Rehabilitation of a Patient With Bickerstaff Brainstem Encephalitis Presenting With Ataxia

一名患有比克斯塔夫脑干脑炎并伴有共济失调的患者的康复治疗

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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.

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