Mexiletine Hydrochloride - CAS 5370-01-4
Catalog number: 5370-01-4
Category: Inhibitor
Not Intended for Therapeutic Use. For research use only.
Molecular Formula:
Molecular Weight:
Sodium Channel
Mexiletine HCl belongs to Class IB anti-arrhythmic group of medicines, inhibits sodium channels to reduce the inward sodium current.
Canonical SMILES:
1.Flecainide-Responsive Myotonia Permanens With SNEL Onset: A New Case and Literature Review.
Portaro S1, Rodolico C2, Sinicropi S2, Musumeci O2, Valenzise M3, Toscano A4. Pediatrics. 2016 Mar 4. pii: peds.2015-3289. [Epub ahead of print]
Sodium channel myotonias are inherited muscle diseases linked to mutations in the voltage-gated sodium channel. These diseases may also affect newborns with variable symptoms. More recently, severe neonatal episodic laryngospasm (SNEL) has been described in a small number of patients. A timely diagnosis of SNEL is crucial because a specific treatment is now available that will likely reduced laryngospasm and improve vital and cerebral outcomes. We report here on an 8-year-old girl who had presented, at birth, with SNEL who subsequently developed myotonia permanens starting at age 3 years. Results of molecular analysis revealed a de novo SCN4A G1306E mutation. The girl was treated with carbamazepine, acetazolamide, and mexiletine, with little improvement; after switching her treatment to flecainide, she experienced a dramatic reduction in muscle stiffness and myotonic symptoms as well as an improvement in behavior.
2.Gene-Specific Therapy With Mexiletine Reduces Arrhythmic Events in Patients With Long QT Syndrome Type 3.
Mazzanti A1, Maragna R1, Faragli A1, Monteforte N1, Bloise R1, Memmi M1, Novelli V1, Baiardi P1, Bagnardi V2, Etheridge SP3, Napolitano C1, Priori SG4. J Am Coll Cardiol. 2016 Mar 8;67(9):1053-8. doi: 10.1016/j.jacc.2015.12.033.
BACKGROUND: Long QT syndrome type 3 (LQT3) is a lethal disease caused by gain-of-function mutations in the SCN5A gene, coding for the alpha-subunit of the sodium channel NaV1.5. Mexiletine is used to block late sodium current and to shorten QT interval in LQT3 patients.
3.A randomized trial of mexiletine in ALS: Safety and effects on muscle cramps and progression.
Weiss MD1, Macklin EA2, Simmons Z2, Knox AS2, Greenblatt DJ2, Atassi N2, Graves M2, Parziale N2, Salameh JS2, Quinn C2, Brown RH Jr2, Distad JB2, Trivedi J2, Shefner JM2, Barohn RJ2, Pestronk A2, Swenson A2, Cudkowicz ME2; Mexiletine ALS Study Group. Neurology. 2016 Feb 24. pii: 10.1212/WNL.0000000000002507. [Epub ahead of print]
OBJECTIVE: To determine the safety and tolerability of mexiletine in a phase II double-blind randomized controlled trial of sporadic amyotrophic lateral sclerosis (SALS).
4.The Complexity of Pain Management in Patients with Erythromelalgia.
Patel N1, Chen E, Cucchiaro G. A A Case Rep. 2015 Nov 1;5(9):151-3. doi: 10.1213/XAA.0000000000000201.
A 15-year-old girl diagnosed with erythromelalgia was admitted to the hospital with severe pain in her feet associated with burning, pruritus, erythema, and swelling. She had not responded to conventional management and received some relief only from cold bath immersions, which resulted in chronic blistering and multiple episodes of superinfection. After a successful trial of spinal cord stimulation, she had a permanent implantation procedure. The spinal cord stimulator relieved her pain and improved function but not the sensation of burning pain. However, this pain resolved after she started daily mexiletine. This case demonstrates that erythromelalgia sometimes can be managed successfully with a combination of pharmacologic and interventional procedures.
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CAS 5370-01-4 Mexiletine Hydrochloride

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