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鞘内注射巴氯芬治疗儿童和青少年重度痉挛性四肢瘫和肌张力障碍

[Intrathecal baclofen in the treatment of severe spastic tetraplegia and dystonia in children and adolescents].

作者信息

Illum Niels Ove, Hansen Flemming Juul, Fischer Claudia, Uldall Peter V, Nielsen Ole Amtoft

机构信息

Neurocentret, Neurokirurgisk Afdeling, H:S Rigshopitalet, Juliane Marie Centret, Paediatrisk Klinik I.

出版信息

Ugeskr Laeger. 2003 Apr 21;165(17):1755-9.

Abstract

INTRODUCTION

Continuous intrathecal baclofen has been used over the past years especially in adult patients with spasticity of spinal origin. Children and young adults with severe spasticity and dystonia of cerebral origin are difficult to treat in spite of optimal systemic antispasmotic therapy with baclofen, tizanidine, dantrolene and/or diazepam. Intrathecal baclofen has therefore been applied in a group of young patients.

MATERIAL AND METHODS

Eight children and young adults from East Denmark with spasticity and 12 with dystonia aged 3-18 years (median 10.9 years) were tested, operated and treated with continuous intrathecal baclofen for a period of 2-64 months (median 22.2 months). Registration of efficacy, fillings, adjustments of baclofen and other therapies were performed in an out patient setting since 1995.

RESULTS

Spasticity in lower extremities was reduced from Ashworth score 3.5-4.5 (median 4.2) to Ashworth score 2.5-4.0 (median 2.9; p < 0.001) during infusion with baclofen 5-33 micrograms/kg/24 hours (median 19 micrograms/kg/24 hours). The infusion catheter tip was placed at levels Th1-Th12 (median Th7.5). Peroral baclofen was reduced from an average of 5.0 to 0.44 mg/kg/24 hours, tizanidine from 0.4 to 0.1 mg/kg/24 hours, and dantrolene from 4.0 to 0.4 mg/kg/24 hours. After initial adjustments successively increased dosages of average 0.46 microgram/kg/month were needed to maintain the same level of efficacy. In questionnaires parents or guardians rated less spasticity in lower extremities in 15 out of 19 patients, and less pain in 13 out of 19 patients.

CONCLUSION

Continuous intrathecal baclofen was effective in treating severe spasticity and dystonia of cerebral origin with major effect on muscles of the lower extremities, pelvis, and back and in particular opisthotonus was relieved. Efficacy on upper extremities was far less pronounced.

摘要

引言

在过去几年中,持续鞘内注射巴氯芬已被应用,尤其用于患有脊髓源性痉挛的成年患者。尽管使用巴氯芬、替扎尼定、丹曲林和/或地西泮进行了最佳的全身抗痉挛治疗,但患有严重脑源性痉挛和肌张力障碍的儿童及年轻成人仍难以治疗。因此,鞘内注射巴氯芬已被应用于一组年轻患者。

材料与方法

对来自丹麦东部的8名患有痉挛的儿童及年轻成人和12名患有肌张力障碍的儿童及年轻成人进行了测试、手术,并给予持续鞘内注射巴氯芬治疗2至64个月(中位数为22.2个月),年龄在3至18岁(中位数为10.9岁)。自1995年起在门诊环境中记录疗效、巴氯芬的填充量、调整情况及其他治疗方法。

结果

在鞘内注射5至33微克/千克/24小时(中位数为19微克/千克/24小时)的巴氯芬期间,下肢痉挛程度从Ashworth评分3.5 - 4.5(中位数为4.2)降至Ashworth评分2.5 - 4.0(中位数为2.9;p < 0.001)。输液导管尖端放置在胸1至胸12水平(中位数为胸7.5)。口服巴氯芬从平均5.0毫克/千克/24小时降至0.44毫克/千克/24小时,替扎尼定从0.4毫克/千克/24小时降至0.1毫克/千克/24小时,丹曲林从4.0毫克/千克/24小时降至0.4毫克/千克/24小时。在初始调整后,为维持相同的疗效水平,平均每月需要依次增加0.46微克/千克的剂量。在问卷调查中,19名患者中有15名患者的父母或监护人评定其下肢痉挛减轻,19名患者中有13名患者疼痛减轻。

结论

持续鞘内注射巴氯芬对治疗严重脑源性痉挛和肌张力障碍有效,对下肢、骨盆和背部肌肉有显著效果,尤其是角弓反张得到缓解。对上肢的疗效则远不明显。

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