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一名以共济失调为表现的Bickerstaff脑干脑炎患者的康复治疗

Rehabilitation of a Patient With Bickerstaff Brainstem Encephalitis Presenting With Ataxia.

作者信息

Uno Isao

机构信息

Rehabilitation Medicine, Sakura Juji Fukuoka Hospital, Fukuoka, JPN.

出版信息

Cureus. 2025 Mar 20;17(3):e80864. doi: 10.7759/cureus.80864. eCollection 2025 Mar.

Abstract

Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune disorder characterized by oculomotor disturbances, ataxia, and impaired consciousness, likely caused by brainstem inflammation. Although acute immunotherapy, including corticosteroids, plasma exchange, and intravenous immunoglobulin, appears to effectively mitigate the inflammatory process, residual neurological deficits often persist, necessitating individualized rehabilitation strategies.  We report the case of a 40-year-old male with BBE who presented with severe impaired consciousness, ataxia, and diplopia following flu-like symptoms. Despite initial immunotherapeutic interventions, the patient continued to experience significant functional limitations, as evidenced by a Functional Independence Measure (FIM) score of 78, a Berg Balance Scale (BBS) score of 22, and a Scale for the Assessment and Rating of Ataxia (SARA) score of 20 upon admission to the rehabilitation ward. An intensive, stepwise, and individualized rehabilitation program was implemented. The protocol initially emphasized static and dynamic balance exercises on stable surfaces, trunk and limb strengthening, and environmental modifications to address diplopia. From the second week, task-specific gait training, including applied stepping, stair climbing, and oculomotor exercises, was introduced. After the third week, the regimen progressed to outdoor ambulation and activities simulating real-life tasks to facilitate social reintegration and occupational readiness. Following 41 days of rehabilitation, marked improvements were observed. The patient's FIM score increased from 78 to 121, BBS improved from 22 to 56, and SARA score decreased from 20 to 3, indicating significant amelioration of ataxia. Enhanced performance on the Timed Up and Go (TUG) test and the 10-meter walk test, as well as an increased 6-minute walking distance, reflected improved balance, mobility, and endurance. Additionally, the patient's diplopia improved, enabling safe ambulation and the successful resumption of leisure activities. This case underscores the pivotal role of an individualized, timely rehabilitation program in facilitating functional recovery in patients with BBE. Task-specific training and sensorimotor integration strategies appear to promote neural plasticity and improve motor and oculomotor functions. Further research is warranted to develop and validate evidence-based rehabilitation protocols tailored to BBE and similar autoimmune neurological disorders.

摘要

比克斯特费尔德脑干脑炎(BBE)是一种罕见的自身免疫性疾病,其特征为眼球运动障碍、共济失调和意识障碍,可能由脑干炎症引起。尽管包括皮质类固醇、血浆置换和静脉注射免疫球蛋白在内的急性免疫疗法似乎能有效减轻炎症过程,但残留的神经功能缺损往往持续存在,因此需要个性化的康复策略。我们报告一例40岁男性BBE患者,其在出现类似流感症状后出现严重意识障碍、共济失调和复视。尽管进行了初始免疫治疗干预,但患者仍存在明显的功能受限,康复病房入院时功能独立性测量(FIM)评分为78分、伯格平衡量表(BBS)评分为22分、共济失调评估与分级量表(SARA)评分为20分,即为证明。实施了强化、逐步且个性化的康复计划。该方案最初强调在稳定表面上进行静态和动态平衡练习、躯干和肢体强化训练以及针对复视的环境调整。从第二周开始,引入了特定任务的步态训练,包括应用迈步、爬楼梯和眼球运动练习。第三周后,治疗方案进展到户外行走和模拟现实生活任务的活动,以促进社会重新融入和职业准备。经过41天的康复治疗,观察到明显改善。患者的FIM评分从78分提高到121分,BBS从22分提高到56分,SARA评分从20分降至3分,表明共济失调明显改善。定时起立行走(TUG)测试和10米步行测试表现增强,以及6分钟步行距离增加,反映了平衡、移动能力和耐力的改善。此外,患者的复视有所改善,能够安全行走并成功恢复休闲活动。该病例强调了个性化、及时的康复计划在促进BBE患者功能恢复中的关键作用。特定任务训练和感觉运动整合策略似乎能促进神经可塑性并改善运动和眼球运动功能。有必要进行进一步研究,以制定和验证针对BBE及类似自身免疫性神经系统疾病的循证康复方案。

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