Stayer C, Tronnier V, Dressnandt J, Mauch E, Marquardt G, Rieke K, Müller-Schwefe G, Schumm F, Meinck H M
Department of Neurology, University of Heidelberg, Germany.
Neurology. 1997 Dec;49(6):1591-7. doi: 10.1212/wnl.49.6.1591.
We report on eight patients with stiff-man syndrome (SMS) or its "plus" variant, progressive encephalomyelopathy with rigidity and myoclonus (PERM) receiving intrathecal baclofen via pump. In six of the patients, follow-ups continued for approximately 2.5 to 6.5 years after pump implantation. Intrathecal baclofen was an effective last-resort alternative for patients who responded poorly to or did not tolerate oral antispasticity medications. General mobility increased, and spasms and rigidity were reduced; however, no complete remissions were observed either before or after pump implantation. PERM patients showed more severe and rapid progression of symptoms and more attacks of autonomic dysregulation than SMS patients. They also required higher doses and more rapid dosage increases. Complications of intrathecal baclofen therapy included spasm-induced rupture of the catheter, catheter dislocation causing radicular symptoms, and pump malfunction resulting in inaccurate dosage administration. Patients suffered fewer side effects with intrathecal baclofen than with oral medication, but overdose resulted in a transient, comalike state in one patient and sudden dosage reduction due to pump failure was fatal in another.
我们报告了8例患有僵人综合征（SMS）或其“附加”型变体——进行性脑脊髓病伴强直和肌阵挛（PERM）的患者，他们通过泵接受鞘内注射巴氯芬治疗。其中6例患者在泵植入后持续随访了约2.5至6.5年。鞘内注射巴氯芬对于那些对口服抗痉挛药物反应不佳或不耐受的患者而言，是一种有效的最后手段。患者的总体活动能力增强，痉挛和强直症状减轻；然而，在泵植入前后均未观察到完全缓解的情况。与SMS患者相比，PERM患者的症状进展更为严重和迅速，自主神经失调发作也更多。他们还需要更高的剂量以及更快的剂量增加速度。鞘内注射巴氯芬治疗的并发症包括痉挛导致的导管破裂、导管移位引起神经根症状以及泵故障导致给药剂量不准确。与口服药物相比，患者使用鞘内注射巴氯芬时出现的副作用较少，但有1例患者因用药过量导致短暂的昏迷状态，另1例患者因泵故障导致突然减少剂量而死亡。
