Clinical characteristics of two cohorts of infantile spasms: response to pyridoxine or topiramate monotherapy
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ORIGINAL ARTICLE
Clinical characteristics of two cohorts of infantile spasms: response to pyridoxine or topiramate monotherapy Jiao Xue1 · Ping Qian1 · Hui Li1 · Ye Wu1 · Hui Xiong1 · Yue‑Hua Zhang1 · Zhi‑Xian Yang1 Received: 13 December 2016 / Revised: 25 April 2017 / Accepted: 25 April 2017 © Children’s Hospital, Zhejiang University School of Medicine 2018
Abstract Background 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). Methods The clinical manifestations, treatment processes and outcomes were analyzed in 11 pyridoxine responsive IS and 17 TPM-control IS. Results 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 TPMcontrol 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. Conclusions 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. Keywords Infantile spasms · Pyridoxine · Topiramate
Introduction Infantile spasms (IS), characterized by brief, symmetric axial muscle contraction (neck, trunk and/or extremities), was first described by West in 1841 [1]. IS most often occurred during the first year of life with an incidence of approximately 1 per 2000–4000 live births [2]. The treatment of IS varied widely among clinicians. Adrenocorticotrophic hormone (ACTH) and vigabatrin (VGB) were suggested as the first-line treatments. Other antiepileptic drugs (AEDs) as well as ketogenic diet might also be useful [3]. Besides, high-dose pyridoxine had been recognized as a treatment choice since first attempted by Ohtahara [4], and widely used as the initial therapeutic agent of IS in Japan [5]. After excluding other pyridoxine-related diseases such * Zhi‑Xian Yang [email protected] 1
Department of Pediatrics, Peking University First Hospital, No.1, Xi’anmen Street, Xicheng District, Beijing 100034, China
as pyridoxine deficient seizures and pyridoxine dependent epilepsy (PDE) [6–8], those IS controlled by pyridoxine was termed as pyridoxine responsive IS, the most common type of pyridoxine responsive epilepsy (PRE) [5]. Up to now, the studies on pyridoxine responsive IS were limited. In recent years, high-dose pyridoxine has been u
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