Ohtsuka Y, Matsuda M, Ogino T, Kobayashi K, Ohtahara S
Department of Child Neurology, Okayama University Medical School, Japan.
Brain Dev. 1987;9(4):418-21. doi: 10.1016/s0387-7604(87)80116-x.
Fifteen (12.7%) among 118 cases of the West syndrome were effectively treated by high-dose pyridoxal phosphate (PAL-P). 1) Clinical seizures were completely suppressed in 12 cases with PAL-P alone, and in 3 cases by an addition of PAL-P to the previously poorly-effective regimen. At the follow-up, 12 cases have continued to be free from seizures, while two cases relapsed into the Lennox-Gastaut syndrome, and one died. 2) Electroencephalographically hypsarhythmia disappeared by PAL-P in all 15 effective cases. 3) Effective daily dose of PAL-P was 30 to 400 mg. 4) Notably, PAL-P was effective even in the cases with obvious organic brain pathology, such as tuberous sclerosis, porencephaly, holoprosencephaly, postmeningitis, besides 5 idiopathic cases. 5) Efficacy of PAL-P was significantly higher in idiopathic cases than symptomatic cases; 35.7% vs 9.6%. 6) Response to PAL-P was not predictable by any laboratory data nor clinical features. 7) Prognosis of PAL-P responsive cases was favorable; as many as 6 cases developed normally among 14 cases followed-up. Treatment with a high-dose PAL-P should be tried in all cases of the West syndrome at first.
118例韦斯特综合征患儿中，15例（12.7%）经大剂量磷酸吡哆醛（PAL-P）治疗有效。1）12例单用PAL-P临床癫痫发作完全得到控制，3例在原疗效不佳的治疗方案基础上加用PAL-P后发作得到控制。随访时，12例持续无发作，2例复发为伦诺克斯-加斯东综合征，1例死亡。2）15例有效病例经PAL-P治疗后脑电图高峰节律紊乱均消失。3）PAL-P的有效日剂量为30至400毫克。4）值得注意的是，除5例特发性病例外，PAL-P对明显器质性脑病变的病例也有效，如结节性硬化症、脑穿通畸形、前脑无裂畸形、脑膜炎后病例。5）PAL-P对特发性病例的疗效显著高于症状性病例；分别为35.7%和9.6%。6）任何实验室数据或临床特征均无法预测对PAL-P的反应。7）对PAL-P有反应病例的预后良好；随访的14例中有6例发育正常。所有韦斯特综合征病例均应首先尝试大剂量PAL-P治疗。
