Background

We evaluated zonisamide therapy in patients with paroxysmal kinesigenic dyskinesia (PKD).

Methods

We analyzed zonisamide therapy in 17 patients with PKD at Saitama Children’s Medical Center between November 1994 and April 2020. We collected information regarding family history, previous history, age at onset, age at zonisamide commencement, dyskinesia characteristics, brain magnetic resonance imaging, interictal electroencephalography, treatment lag, zonisamide efficacy, zonisamide dose, serum zonisamide concentration, and adverse effects. We evaluated PKD frequency at six months after zonisamide therapy commencement.

Results

Fourteen patients met the inclusion criteria. The median age at zonisamide therapy commencement was 12.8 (9.4 to 16.3) years. Zonisamide therapy was effective in 13 of 14 (92.9%) patients: complete remission for more than three months after zonisamide therapy (n = 7), decreased dyskinesia frequency by more than 90% (n = 4), dyskinesia frequency by 75% to 90% (n = 2), and no change of dyskinesia frequency (n = 1). The initial and maintenance zonisamide doses were 2.0 (1.4 to 3.8) and 2.0 (1.5 to 5.9) mg/kg/day, respectively. The median duration between zonisamide therapy commencement and dyskinesia decrease or cessation was 4 (1 to 60) days: 10 of 14 (71.4%) patients responded to zonisamide within one week after zonisamide therapy commencement. Regarding adverse effects, two patients experienced somnolence and one developed reduced perspiration.

Conclusions

We suggest that zonisamide monotherapy is effective for patients with PKD as a first-line treatment. We can evaluate the efficacy of zonisamide therapy within one week. Because zonisamide lacks the enzyme-inducing effects of carbamazepine and phenytoin, it may be useful for PKD treatment.

中文翻译：

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唑尼沙胺疗法用于阵发性人机代运动障碍的患者。

背景

我们评估了佐尼沙胺治疗阵发性运动性运动障碍（PKD）的患者。

方法

我们在1994年11月至2020年4月期间于琦玉儿童医学中心对17例PKD患者的zonisamide治疗进行了分析。我们收集了有关家族史，既往史，发病年龄，zonisamide起始年龄，运动障碍特征，脑磁共振成像，小脑电图的信息。 ，治疗滞后，唑尼沙胺疗效，唑尼沙胺剂量，血清唑尼沙胺浓度和不良反应。我们在唑尼沙胺治疗开始六个月后评估了PKD频率。

结果

14名患者符合纳入标准。zonisamide治疗开始时的中位年龄为12.8（9.4至16.3）年。唑尼沙胺治疗对14例患者中的13例有效（92.9％）：唑尼沙胺治疗后完全缓解三个月以上（n = 7），运动障碍频率降低90％以上（n = 4），运动障碍频率降低75％至90％（n = 2），并且运动障碍频率无变化（n = 1）。唑尼沙胺的初始和维持剂量分别为2.0（1.4至3.8）和2.0（1.5至5.9）mg / kg /天。唑尼沙胺治疗开始与运动障碍减少或停止之间的中位持续时间为4（1至60）天：唑尼沙胺治疗开始后一周内，14名患者中有10名（71.4％）对唑尼沙胺有反应。关于不良反应，两名患者出现嗜睡现象，一名患者排汗减少。

结论

我们建议zonisamide单一疗法作为一线治疗对PKD患者有效。我们可以在一周内评估zonisamide治疗的疗效。由于唑尼沙胺缺乏卡马西平和苯妥英钠的酶诱导作用，因此对PKD治疗可能有用。