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L-5-羟色氨酸用于治疗几种以肌阵挛突出的不同综合征。

L-5-hydroxytryptophan in treatment of several different syndromes in which myoclonus is prominent.

作者信息

Growdon J H, Young R R, Shahani B T

出版信息

Neurology. 1976 Dec;26(12):1135-40. doi: 10.1212/wnl.26.12.1135.

Abstract

The serotonin precursor L-5-hydroxytryptophan is useful therapy for patients with posthypoxic intention myoclonus. L-5-hydroxytryptophan plus carbidopa was administered to eight patients with this disorder or other syndromes in which myoclonus is prominent. This treatment (1) decreased the frequency of occurrence and amplitude of intention myoclonus in two patients with posthypoxic intention myoclonus and in one with idiopathic myoclonus, (2) had no effect in one patient with congenital encephalopathy and myoclonus, and (3) increased the frequency of occurrence and amplitude of myoclonus in two patients with lipid storage disease, one with myoclonic epilepsy, and in an additional patient with idiopathic myoclonus. Therefore, L-5-hydroxytryptophan does not effect improvement in all forms of myoclonus; it should be given with caution because it produces a high incidence of side effects. A patient's response to L-5-hydroxytryptophan therapy may be important in a diagnostic classification of myoclonic syndromes based on differences in indoleamine neurotransmitter function.

摘要

血清素前体L - 5 - 羟色氨酸对缺氧后意向性肌阵挛患者是一种有效的治疗方法。我们对8例患有这种疾病或其他以肌阵挛为突出表现的综合征的患者给予L - 5 - 羟色氨酸加卡比多巴治疗。该治疗方法:(1)使2例缺氧后意向性肌阵挛患者和1例特发性肌阵挛患者的意向性肌阵挛发作频率和幅度降低;(2)对1例先天性脑病伴肌阵挛患者无效;(3)使2例脂质贮积病患者、1例肌阵挛性癫痫患者及另1例特发性肌阵挛患者的肌阵挛发作频率和幅度增加。因此,L - 5 - 羟色氨酸并非对所有形式的肌阵挛均有改善作用;因其副作用发生率高,故应谨慎使用。基于吲哚胺神经递质功能差异,患者对L - 5 - 羟色氨酸治疗的反应在肌阵挛综合征的诊断分类中可能具有重要意义。

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