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鞘内注射巴氯芬治疗神经纤维瘤病相关脊髓损伤伴痉挛——病例报告

INTRATHECAL BACLOFEN FOR NEUROFIBROMATOSIS RELATED SPINAL CORD INJURY WITH SPASTICITY - A CASE REPORT.

作者信息

O'Brien Carl, Stowe Jacqui, O'Connor Michael, Morgan Jacinta, Murphy Paul, Roddy Darren, Levins Kirk

机构信息

National Rehabilitation Hospital, Rochestown Avenue, Dun Laoghaire Co.

Royal College of Surgeons.

出版信息

J Rehabil Med Clin Commun. 2024 Aug 13;7:25912. doi: 10.2340/jrm-cc.v7.25912. eCollection 2024.

Abstract

This case presents a 35-year-old male with spinal predominant neurofibromatosis-1 who developed an incomplete spinal cord injury (C3 Asia C) which did not improve despite urgent decompressive surgery for multiple cervical neurofibromas. This report outlines a novel indication for intrathecal baclofen. The patient suffered from lower limb weakness with severe spasticity and required assistance of at least two for all activities. The aim of rehabilitation was to improve overall tone while focusing on independence, mobility and quality of life. After a successful trial of intrathecal baclofen, he underwent implantation of an intrathecal baclofen pump. The dose was gradually increased while he received a progressive programme of stretching and functional rehabilitation therapy. After 6 weeks, his MAS had improved to 1-2/4 and he had progressed to independent transfers, independence for most activities of daily living and was able to discharge to his family home with minimal support.

摘要

该病例为一名35岁男性,患有以脊柱为主的1型神经纤维瘤病,发生了不完全性脊髓损伤(C3亚洲损伤分级C级),尽管因多发性颈椎神经纤维瘤接受了紧急减压手术,但病情并未改善。本报告概述了鞘内注射巴氯芬的一种新适应症。患者下肢无力且伴有严重痉挛,所有活动至少需要两人协助。康复的目标是改善整体肌张力,同时注重独立性、活动能力和生活质量。在鞘内注射巴氯芬试验成功后,他接受了鞘内巴氯芬泵植入术。在他接受渐进性伸展和功能康复治疗的同时,剂量逐渐增加。6周后,他的改良Ashworth量表评分改善至1 - 2/4级,已进展到能够独立转移,大多数日常生活活动能够自理,并且在极少的支持下即可出院回家。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e5ef/11334347/cef00d39d571/JRMCC-7-25912-g001.jpg

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