Portaro Simona, Biasini Fiammetta, Bramanti Placido, Naro Antonino, Calabrò Rocco Salvatore
IRCCS Centro Neurolesi Bonino Pulejo, Messina, Italy.
Medicine (Baltimore). 2020 Jul 10;99(28):e21117. doi: 10.1097/MD.0000000000021117.
we report on the first case of a woman affected by chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and recessive myotonia congenita (MC), treated with mexiletine. We aimed at describing the possible role of mexiletine in CIDP management.
A 44-year-old female affected by CIDP and MC, gained beneficial effects for CIDP symptoms (muscle weakness, cramps, and fatigue) and relapses, after mexiletine intake (200 mg twice a day). The patient presented with detrimental effects after mexiletine drop out, with a worsening of CIDP symptoms.
The patient reported a nearly complete remission of muscle stiffness and weakness up to 3 years since mexiletine intake. Then, she developed an allergic reaction with glottis edema, maybe related to mexiletine intake, as per emergency room doctors' evaluation, who suggested withdrawing the drug.
The patient significantly worsened after the medication drop out concerning both CIDP and MC symptoms.
This is the first report on the association of CIDP and MC in the same patient. Such diseases may share some clinical symptoms related to a persistent sodium currents increase, which maybe due either to the over-expression of sodium channels following axonal damage due to demyelination or to the chloride channel genes mutations. This is the possible reason why mexiletine maybe promising to treat CIDP symptoms.
我们报告了首例患有慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)和隐性先天性肌强直(MC)的女性患者,使用美西律进行治疗。我们旨在描述美西律在CIDP治疗中的可能作用。
一名44岁患有CIDP和MC的女性,在服用美西律(每日两次,每次200毫克)后,CIDP症状(肌肉无力、痉挛和疲劳)及病情复发得到了改善。患者在停用美西律后出现了有害影响,CIDP症状恶化。
患者报告自服用美西律以来,肌肉僵硬和无力几乎完全缓解,长达3年。然后,根据急诊室医生的评估,她出现了伴有声门水肿的过敏反应,可能与服用美西律有关,医生建议停用该药物。
停药后,患者的CIDP和MC症状均显著恶化。
这是首例关于同一患者同时患有CIDP和MC的报告。这些疾病可能共享一些与持续性钠电流增加相关的临床症状,这可能是由于脱髓鞘导致轴突损伤后钠通道过度表达,或者是由于氯通道基因突变。这可能是美西律有望治疗CIDP症状的原因。