Primary lateral sclerosis is a disorder categorized by insidious onset of progressive upper motor neuron dysfunction without lower motor neuron involvement. PLS often presents with gradual-onset, progressive lower extremity stiffness and pain due to muscle spasticity. Intrathecal Baclofen pumps (ITB) have been used to effectively treat spasticity in several neurologic conditions including MS and spinal cord injury. This study aimed at reviewing a cohort of PLS patients with spasticity requiring ITB to assess the clinical course, benefits, and complications in these patients. A series of 5 patients were identified who were diagnosed with PLS and received ITB as treatment for spasticity. The average age of the patients at the time of ITB insertion was 56.4 years. The average length of treatment was 10.4 years with a range of 4-15 years. All patients reported improvement in spasticity as measured by clinical examinations and Ashworth scores; 1/5 had complications with the pump related to migration of catheter. No patients required permanent removal of the ITB. ITB is a safe and effective treatment for spasticity in PLS and should be considered in other patients.

中文翻译：

---

鞘内注射巴氯芬泵在原发性侧索硬化症中的应用

原发性侧索硬化是一种疾病，分类为渐进性上运动神经元功能障碍的隐匿性发作，而没有下运动神经元受累。由于肌肉痉挛，PLS 常表现为渐进性、渐进性下肢僵硬和疼痛。鞘内注射巴氯芬泵 (ITB) 已被用于有效治疗多种神经系统疾病的痉挛状态，包括 MS 和脊髓损伤。本研究旨在审查一组需要 ITB 的痉挛 PLS 患者，以评估这些患者的临床病程、益处和并发症。确定了一系列 5 名患者，他们被诊断患有 PLS 并接受 ITB 作为痉挛的治疗。插入 ITB 时患者的平均年龄为 56.4 岁。平均治疗时间为10.4年，范围为4-15年。根据临床检查和 Ashworth 评分，所有患者均报告痉挛状态有所改善；1/5 有与导管迁移相关的泵并发症。没有患者需要永久移除 ITB。ITB 是一种安全有效的 PLS 痉挛治疗方法，应考虑用于其他患者。