Zhang Bruce, Lau Richard, van Why David, Saulino Michael
Department of Physical Medicine and Rehabilitation, Temple University Hospital, Philadelphia, PA, USA.
MossRehab, Elkins Park, PA, USA.
J Rehabil Med Clin Commun. 2021 Mar 3;4:1000052. doi: 10.2340/20030711-1000052. eCollection 2021.
Intrathecal baclofen is considered an adjuvant therapy for patients with intractable spasms due to stiff-person syndrome. There is increasing evidence to support the use of intrathecal baclofen in the management of symptomatic stiffperson syndrome, with improvement in function.
A 38-year-old woman with stiff- person syndrome initially presented to inpatient rehabilitation for intractable muscle spasms. The symptoms made her non-ambulatory and limited her tolerance to wheelchair use for mobility. The patient underwent up-titration of oral baclofen and diazepam, with concurrent intravenous immunoglobulin cycles, leading to transient symptom relief. She agreed to explore intrathecal baclofen therapy. An initial trial of a single bolus of 50 μg intrathecal baclofen resulted in a significant decrease in spontaneous spasms, enabling modified independence in transfers and ambulation. The patient was subsequently implanted with a permanent intrathecal delivery system. To date, the intrathecal baclofen had been titrated to 186 μg per day with simple continuous delivery. The patient was weaned off oral baclofen. She attained complete functional independence with ambulation without the need for assistive devices, and has had no lasting post-procedural complications to date.
This case report adds to the increasing evidence of cases of refractory stiff-person syndrome managed successfully using intrathecal baclofen therapy.
鞘内注射巴氯芬被认为是治疗僵人综合征所致顽固性痉挛患者的一种辅助疗法。越来越多的证据支持鞘内注射巴氯芬用于有症状的僵人综合征的治疗，并能改善功能。
一名38岁患有僵人综合征的女性最初因顽固性肌肉痉挛入住住院康复科。这些症状使她无法行走，并限制了她使用轮椅活动的耐受性。患者接受了口服巴氯芬和地西泮的剂量递增治疗，并同时进行静脉注射免疫球蛋白治疗，症状得到短暂缓解。她同意探索鞘内注射巴氯芬治疗。鞘内注射50μg巴氯芬单次推注的初始试验使自发性痉挛显著减少，使患者在转移和行走方面实现了改良的独立。患者随后植入了永久性鞘内给药系统。迄今为止，鞘内注射巴氯芬已通过简单持续给药滴定至每日186μg。患者停用了口服巴氯芬。她在行走方面实现了完全功能独立，无需辅助设备，且迄今为止没有持久的术后并发症。
本病例报告进一步证明了鞘内注射巴氯芬疗法成功治疗难治性僵人综合征病例的证据不断增加。
