{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,13]],"date-time":"2025-05-13T15:41:44Z","timestamp":1747150904064,"version":"3.40.5"},"reference-count":20,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2021,4,20]],"date-time":"2021-04-20T00:00:00Z","timestamp":1618876800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Muscle and Nerve"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Introduction\/Aims<\/jats:title><jats:p>Paramyotonia congenita (PMC) is a skeletal muscle sodium channelopathy characterized by paradoxical myotonia, cold sensitivity, and exercise\/cold\u2010induced paralysis. Treatment with sodium\u2010channel\u2013blocking antiarrhythmic agents may expose patients to a risk of arrhythmia or may be poorly tolerated or ineffective. In this study we explored the effectiveness of non\u2010antiarrhythmic sodium\u2010channel blockers in two patients with PMC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Earlier treatment with mexiletine was discontinued for gastrointestinal side effects in one of the patients and lack of clinical benefit in the other. One patient received lacosamide, ranolazine, and buprenorphine, and the other was given buprenorphine only. Drug efficacy was assessed by clinical scores, timed tests, and by long and short exercise tests.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In both patients, buprenorphine improved pain scores by at least 50%, stiffness and weakness levels, and handgrip\/eyelid\u2010opening times. The fall in compound muscle action potential (CMAP) during short exercise normalized in both patients at baseline, and improved after cooling. During long exercise, one patient showed an earlier recovery of CMAP, and the other patient had a less severe decrease (&lt;60%). With buprenorphine, the fall in CMAP induced by cooling normalized in one patient (from \u221272% to \u22124%) and improved (from \u221249% to \u221237%) in the other patient.<\/jats:p><\/jats:sec><jats:sec><jats:title>Discussion<\/jats:title><jats:p>Buprenorphine showed promising results for the treatment of exercise\u2010induced paralysis and cold intolerance in the two patients assessed. The exercise test may be useful for quantitative assessment of treatment response. Further studies on a larger number of patients, under carefully controlled conditions, should be considered to address the effectiveness and long\u2010term tolerability of this therapeutic option.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mus.27249","type":"journal-article","created":{"date-parts":[[2021,4,9]],"date-time":"2021-04-09T14:16:06Z","timestamp":1617977766000},"page":"95-99","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Buprenorphine may be effective for treatment of paramyotonia congenita"],"prefix":"10.1002","volume":"64","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8749-3706","authenticated-orcid":false,"given":"Sabrina","family":"Ravaglia","sequence":"first","affiliation":[{"name":"IRCCS Mondino Foundation Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lorenzo","family":"Maggi","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Istituto Neurologico Carlo Besta Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Antonio","family":"Zito","sequence":"additional","affiliation":[{"name":"IRCCS Mondino Foundation Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sebastiano","family":"Arceri","sequence":"additional","affiliation":[{"name":"IRCCS Mondino Foundation Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pietro","family":"Gallotti","sequence":"additional","affiliation":[{"name":"Istituto Clinico \"Beato Matteo,\" Hospital Group San Donato Vigevano Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Concetta","family":"Altamura","sequence":"additional","affiliation":[{"name":"Sezione di Farmacologia, Dipartimento di Scienze Biomediche e Oncologia Umana, Scuola di Medicina Universit\u00e0 degli Studi di Bari Aldo Moro Bari Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean Francois","family":"Desaphy","sequence":"additional","affiliation":[{"name":"Sezione di Farmacologia, Dipartimento di Scienze Biomediche e Oncologia Umana, Scuola di Medicina Universit\u00e0 degli Studi di Bari Aldo Moro Bari Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pia","family":"Bernasconi","sequence":"additional","affiliation":[{"name":"Fondazione IRCCS Istituto Neurologico Carlo Besta Milan Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Enrico","family":"Alfonsi","sequence":"additional","affiliation":[{"name":"IRCCS Mondino Foundation Pavia Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2021,4,20]]},"reference":[{"key":"e_1_2_10_2_1","first-page":"1598","article-title":"Muscle channelopathies: the nondystrophic myotonias and periodic paralyses","volume":"19","author":"Statland JM","year":"2013","journal-title":"Continuum (Minneap Minn)."},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.25956"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23683"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2012.12607"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/jama.2018.18020"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2015.2338"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/ejhpharm-2015-000839"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24331"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.4161\/chan.5.2.14851"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.26372"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awx192"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/AAP.0000000000000596"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1007\/978-2-8178-0855-0_15"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.4172\/2167-1052.1000145"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/ALN.0b013e3182557917"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20241"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.20905"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20856"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.0000000000004229"},{"issue":"15","key":"e_1_2_10_21_1","doi-asserted-by":"crossref","first-page":"P5.443","DOI":"10.1212\/WNL.90.15_supplement.P5.443","article-title":"Investigation of ranolazine as an anti\u2010myotonia treatment in myotonic dystrophy type 1","volume":"90","author":"Lawless M","year":"2018","journal-title":"Neurology"}],"container-title":["Muscle &amp; 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