the Declaration of Helsinki of 1964, and its later amendments. Approval was granted by the local Ethics Committee Comitato Etico Territoriale Lombardia 6 (22 November 2024, No. 0063419\/24). All subjects provided informed consent to participate in the study.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical Approval"}}]}}