Bickerstaff’s 脑干脑炎8 例并文献复习

陈彬;脱厚珍

神经损伤与功能重建 ›› 2019, Vol. 14 ›› Issue (6) : 288-291.

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神经损伤与功能重建 ›› 2019, Vol. 14 ›› Issue (6) : 288-291.
论著

Bickerstaff’s 脑干脑炎8 例并文献复习

  • 陈彬,脱厚珍
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Bickerstaff Brainstem Encephalitis: 8 Cases and Review of Literature

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摘要

目的:分析Bickerstaff 脑干脑炎(BBE)的临床特点,以提高临床诊疗水平。方法:对8 例BBE患者的 临床资料进行回顾性分析,并结合相关文献,总结其临床特点、发病机制、治疗方法及预后。结果:8 例患者 均为急性起病,发病前有上呼吸道感染者2 例,腹泻2 例,水痘-带状疱疹病毒感染1 例。首发症状为肢体麻 木无力伴言语含混3 例;意识不清、双下肢无力、声音嘶哑伴吞咽困难、面部和手指麻木、头晕伴行走不稳的 各1 例。临床主要表现为急性眼肌麻痹(眼外肌麻痹7 例,眼内肌麻痹1 例),共济失调4 例,意识障碍4 例,腱 反射减弱或消失5 例,病理征阳性8 例,累及面神经4 例,累及舌咽和迷走神经6 例,同时累及脑神经(面神 经、舌咽、迷走神经、舌下神经)3 例。2 例患者脑脊液检查正常,4 例头颅MRI检查无特征性表现,2 例在脑 干内相应部位有异常病灶,1 例硬脑膜广泛强化。5 例行血清抗GQ1b 抗体检查者均为阴性。8 例患者均使 用免疫球蛋白或联合糖皮质激素治疗,7 例患者预后较好,1 例患者死亡。结论:BBE多呈单相良性病程,确 诊主要依据临床表现、血清抗体、脑脊液病原基因组、影像学及神经电生理作为辅助诊断,免疫球蛋白或联 合糖皮质激素治疗有效。

Abstract

To investigate the clinical features of Bickerstaff brainstem encephalitis (BBE) improving disease awareness and standards of diagnosis and treatment. Methods: Clinical data of 8 BBE patients were collected and retrospectively analyzed. Relevant literature was reviewed to summarize clinical features, pathogenesis, treatment, and prognosis of the disease. Results: All 8 patients showed acute onset. Before onset of BBE, 2 cases displayed upper respiratory infection, 2 cases diarrhea, and 1 case varicella zoster virus infection. In 3 cases, the initial symptom was numbness and weakness of the limbs with disorganized speech. There was 1 case each of unclear consciousness, weakness of both lower limbs, hoarseness with dysphagia, numbness of face and fingers, and dizziness with unsteady gait. The main clinical manifestations were as follows: acute ophthalmoplegia (extraocular n=7, intraocular n=1), ataxia (n=4), impaired consciousness (n= 4), deep tendon hyporeflexia or areflexia (n=5), positive pathology (n=8), facial nerve involvement (n=4), glossopharyngeal and vagus nerve involvement (n=6), and simultaneous involvement of cranial nerves (facial, glossopharyngeal, vagus, and hypoglossal nerves, n=3). Cerebrospinal fluid examination was normal in 2 patients, head MRI examination showed no characteristic manifestations in 4 patients, abnormal lesions in corresponding parts of the brainstem were found in 2 patients, and extensive dural enhancement was seen in 1 patient. All 5 cases that underwent routine serum GQ1b antibody testing were negative. All 8 patients were treated with immunoglobulin or combined glucocorticoid; 7 patients had a good prognosis, and 1 patient died. Conclusion: BBE mostly presents a single-phase benign course. Diagnosis is mainly based on clinical manifestations, and serum antibodies, cerebrospinal fluid pathogen genome, imaging, and neuroelectrophysiology serve in auxiliary diagnosis. Immunoglobulin or combined glucocorticoid therapy is effective treatment.

关键词

Bickerstaff 脑干脑炎 / 吉兰-巴雷综合征 / Miller-Fisher综合征 / 临床特点 / 治疗

Key words

Bickerstaff brainstem encephalitis

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陈彬;脱厚珍. Bickerstaff’s 脑干脑炎8 例并文献复习[J]. 神经损伤与功能重建. 2019, 14(6): 288-291
Bickerstaff Brainstem Encephalitis: 8 Cases and Review of Literature[J]. Neural Injury and Functional Reconstruction. 2019, 14(6): 288-291

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