Bickerstaff’s脑干脑炎8例并文献复习

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。

Bickerstaff Brainstem Encephalitis: 8 Cases and Review of Literature

To investigate the clinical features of Bickerstaff brainstem encephalitis (BBE)improving disease awareness and standards of diagnosis and treatment. Methods: Clinical data of 8 BBEpatients were collected and retrospectively analyzed. Relevant literature was reviewed to summarize clinicalfeatures, 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 zostervirus infection. In 3 cases, the initial symptom was numbness and weakness of the limbs with disorganizedspeech. There was 1 case each of unclear consciousness, weakness of both lower limbs, hoarseness withdysphagia, numbness of face and fingers, and dizziness with unsteady gait. The main clinical manifestations wereas 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 2patients, head MRI examination showed no characteristic manifestations in 4 patients, abnormal lesions incorresponding parts of the brainstem were found in 2 patients, and extensive dural enhancement was seen in 1patient. All 5 cases that underwent routine serum GQ1b antibody testing were negative. All 8 patients weretreated 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 clinicalmanifestations, and serum antibodies, cerebrospinal fluid pathogen genome, imaging, andneuroelectrophysiology serve in auxiliary diagnosis. Immunoglobulin or combined glucocorticoid therapy iseffective treatment.