Albright A Leland, Ferson Susan S
Department of Neurosurgery, University of Wisconsin Health Center, Madison, Wisconsin 53792, USA.
J Neurosurg Pediatr. 2009 Jan;3(1):11-4. doi: 10.3171/2008.10.PEDS0847.
The aim of this study was to evaluate the use of intraventricular baclofen (IVB) for the treatment of severe generalized secondary and heredodegenerative dystonia.
Nine children and 1 adult with severe dystonia unresponsive to multiple oral medications were treated with IVB. Intraventricular catheters were positioned endoscopically in the third ventricle.
Eight of the 10 patients responded to IVB; their mean dystonia scores on the Barry-Albright dystonia scale decreased from 23 to 8. The 2 patients who did not respond had not responded to previous high doses of intrathecal baclofen. No adverse side effects related to IVB occurred. One child developed a pump infection that required pump removal, 1 developed a CSF infection that cleared after antibiotic administration, and 1 developed ventriculomegaly that required a shunt placement.
Intraventricular baclofen is an effective method of infusing baclofen to treat severe, generalized secondary dystonia, and, at times, heredodegenerative dystonia. The site of baclofen's activity when treating dystonia may be at the cortical level, and intraventricular infusion may result in higher baclofen concentrations over the cortex than intrathecal infusion. Additional studies are necessary to determine whether IVB is effective at lower doses than those used with intrathecal baclofen administration.
本研究旨在评估脑室内注射巴氯芬(IVB)治疗严重全身性继发性和遗传性变性肌张力障碍的效果。
9名儿童和1名对多种口服药物无反应的严重肌张力障碍成人接受了IVB治疗。通过内镜将脑室内导管置于第三脑室。
10名患者中有8名对IVB有反应;他们在巴里 - 奥尔布赖特肌张力障碍量表上的平均肌张力障碍评分从23降至8。2名无反应的患者对先前高剂量鞘内注射巴氯芬也无反应。未发生与IVB相关的不良副作用。1名儿童发生泵感染,需要移除泵;1名发生脑脊液感染,抗生素治疗后清除;1名发生脑室扩大,需要放置分流管。
脑室内注射巴氯芬是一种有效的输注巴氯芬的方法,可用于治疗严重的全身性继发性肌张力障碍,有时也可用于治疗遗传性变性肌张力障碍。巴氯芬治疗肌张力障碍时的作用部位可能在皮质水平,脑室内输注可能比鞘内输注在皮质上产生更高的巴氯芬浓度。需要进一步研究以确定IVB是否在比鞘内注射巴氯芬更低的剂量下有效。