Department of Pediatrics, Peking University First Hospital, No.1, Xi'anmen Street, Xicheng District, Beijing 100034, China.
World J Pediatr. 2018 Jun;14(3):290-297. doi: 10.1007/s12519-018-0127-9. Epub 2018 Apr 26.
Infantile spasms (IS) was an epileptic disease with varied treatment widely among clinicians. Here, we aimed to compare and analyze the clinical characteristics of IS response to pyridoxine or topiramate monotherapy (TPM control IS).
The clinical manifestations, treatment processes and outcomes were analyzed in 11 pyridoxine responsive IS and 17 TPM-control IS.
Of the 11 patients with pyridoxine responsive IS, nine were cryptogenic/idiopathic. Age of seizure onset was 5.36 ± 1.48 months. Spasms were controlled within a week in most of the patients. At the last follow-up, EEG returned to normal in 8. Psychomotor development was normal in 6, mild delay in 3, severe delay in 2. Of the 17 patients with TPM-control IS, 10 were cryptogenic/idiopathic. The age of seizure onset was 5.58 ± 2.09 months. All patients were controlled within a month. At the last follow-up, EEG was normal in 10. Psychomotor development was normal in 8, mild delay in 5, severe delay in 4. Genetic analysis did not show any meaningful results.
The clinical characteristics and disease courses of pyridoxine responsive IS and TPM-control IS were similar, which possibly clued for a same pathogenic mechanism. Pyridoxine should be tried first in all IS patients, even in symptomatic cases. If patients were not responsive to pyridoxine, TPM could be tried.
婴儿痉挛症(IS)是一种癫痫疾病,临床治疗方法多样。本研究旨在比较和分析吡哆醇或托吡酯单药治疗(TPM 控制 IS)对 IS 反应的临床特征。
分析了 11 例吡哆醇反应性 IS 和 17 例 TPM 对照 IS 的临床表现、治疗过程和结局。
11 例吡哆醇反应性 IS 中,9 例为隐源性/特发性。发病年龄为 5.36±1.48 个月。大多数患者在一周内控制痉挛。最后一次随访时,8 例脑电图恢复正常。6 例精神运动发育正常,3 例轻度延迟,2 例重度延迟。17 例 TPM 对照 IS 中,10 例为隐源性/特发性。发病年龄为 5.58±2.09 个月。所有患者均在一个月内得到控制。最后一次随访时,10 例脑电图正常。8 例精神运动发育正常,5 例轻度延迟,4 例重度延迟。遗传分析未显示任何有意义的结果。
吡哆醇反应性 IS 和 TPM 对照 IS 的临床特征和病程相似,可能提示存在相同的发病机制。所有 IS 患者均应首先尝试使用吡哆醇,即使是症状性病例。如果患者对吡哆醇无反应,可尝试使用托吡酯。