>Introduction: Treatment with intrathecal baclofen (ITB) is a therapeutic option in the management of severe spasticity in patients with hereditary spastic paraparesis (HSP). However, information on the impact of ITB on the natural course of disease, especially the effect of ITB on functional parameters over time is limited.>Materials and Methods: We evaluated seven patients with HSP retrospectively who were treated with an ITB device. The following parameters were measured before (pre-implantation) and after implantation (post-implantation) of the ITB device at steady state dosage of ITB and annually until last follow-up: modified Ashworth Scale, Reflex Scale, modified Rankin Scale, and Rivermead Mobility Index. The ITB dosages were assessed after reaching steady state as well as annually until last follow-up.>Results: The ITB device was implanted 13 ± 6 (range 9–16) years after diagnosis of HSP on average. Severe spasticity was controlled in all patients by a mean baclofen dosage of 188 ± 60 (range 145–230) μg per day at steady state post-implantation. The modified Ashworth Scale improved significantly from 3 (interquartile range [IQR] 3–3.25) to 1 (IQR 1–1.25; p = 0.046), as did the Reflex Scale from 5 (IQR 4.75–5) to 3 (IQR 2.75–3; p = 0.046) at steady state dosage of ITB. The modified Rankin Scale improved from 2 (IQR 2–2) to 1 (IQR 1–1.5; p = 0.083) and the Rivermead Mobility Index remained 14 (IQR 13.5–14 pre-implantation, IQR 14–14 post-implantation; p = 0.18). Post-implantation, spasticity improved for 2–3 years, followed by a stable phase of ambulatory and other mobility functions for 4–5 years. Thereafter, the maintenance or progressive loss of mobility depended on individual courses of the disease. No ITB-related severe side effects occurred.>Discussion: Our data further support the role of ITB in the treatment of severe spasticity in patients with deteriorated walking performance suffering HSP. ITB therapy may initially improve spasticity and stabilize mobility functions for the first 6–8 years in patients with HSP.
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机译：>简介：鞘内注射巴氯芬（ITB）是遗传性痉挛性轻瘫（HSP）患者严重痉挛的治疗选择。但是，有关ITB对疾病自然进程的影响，尤其是随时间推移ITB对功能参数的影响的信息有限。>材料和方法：我们回顾性评估了7例接受过HSP治疗的HSP患者ITB设备。在ITB装置的稳态剂量下以及每年直至最后一次随访之前，在ITB装置植入前（植入前）和植入后（植入后）测量以下参数：改良的Ashworth量表，反射量表，兰金量表和Rivermead流动性指数。在达到稳定状态后以及每年一次直至最后一次随访之前，对ITB剂量进行评估。>结果：平均而言，ITB装置在HSP诊断后13±6年（9-16年）被植入。在植入后的稳态下，所有患者的严重痉挛状态均通过每天平均188±60μg（范围145–230）μg的巴氯芬剂量来控制。改良的Ashworth量表从3（四分位数范围[IQR] 3–3.25）显着提高到1（IQR 1–1.25； p = 0.046），反射量表也从5（IQR 4.75–5）提高到3（IQR 2.75– 3； p = 0.046）在ITB的稳态剂量下。改良的兰金量表从2（IQR 2–2）改善为1（IQR 1–1.5； p = 0.083），Rivermead流动性指数保持14（IQR 13.5–14植入前，IQR 14–14植入后； p = 0.18）。植入后，痉挛改善了2–3年，随后进入了动态状态和其他活动功能稳定阶段4–5年。此后，维持或逐渐丧失活动能力取决于疾病的各个病程。没有发生与ITB相关的严重副作用。>讨论：我们的数据进一步支持了ITB在患有HSP的步行功能恶化的患者中严重痉挛的治疗中的作用。对于HSP患者，在最初的6-8年中，ITB疗法可最初改善痉挛并稳定其活动功能。
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