Hirai K, Seki T, Takuma Y
Department of Pediatrics, Keio University, School of Medicine, Tokyo, Japan.
Neuropeptides. 1998 Dec;32(6):581-6. doi: 10.1016/s0143-4179(98)90089-0.
Eighteen children with West syndrome (5-11 months of age) were selected to receive an oral dose of pyridoxal phosphate, (20-50 mg/kg) for 14 d. Seizures disappeared in one patient. The remaining 17 patients were treated with 0.01 mg/kg synthesized corticotropin intramuscularly for 2 weeks as an additional therapy. Seizures disappeared in all 17 patients within a few days after initiation of the corticotropin. Levels of somatostatin in the cerebrospinal fluid were as follows: 61.0+/-10.7 pg/ml before therapy, 34.2+/-6.4 pg/ml during pyridoxal phosphate therapy, and 26.8+/-4.2 pg/ml after 2 weeks corticotropin therapy. Somatostatin levels in untreated patients were higher (p < 0.05) than those of age-matched controls (35.7+/-11.8 pg/ml) and decreased (p < 0.05) after pyridoxal phosphate treatment. Somatostatin is a hypothalamic tetradecapeptide with excitatory effects on neurons and pyridoxal phosphate might subclinically influence neuronal excitation.
选取18名患有韦斯特综合征（年龄5 - 11个月）的儿童，口服磷酸吡哆醛（20 - 50毫克/千克），持续14天。1例患者癫痫发作消失。其余17例患者作为附加治疗，肌内注射0.01毫克/千克合成促肾上腺皮质激素，持续2周。在开始促肾上腺皮质激素治疗后的几天内，所有17例患者的癫痫发作均消失。脑脊液中生长抑素水平如下：治疗前为61.0±10.7皮克/毫升，磷酸吡哆醛治疗期间为34.2±6.4皮克/毫升，促肾上腺皮质激素治疗2周后为26.8±4.2皮克/毫升。未经治疗的患者生长抑素水平高于年龄匹配的对照组（35.7±11.8皮克/毫升）（p < 0.05），且在磷酸吡哆醛治疗后降低（p < 0.05）。生长抑素是一种对神经元有兴奋作用的下丘脑十四肽，磷酸吡哆醛可能在亚临床水平影响神经元兴奋。
