Page last updated: 2024-10-31

mexiletine and Myotonia

mexiletine has been researched along with Myotonia in 28 studies

Mexiletine: Antiarrhythmic agent pharmacologically similar to LIDOCAINE. It may have some anticonvulsant properties.
mexiletine : An aromatic ether which is 2,6-dimethylphenyl ether of 2-aminopropan-1-ol.

Myotonia: Prolonged failure of muscle relaxation after contraction. This may occur after voluntary contractions, muscle percussion, or electrical stimulation of the muscle. Myotonia is a characteristic feature of MYOTONIC DISORDERS.

Research Excerpts

ExcerptRelevanceReference
"To determine the effects of mexiletine for symptoms and signs of myotonia in patients with NDMs."9.16Mexiletine for symptoms and signs of myotonia in nondystrophic myotonia: a randomized controlled trial. ( Barohn, RJ; Bundy, BN; Ciafaloni, E; Griggs, RC; Hanna, MG; Herbelin, L; Matthews, E; Meola, G; Rayan, DR; Salajegheh, MK; Sansone, VA; Statland, JM; Trivedi, JR; Venance, SL; Wang, Y, 2012)
"To determine if mexiletine is safe and effective in reducing myotonia in myotonic dystrophy type 1 (DM1)."9.14Mexiletine is an effective antimyotonia treatment in myotonic dystrophy type 1. ( Annis, CL; Barbieri, CA; Dilek, N; Logigian, EL; Martens, WB; McDermott, MP; Moxley, RT; Pearson, AT; Thornton, CA; Wiegner, AW, 2010)
"Although mexiletine effectively treats myotonia, supply disruptions affected Europe between 2008-2018."8.31Impact of restricted access to, and low awareness of, mexiletine on people with myotonia: a real-world European survey. ( Díaz-Manera, J; Foerster, D; Kole, A; Schey, C; Urtizberea, JA; von Gallwitz, P; Whiting, A; Zozulya-Weidenfeller, A, 2023)
"Consensus was achieved for key questions including the average long-term dosage of mexiletine (NaMuscla) in practice, the criteria for eligibility of myotonia treatment, the clinical importance of QoL outcomes in MYOMEX, the higher proportion of patients with increased QoL, and the reduction in the need for mental health resources for patients receiving mexiletine (NaMuscla)."8.02Improving the understanding of how patients with non-dystrophic myotonia are selected for myotonia treatment with mexiletine (NaMuscla): outcomes of treatment impact using a European Delphi panel. ( Chapman, AM; Ellis, C; Lee, H; Omar, A; Schneider-Gold, C; Schurer, M; Sonecha, S; Weidenfeller, AZ; Weijers, L, 2021)
"Mexiletine is a long-known drug used for the treatment of arrhythmias and repurposed in the 1980s for patients with nondystrophic myotonia (NDM)."8.02Cost-Based Price Calculation of Mexiletine for Nondystrophic Myotonia. ( de Visser, SJ; Hollak, CEM; Postema, PG; Stunnenberg, BC; Timmers, L; van den Berg, S; van der Ree, MH; van der Wel, V, 2021)
"To investigate the effectiveness of mexiletine in nondystrophic myotonia using an aggregated N-of-1 trials design and compare results between this innovative design and a previously conducted RCT."7.88Effect of Mexiletine on Muscle Stiffness in Patients With Nondystrophic Myotonia Evaluated Using Aggregated N-of-1 Trials. ( Drost, G; Griggs, RC; Groenewoud, HM; Matthews, E; Raaphorst, J; Saris, CGJ; Schouwenberg, BJ; Statland, JM; Stegeman, DF; Stunnenberg, BC; Timmermans, J; Trivedi, J; van der Wilt, GJ; van Engelen, BGM; Woertman, W, 2018)
"1 The antiarrhythmic drug mexiletine (Mex) is also used against myotonia."7.70Increased hindrance on the chiral carbon atom of mexiletine enhances the block of rat skeletal muscle Na+ channels in a model of myotonia induced by ATX. ( Conte Camerino, D; De Luca, A; Desaphy, JF; Franchini, C; Lentini, G; Tortorella, V, 1999)
"Mexiletine treatment started at 200 mg/day and was up-titrated by 200 mg increment each three days to reach a maximum dose of 600 mg/day for total treatment duration of 18 days for each cross-over period."7.01Efficacy and safety of mexiletine in non-dystrophic myotonias: A randomised, double-blind, placebo-controlled, cross-over study. ( Behin, A; Bouhour, F; Fontaine, B; Fournier, E; Franques, J; Hézode, M; Lacomblez, L; Magot, A; Nadaj-Pakleza, A; Payan, C; Péréon, Y; Sacconi, S; Vicart, S; Zahr, N, 2021)
"Considering the large number of rare diseases with a lack of treatment evidence, more experience with innovative trial designs is needed."6.80Combined N-of-1 trials to investigate mexiletine in non-dystrophic myotonia using a Bayesian approach; study rationale and protocol. ( Drost, G; Griggs, RC; Groenewoud, HM; Raaphorst, J; Saris, CG; Schouwenberg, BJ; Statland, JM; Stegeman, DF; Stunnenberg, BC; Timmermans, J; van der Wilt, GJ; van Engelen, BG; Woertman, W, 2015)
"Mexiletine has beneficial effects on myotonia."5.30Effect of mexiletine on sea anemone toxin-induced non-inactivating sodium channels of rat skeletal muscle: a model of sodium channel myotonia. ( Camerino, DC; De Luca, A; Desaphy, JF; Tortorella, V, 1999)
"To determine the effects of mexiletine for symptoms and signs of myotonia in patients with NDMs."5.16Mexiletine for symptoms and signs of myotonia in nondystrophic myotonia: a randomized controlled trial. ( Barohn, RJ; Bundy, BN; Ciafaloni, E; Griggs, RC; Hanna, MG; Herbelin, L; Matthews, E; Meola, G; Rayan, DR; Salajegheh, MK; Sansone, VA; Statland, JM; Trivedi, JR; Venance, SL; Wang, Y, 2012)
"To determine if mexiletine is safe and effective in reducing myotonia in myotonic dystrophy type 1 (DM1)."5.14Mexiletine is an effective antimyotonia treatment in myotonic dystrophy type 1. ( Annis, CL; Barbieri, CA; Dilek, N; Logigian, EL; Martens, WB; McDermott, MP; Moxley, RT; Pearson, AT; Thornton, CA; Wiegner, AW, 2010)
"Although mexiletine effectively treats myotonia, supply disruptions affected Europe between 2008-2018."4.31Impact of restricted access to, and low awareness of, mexiletine on people with myotonia: a real-world European survey. ( Díaz-Manera, J; Foerster, D; Kole, A; Schey, C; Urtizberea, JA; von Gallwitz, P; Whiting, A; Zozulya-Weidenfeller, A, 2023)
"Consensus was achieved for key questions including the average long-term dosage of mexiletine (NaMuscla) in practice, the criteria for eligibility of myotonia treatment, the clinical importance of QoL outcomes in MYOMEX, the higher proportion of patients with increased QoL, and the reduction in the need for mental health resources for patients receiving mexiletine (NaMuscla)."4.02Improving the understanding of how patients with non-dystrophic myotonia are selected for myotonia treatment with mexiletine (NaMuscla): outcomes of treatment impact using a European Delphi panel. ( Chapman, AM; Ellis, C; Lee, H; Omar, A; Schneider-Gold, C; Schurer, M; Sonecha, S; Weidenfeller, AZ; Weijers, L, 2021)
"To investigate the effectiveness of mexiletine in nondystrophic myotonia using an aggregated N-of-1 trials design and compare results between this innovative design and a previously conducted RCT."3.88Effect of Mexiletine on Muscle Stiffness in Patients With Nondystrophic Myotonia Evaluated Using Aggregated N-of-1 Trials. ( Drost, G; Griggs, RC; Groenewoud, HM; Matthews, E; Raaphorst, J; Saris, CGJ; Schouwenberg, BJ; Statland, JM; Stegeman, DF; Stunnenberg, BC; Timmermans, J; Trivedi, J; van der Wilt, GJ; van Engelen, BGM; Woertman, W, 2018)
"Although the sodium channel blocker, mexiletine, is the first choice drug in myotonia, some myotonic patients remain unsatisfied due to contraindications, lack of tolerability, or incomplete response."3.85Increased sodium channel use-dependent inhibition by a new potent analogue of tocainide greatly enhances in vivo antimyotonic activity. ( Carbonara, R; Carratù, MR; Conte Camerino, D; Corbo, F; De Bellis, M; De Luca, A; Desaphy, JF; Farinato, A; Franchini, C; Massari, A; Muraglia, M; Pierno, S; Roussel, J, 2017)
"Flecainide, a class Ic antiarrhythmic drug, has been anecdotally reported to improve myotonia, but little is known about its kinetics on human skeletal muscle sodium channels applicable in vivo."3.73Class Ic antiarrhythmics block human skeletal muscle Na channel during myotonia-like stimulation. ( Aoike, F; Sakoda, S; Takahashi, MP, 2006)
"1 The antiarrhythmic drug mexiletine (Mex) is also used against myotonia."3.70Increased hindrance on the chiral carbon atom of mexiletine enhances the block of rat skeletal muscle Na+ channels in a model of myotonia induced by ATX. ( Conte Camerino, D; De Luca, A; Desaphy, JF; Franchini, C; Lentini, G; Tortorella, V, 1999)
"To search for use-dependent sodium channel blockers to selectively solve skeletal muscle hyperexcitability in hereditary myotonias, mexiletine (MEX; compound I) and its newly synthetized analogs, 2-(4-chloro-2-methylphenoxy)-benzenethanamine (compound II) and (-)-S-3-(2,6-dimethylphenoxy)-2-methylpropanamine (compound III), were tested on intercostal muscle fibers from the myotonic ADR mouse through use of the standard current-clamp microelectrode technique."3.69Evaluation of the antimyotonic activity of mexiletine and some new analogs on sodium currents of single muscle fibers and on the abnormal excitability of the myotonic ADR mouse. ( Camerino, DC; De Luca, A; Duranti, A; Franchini, C; Jockusch, H; Lentini, G; Natuzzi, F; Pierno, S; Tortorella, V, 1997)
"Mexiletine treatment started at 200 mg/day and was up-titrated by 200 mg increment each three days to reach a maximum dose of 600 mg/day for total treatment duration of 18 days for each cross-over period."3.01Efficacy and safety of mexiletine in non-dystrophic myotonias: A randomised, double-blind, placebo-controlled, cross-over study. ( Behin, A; Bouhour, F; Fontaine, B; Fournier, E; Franques, J; Hézode, M; Lacomblez, L; Magot, A; Nadaj-Pakleza, A; Payan, C; Péréon, Y; Sacconi, S; Vicart, S; Zahr, N, 2021)
"Myotonia is a skeletal muscle condition characterized by the over-excitability of the sarcolemma, resulting in delayed relaxation after contraction and muscle stiffness."3.01Blockers of Skeletal Muscle Na ( Boccanegra, B; Cerchiara, AG; De Bellis, M; De Luca, A; Imbrici, P, 2023)
"Considering the large number of rare diseases with a lack of treatment evidence, more experience with innovative trial designs is needed."2.80Combined N-of-1 trials to investigate mexiletine in non-dystrophic myotonia using a Bayesian approach; study rationale and protocol. ( Drost, G; Griggs, RC; Groenewoud, HM; Raaphorst, J; Saris, CG; Schouwenberg, BJ; Statland, JM; Stegeman, DF; Stunnenberg, BC; Timmermans, J; van der Wilt, GJ; van Engelen, BG; Woertman, W, 2015)
"Non-dystrophic myotonias are rare diseases caused by mutations in skeletal muscle chloride and sodium ion channels with considerable phenotypic overlap between diseases."1.39Non-dystrophic myotonia: prospective study of objective and patient reported outcomes. ( Amato, A; Barohn, RJ; Bundy, B; Cannon, S; Cleland, J; Fialho, D; Gorham, N; Griggs, RC; Hanna, MG; Herbelin, L; Matthews, E; Rayan, DR; Salajegheh, M; Statland, J; Trivedi, JR; Venance, SL; Wang, Y, 2013)
"He had myotonia congenita."1.30Unusual causes of stiffness in two hockey players. ( Burnham, R, 1997)
"Mexiletine has beneficial effects on myotonia."1.30Effect of mexiletine on sea anemone toxin-induced non-inactivating sodium channels of rat skeletal muscle: a model of sodium channel myotonia. ( Camerino, DC; De Luca, A; Desaphy, JF; Tortorella, V, 1999)

Research

Studies (28)

TimeframeStudies, this research(%)All Research%
pre-19901 (3.57)18.7374
1990's8 (28.57)18.2507
2000's4 (14.29)29.6817
2010's9 (32.14)24.3611
2020's6 (21.43)2.80

Authors

AuthorsStudies
Vicart, S1
Franques, J1
Bouhour, F1
Magot, A1
Péréon, Y1
Sacconi, S1
Nadaj-Pakleza, A1
Behin, A1
Zahr, N1
Hézode, M1
Fournier, E1
Payan, C1
Lacomblez, L1
Fontaine, B2
Chapman, AM1
Schurer, M1
Weijers, L1
Omar, A1
Lee, H1
Weidenfeller, AZ1
Ellis, C1
Sonecha, S1
Schneider-Gold, C1
De Bellis, M3
Boccanegra, B1
Cerchiara, AG1
Imbrici, P1
De Luca, A7
Díaz-Manera, J1
Urtizberea, JA1
Schey, C1
Kole, A1
von Gallwitz, P1
Whiting, A1
Foerster, D1
Zozulya-Weidenfeller, A1
Salguero-Bodes, R1
Ruiz-Curiel, A1
Palomino-Doza, J1
Valverde-Gómez, M1
Domínguez-González, C1
Arribas-Ynsaurriaga, F1
van den Berg, S1
van der Wel, V1
de Visser, SJ1
Stunnenberg, BC3
Timmers, L1
van der Ree, MH1
Postema, PG1
Hollak, CEM1
Raaphorst, J2
Groenewoud, HM2
Statland, JM3
Griggs, RC4
Woertman, W2
Stegeman, DF2
Timmermans, J2
Trivedi, J1
Matthews, E3
Saris, CGJ1
Schouwenberg, BJ2
Drost, G2
van Engelen, BGM1
van der Wilt, GJ2
Trivedi, JR2
Bundy, B1
Statland, J1
Salajegheh, M1
Rayan, DR2
Venance, SL2
Wang, Y2
Fialho, D1
Cleland, J1
Gorham, N1
Herbelin, L2
Cannon, S1
Amato, A1
Hanna, MG2
Barohn, RJ2
Saris, CG1
van Engelen, BG1
Carbonara, R1
Roussel, J1
Farinato, A1
Massari, A1
Pierno, S3
Muraglia, M1
Corbo, F1
Franchini, C5
Carratù, MR1
Conte Camerino, D4
Desaphy, JF3
Carocci, A1
Catalano, A1
Bruno, C1
Lentini, G4
Logigian, EL1
Martens, WB1
Moxley, RT3
McDermott, MP1
Dilek, N1
Wiegner, AW1
Pearson, AT1
Barbieri, CA1
Annis, CL1
Thornton, CA1
Zhao, J1
Duprè, N1
Puymirat, J1
Chahine, M1
Hoffman, EP1
Kaminski, HJ1
Bundy, BN1
Sansone, VA1
Salajegheh, MK1
Ciafaloni, E1
Meola, G1
Aoike, F1
Takahashi, MP2
Sakoda, S1
Courtney, KR1
Ricker, K1
Heine, R1
Lehmann-Horn, F2
Burnham, R1
Natuzzi, F1
Duranti, A2
Tortorella, V4
Jockusch, H1
Camerino, DC2
Fleischhauer, R1
Mitrovic, N1
Deymeer, F1
Lerche, H1
Loiodice, F1
Nemoto, H1
Kurihara, T1
Cannon, SC1
Kwieciński, H1
Ryniewicz, B1
Ostrzycki, A1

Clinical Trials (3)

Trial Overview

TrialPhaseEnrollmentStudy TypeStart DateStatus
Combining N-of-1 Trials to Estimate Population Clinical and Cost-effectiveness of Drugs Using Bayesian Hierarchical Modeling. The Case of Mexiletine for Patients With Non- Dystrophic Myotonia.[NCT02045667]Phase 230 participants (Actual)Interventional2014-01-31Completed
A Randomized, Placebo Controlled, Clinical Efficacy Trial of Mexiletine for Myotonic Dystrophy Type-1 (DM1)[NCT01406873]Phase 242 participants (Actual)Interventional2011-06-30Completed
Phase II Therapeutic Trial of Mexiletine in Non-Dystrophic Myotonia[NCT00832000]Phase 259 participants (Actual)Interventional2008-12-31Completed
[information is prepared from clinicaltrials.gov, extracted Sep-2024]

Trial Outcomes

Mean Change From Baseline in Ambulation Using the 6 Minute Walk Distance

During this assessment, participants were asked to walk as far as they could back and forth on a fixed 20 meter route for 6 minutes. The total distance walked during the 6 minutes was recorded in meters. Change from baseline was defined as the difference between the average 6 minute walk distance at baseline and the average 6 minute walk distance at 6 months. (NCT01406873)
Timeframe: Baseline to 6 months

InterventionMeters (Mean)
Mexiletine17.44
Placebo7.25

Mean Change From Baseline in Manual Muscle Testing (MMT) Score

Manual muscle testing was performed on 26 muscle groups (shoulder abductors, elbow flexors, wrist flexors, wrist extensors, hip flexors, knee extensors, hip extensors, knee flexors, hip abductors, elbow extensors, ankle dorsiflexors, and plantar flexors on the right and left plus neck extensor and neck flexors). The muscles were tested in various positions including sitting, supine, prone, and side lying and each graded on a modification of the Medical Research Council (MRC) scale of 0 to 5 (5 representing normal strength). Average MMT score is derived by averaging the individual MMT scores across the 26 individual muscles. (NCT01406873)
Timeframe: Baseline to 6 months

InterventionUnits on a scale (Mean)
Mexiletine0.05
Placebo-0.06

Mean Change From Baseline in Quantitative Measure of Hand Grip Myotonia

Relaxation time of the long finger flexor muscles of the right hand after a maximum voluntary isometric contraction performed in a standardized fixed position of the right arm elbow/wrist/hand. Relaxation time for this measurement is defined as the time to relax from 90% to 5% of the maximum isometric force of contraction of the hand (the first of 6 serial contractions averaged over two consecutive trials performed 10 minutes apart). (NCT01406873)
Timeframe: Baseline to 6 months

InterventionSeconds (Mean)
Mexiletine-1.01
Placebo0.43

Mean Change From Baseline in Patient-Reported Disease Burden and Quality of Life

"The Myotonic Dystrophy Health Index (MDHI) is a validated disease-specific measure of patient-reported disease burden. The MDHI total score is a weighted average derived from 17 subscales. MDHI total scores range form 0-100 with 0 representing no patient-reported disease burden and 100 representing the most severe patient-reported disease burden.~The Individualized Neuromuscular Quality of Life Questionnaire (INQoL) is a measure of quality of life in neuromuscular disease. The INQoL summary score is a weighted average made up of 5 sub-domains. Scores range from 0-100, and can be interpreted as the percent of maximal detrimental impact on quality of life with higher scores indicating more detrimental impact.~The 36-Item Short Form Survey (SF-36) is a generic measure of quality of life across 8 domains. Two summary metrics are produced from the 8 domains, ranging from 0-100% with lower scores representing worse levels of functioning." (NCT01406873)
Timeframe: Baseline to 6 months

,
InterventionUnits on a scale (Mean)
INQoLMDHISF-36 Physical Component SummarySF-36 Mental Component Summary
Mexiletine5.110.01-1.73-0.62
Placebo2.33-1.10-1.35-0.79

Mean Change From Baseline in PR, QRS, and QTc Intervals, and Average Minimum Heart Rate (HR) Via Electrocardiogram (ECG) Monitoring

PR, QRS, and QTc intervals as well as average minimum heart rate (HR) were obtained through standard 12 lead electrocardiograms (ECGs). Values were computer generated and verified by the study investigator and study cardiologist. (NCT01406873)
Timeframe: Baseline to 6 Months

,
InterventionMilliseconds (Mean)
PR IntervalQRS IntervalQTc IntervalAverage Minimum Heart Rate
Mexiletine11.350.70-4.400.65
Placebo8.891.00-1.55-0.25

Percentage of Participants That Had a Dose Reduction or a Study Drug Withdrawal or Suspension Over 6 Months

Adverse events were monitored at the three in-person evaluations (Months 0, 3, and 6), at telephone evaluations every 2 weeks, and via patient-completed side effect diaries. The study investigators and safety monitoring committee reviewed adverse events and made decisions regarding drug withdrawals, suspensions, and dose reductions as needed. (NCT01406873)
Timeframe: 6 months

,
InterventionParticipants (Count of Participants)
Study Drug WithdrawalStudy Drug Dose ReductionStudy Drug Temporary Suspension
Mexiletine211
Placebo000

Clinical Eye Closure Myotonia Evaluation (Seconds)

Time to open the eyes after forced eye closure as measured on a stopwatch. (NCT00832000)
Timeframe: The end of period 1 (week 4) and the end of period 2 (week 9)

InterventionSeconds (Mean)
Mexiletine0.161
Placebo0.474

Clinical Hand Grip Myotonia Evaluation (Seconds)

The time to open the fist after a forced handgrip as measured on a stopwatch. (NCT00832000)
Timeframe: The end of period 1 (week 4) and the end of period 2 (week 9)

InterventionSeconds (Mean)
Mexiletine0.164
Placebo0.494

Compound Motor Action Potentials After Long Exercise Test

Compound muscle action potential (CMAP) after long periods of exercise as a percentage of baseline. (NCT00832000)
Timeframe: The end of period 1 (week 4) and period 2 (week 9)

Interventionpercentage of baseline CMAP amplitude (Mean)
Mexiletine81.8
Placebo80.1

Compound Motor Action Potentials After Short Exercise Test

The maximal post-exercise compound muscle action potential (CMAP) after short periods of exercise as a percent of the baseline measurement. (NCT00832000)
Timeframe: The end of period 1 (week 4) and period 2 (week 9)

Interventionpercentage of baseline CMAP amplitude (Mean)
Mexiletine83.1
Placebo78.6

Graded Myotonia by Needle Electromyography - Right Abductor Digiti Minimi

Measured the amount of myotonia present on needle exam by assigning a number 1-3, with 1 being minimal amount of myotonia on needle stick and 3 being maximal amount of myotonia present on needle stick. (NCT00832000)
Timeframe: The end of period 1 (week 4) and period 2 (week 9)

Interventionunits on a scale (Mean)
Mexiletine2.05
Placebo2.62

Graded Myotonia by Needle Electromyography - Right Tibialis Anterior

Measured the amount of myotonia present on needle exam by assigning a number 1-3, with 1 being minimal amount of myotonia on needle stick and 3 being maximal amount of myotonia present on needle stick. (NCT00832000)
Timeframe: The end of period 1 (week 4) and period 2 (week 9)

Interventionunits on a scale (Mean)
Mexiletine2.07
Placebo2.54

Individualized Neuromuscular Quality of Life Scale - Summary Score

Quality of life scale for patinets with neuromuscular disorders. The INQoL summary score is a weighted average made up of 5 subdomains (activities, social relationships, independence, emotions, and body image) which document the impact of a disease on a patients' quality of life. Scores range from 0-100, and can be interpreted as the percent of maximal detrimental impact on quality of life. A higher score indicates more detrimental impact. (NCT00832000)
Timeframe: The end of period 1 (week 4) and period 2 (week 9)

Interventionunits on a scale (Mean)
Mexiletine14.0
Placebo16.7

Patient Reported Pain on the IVR

Pain measured on a 1-9 scale, 1 being minimal, 9 the worst ever experienced. 0=no symptom reported. For analysis the average severity of pain for each participant was calculated from daily calls made in weeks 3-4 of each period. (NCT00832000)
Timeframe: Weeeks 3-4 of each period

Interventionunits on a scale (Mean)
Mexiletine1.54
Placebo3.17

Patient Reported Tiredness on the IVR

Tiredness measured on a 1-9 scale, 1 being minimal, 9 the worst ever experienced. 0=no symptom reported. For analysis the average severity of tiredness for each participant was calculated from daily calls made in weeks 3-4 of each period. (NCT00832000)
Timeframe: Weeks 3-4 of each period

Interventionunits on a scale (Mean)
Mexiletine2.90
Placebo3.82

Patient Reported Weakness on the IVR

Weakness measured on a 1-9 scale, 1 being minimal, 9 the worst ever experienced. 0=no symptom reported. For analysis the average severity of weakness for each participant was calculated from daily calls made in weeks 3-4 of each period. (NCT00832000)
Timeframe: Weeks 3-4 of each period

Interventionunits on a scale (Mean)
Mexiletine1.96
Placebo3.22

Patient-reported Stiffness on the IVR

Stiffness measured on a 1-9 scale, 1 being minimal, 9 the worst ever experienced. 0=no symptom reported. For analysis the average severity of stiffness for each participant was calculated from daily calls made in weeks 3-4 of each period. (NCT00832000)
Timeframe: Weeks 3-4 of each period

Interventionunits on a scale (Mean)
Mexiletine - Period 12.53
Placebo - Period 14.21
Mexiletine - Period 21.60
Placebo - Period 25.27

Quantitative Measure of Hand Grip Myotonia (Seconds)

Maximum voluntary contractions following forced right hand grip were recorded and the time to relax from 90% to 5% of average maximal force was determined using automated analysis software. (NCT00832000)
Timeframe: The end of period 1 (week 4) and period 2 (week 9)

Interventionseconds (Mean)
Mexiletine0.321
Placebo0.429

Short Form 36 - Mental Composite Score

The SF-36 is a standard quality of life instrument. The mental composite score represents the the mental burden on quality of life and is a summary of questions related to mental impact of a disease or condition (mental function, role emotional, vitality, and mental health). The score is nomralized to the population and ranges from 0-100, with the US normal value of 50. A lower score represents a greater impact of quality of life. (NCT00832000)
Timeframe: The end of period 1 (week 4) and period 2 (week 9)

Interventionunits on a scale (Mean)
Mexiletine - Period 147.4
Placebo - Period 147.7
Mexiletine - Period 253.1
Placebo - Period 242.7

Short Form 36 - Physical Composite Score

The SF-36 is a standard quality of life instrument. The physical composite score represents the the physical burden on quality of life and is a summary of questions related to physical impact of a disease or condition (physical function, role physical, bodily pain, and general health). The score is nomralized to the population and ranges from 0-100, with the US normal value of 50. A lower score represents a greater impact of quality of life. (NCT00832000)
Timeframe: Particiapnts who experienced weakness on mexiletine in either period 1 or period 2.

Interventionunits on a scale (Mean)
Mexiletine44.8
Placebo39.2

Reviews

3 reviews available for mexiletine and Myotonia

ArticleYear
Blockers of Skeletal Muscle Na
    International journal of molecular sciences, 2023, Jan-03, Volume: 24, Issue:1

    Topics: Humans; Mexiletine; Muscle, Skeletal; Myotonia; NAV1.4 Voltage-Gated Sodium Channel; Syndrome; Volta

2023
[Ion-channel related muscular diseases].
    La Revue de medecine interne, 1999, Volume: 20, Issue:9

    Topics: Acetazolamide; Anti-Arrhythmia Agents; Anticonvulsants; Carbamazepine; Humans; Hypokalemic Periodic

1999
[Na channel myotonia].
    Ryoikibetsu shokogun shirizu, 2001, Issue:35

    Topics: Chromosomes, Human, Pair 7; Diagnosis, Differential; Exons; Genes, Dominant; Humans; Mexiletine; Mut

2001

Trials

5 trials available for mexiletine and Myotonia

ArticleYear
Efficacy and safety of mexiletine in non-dystrophic myotonias: A randomised, double-blind, placebo-controlled, cross-over study.
    Neuromuscular disorders : NMD, 2021, Volume: 31, Issue:11

    Topics: Adult; Aged; Cross-Over Studies; Double-Blind Method; Female; Humans; Male; Mexiletine; Middle Aged;

2021
Combined N-of-1 trials to investigate mexiletine in non-dystrophic myotonia using a Bayesian approach; study rationale and protocol.
    BMC neurology, 2015, Mar-25, Volume: 15

    Topics: Adolescent; Adult; Aged; Algorithms; Bayes Theorem; Cost-Benefit Analysis; Cross-Over Studies; Doubl

2015
Mexiletine is an effective antimyotonia treatment in myotonic dystrophy type 1.
    Neurology, 2010, May-04, Volume: 74, Issue:18

    Topics: Adult; Anti-Arrhythmia Agents; Cross-Over Studies; Double-Blind Method; Female; Humans; Male; Mexile

2010
Mexiletine for symptoms and signs of myotonia in nondystrophic myotonia: a randomized controlled trial.
    JAMA, 2012, Oct-03, Volume: 308, Issue:13

    Topics: Administration, Oral; Adolescent; Adult; Aged; Anti-Arrhythmia Agents; Cross-Over Studies; Double-Bl

2012
Treatment of myotonia with antiarrhythmic drugs.
    Acta neurologica Scandinavica, 1992, Volume: 86, Issue:4

    Topics: Adolescent; Adult; Anti-Arrhythmia Agents; Disopyramide; Dose-Response Relationship, Drug; Drug Admi

1992

Other Studies

20 other studies available for mexiletine and Myotonia

ArticleYear
Improving the understanding of how patients with non-dystrophic myotonia are selected for myotonia treatment with mexiletine (NaMuscla): outcomes of treatment impact using a European Delphi panel.
    BMC neurology, 2021, Dec-01, Volume: 21, Issue:1

    Topics: Adult; Channelopathies; Humans; Mexiletine; Myotonia; NAV1.4 Voltage-Gated Sodium Channel; Quality o

2021
Impact of restricted access to, and low awareness of, mexiletine on people with myotonia: a real-world European survey.
    Neuromuscular disorders : NMD, 2023, Volume: 33, Issue:2

    Topics: Adult; Cross-Sectional Studies; Humans; Mexiletine; Myotonia; Myotonic Dystrophy; Quality of Life; S

2023
Cardiovascular effects of mexiletine for treatment of myotonia in myotonic dystrophy type 1.
    Revista espanola de cardiologia (English ed.), 2021, Volume: 74, Issue:11

    Topics: Humans; Mexiletine; Myotonia; Myotonic Dystrophy

2021
Cost-Based Price Calculation of Mexiletine for Nondystrophic Myotonia.
    Value in health : the journal of the International Society for Pharmacoeconomics and Outcomes Research, 2021, Volume: 24, Issue:7

    Topics: Anti-Arrhythmia Agents; Commerce; Drug Repositioning; Europe; Humans; Mexiletine; Myotonia; Orphan D

2021
Effect of Mexiletine on Muscle Stiffness in Patients With Nondystrophic Myotonia Evaluated Using Aggregated N-of-1 Trials.
    JAMA, 2018, 12-11, Volume: 320, Issue:22

    Topics: Adult; Bayes Theorem; Double-Blind Method; Female; Humans; Male; Mexiletine; Models, Statistical; My

2018
Non-dystrophic myotonia: prospective study of objective and patient reported outcomes.
    Brain : a journal of neurology, 2013, Volume: 136, Issue:Pt 7

    Topics: Adult; Chloride Channels; Cohort Studies; Electrodiagnosis; Exercise; Female; Humans; International

2013
Increased sodium channel use-dependent inhibition by a new potent analogue of tocainide greatly enhances in vivo antimyotonic activity.
    Neuropharmacology, 2017, Volume: 113, Issue:Pt A

    Topics: Animals; Dose-Response Relationship, Drug; Ether-A-Go-Go Potassium Channels; Humans; Male; Mexiletin

2017
Synthesis and in vitro sodium channel blocking activity evaluation of novel homochiral mexiletine analogs.
    Chirality, 2010, Volume: 22, Issue:3

    Topics: Animals; Mexiletine; Muscles; Myotonia; Sodium Channel Blockers

2010
Biophysical characterization of M1476I, a sodium channel founder mutation associated with cold-induced myotonia in French Canadians.
    The Journal of physiology, 2012, Jun-01, Volume: 590, Issue:11

    Topics: Canada; Cell Line; Cold Temperature; Humans; Mexiletine; Mutation; Myotonia; Potassium; Sodium Chann

2012
Mexiletine for treatment of myotonia: a trial triumph for rare disease networks.
    JAMA, 2012, Oct-03, Volume: 308, Issue:13

    Topics: Anti-Arrhythmia Agents; Female; Humans; Male; Mexiletine; Myotonia

2012
Class Ic antiarrhythmics block human skeletal muscle Na channel during myotonia-like stimulation.
    European journal of pharmacology, 2006, Feb-17, Volume: 532, Issue:1-2

    Topics: Anti-Arrhythmia Agents; Cell Line; Dose-Response Relationship, Drug; Flecainide; Humans; Ion Channel

2006
Depressant effects of drugs used in myotonia therapy.
    Proceedings of the Western Pharmacology Society, 1982, Volume: 25

    Topics: Animals; In Vitro Techniques; Ion Channels; Lidocaine; Mexiletine; Muscle Contraction; Myotonia; Phe

1982
Myotonia fluctuans. A third type of muscle sodium channel disease.
    Archives of neurology, 1994, Volume: 51, Issue:11

    Topics: Base Sequence; Exercise Test; Female; Forearm; Humans; Male; Mexiletine; Molecular Sequence Data; My

1994
Unusual causes of stiffness in two hockey players.
    Clinical journal of sport medicine : official journal of the Canadian Academy of Sport Medicine, 1997, Volume: 7, Issue:2

    Topics: Adolescent; Adult; Hockey; Humans; Male; Mexiletine; Myotonia; Myotonia Congenita

1997
Evaluation of the antimyotonic activity of mexiletine and some new analogs on sodium currents of single muscle fibers and on the abnormal excitability of the myotonic ADR mouse.
    The Journal of pharmacology and experimental therapeutics, 1997, Volume: 282, Issue:1

    Topics: Animals; Anti-Arrhythmia Agents; Mexiletine; Mice; Muscle Fibers, Skeletal; Myotonia; Potassium Chan

1997
Effects of temperature and mexiletine on the F1473S Na+ channel mutation causing paramyotonia congenita.
    Pflugers Archiv : European journal of physiology, 1998, Volume: 436, Issue:5

    Topics: Amino Acid Substitution; Biological Transport; Cell Line; Embryo, Mammalian; Genetic Diseases, Inbor

1998
Effect of mexiletine on sea anemone toxin-induced non-inactivating sodium channels of rat skeletal muscle: a model of sodium channel myotonia.
    Neuromuscular disorders : NMD, 1999, Volume: 9, Issue:3

    Topics: Animals; Anti-Arrhythmia Agents; Cnidarian Venoms; Disease Models, Animal; In Vitro Techniques; Memb

1999
Increased hindrance on the chiral carbon atom of mexiletine enhances the block of rat skeletal muscle Na+ channels in a model of myotonia induced by ATX.
    British journal of pharmacology, 1999, Volume: 128, Issue:6

    Topics: Animals; Butylamines; Carbon; Cnidarian Venoms; Membrane Potentials; Mexiletine; Muscle Fibers, Skel

1999
Homologation of mexiletine alkyl chain and stereoselective blockade of skeletal muscle sodium channels.
    European journal of medicinal chemistry, 2000, Volume: 35, Issue:1

    Topics: Animals; Anti-Arrhythmia Agents; Electric Conductivity; Mexiletine; Mice; Mice, Mutant Strains; Mole

2000
Mexiletine block of disease-associated mutations in S6 segments of the human skeletal muscle Na(+) channel.
    The Journal of physiology, 2001, Dec-15, Volume: 537, Issue:Pt 3

    Topics: Binding, Competitive; Cell Line; Computer Simulation; Electric Stimulation; Humans; Ion Channel Gati

2001