Kelemen Anna, Rásonyl György, Neuwirth Magdolna, Barcs Gábor, Szucs Anna, Jakus Rita, Fabó Dániel, Juhos Vera, Pálfy Beatrix, Halász Péter
National Institute of Neurosciences, Epilepsy Center, Budapest.
Ideggyogy Sz. 2011 Mar 30;64(5-6):187-92.
Zonisamide is licensed in the European Union for adjunctive therapy for partial epilepsy, but its efficacy in generalized epilepsy was less explored.
This prospective observational study included 47 patients (mean age 29 years, range 3-50) with different resistant generalized epilepsy syndromes: idiopathic generalized syndromes (IGE) 15 patients, (juvenile myoclonic epilepsy four, absence epilepsy four, myoclonic absence two, unclassified IGE five), progressive myoclonic epilepsy type 1 (PME1) four, severe myoclonic epilepsy of infancy (SMEI) three, borderline SMEI three, Lennox-Gastaut syndrome/secondary generalized epileptic encephalopties 23 patients. All patients were followed up for at least six months. The mean dose given was 367 mg/day (range 100-600 mg/day), the patients received at least one and no more than two concomitant AE. Response was defined as more than 50% seizure reduction or seizure freedom.
The best effect was achieved in PME one, all the patients were responders. Myoclonic seizures were reduced 80%, none of the patients had generalized tonic clonic (GTC) seizures. In two of the four patients all other antiepileptics were tapered of (including piracetam), so they were GTC seizure and almost myoclonia free on zonisamide only. Responder rates were in GEFS +/- SME 62.5%, in resistant IGE 62.5%, and in epileptic encephalopathies 33.3% patients. Tolerance after initial efficacy developed in six patients. Adverse effects were mild: weight loss, somnolence and confusion were repeatedly reported. Three patients reported cognitive improvement.
Clinical benefit of a broad spectrum antiepileptic zonisamide extends across seizure types, ages and epilepsy syndromes. The efficacy in PME proved to be excellent.
唑尼沙胺在欧盟被批准用于部分性癫痫的辅助治疗，但其在全身性癫痫中的疗效研究较少。
这项前瞻性观察性研究纳入了47例（平均年龄29岁，范围3 - 50岁）患有不同难治性全身性癫痫综合征的患者：特发性全身性综合征（IGE）15例（青少年肌阵挛癫痫4例、失神癫痫4例、肌阵挛失神2例、未分类的IGE 5例），1型进行性肌阵挛癫痫（PME1）4例，婴儿严重肌阵挛癫痫（SMEI）3例，边缘性SMEI 3例，Lennox - Gastaut综合征/继发性全身性癫痫性脑病23例。所有患者均随访至少6个月。平均给药剂量为367毫克/天（范围100 - 600毫克/天），患者接受至少一种且不超过两种伴随的抗癫痫药物。反应定义为癫痫发作减少超过50%或无癫痫发作。
PME1患者取得了最佳效果，所有患者均有反应。肌阵挛发作减少了80%，没有患者出现全身性强直阵挛（GTC）发作。在4例患者中的2例中，所有其他抗癫痫药物都逐渐减量（包括吡拉西坦），因此他们仅使用唑尼沙胺就无GTC发作且几乎无肌阵挛。在GEFS +/- SME患者中的反应率为62.5%，在难治性IGE患者中为62.5%，在癫痫性脑病患者中为33.3%。6例患者在初始疗效后出现耐受性。不良反应轻微：体重减轻、嗜睡和意识模糊被反复报告。3例患者报告认知功能改善。
广谱抗癫痫药物唑尼沙胺的临床益处涵盖多种癫痫发作类型、年龄和癫痫综合征。其在PME中的疗效被证明非常出色。
