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一名中年男性的急性比克斯特法夫脑干脑炎:诊断挑战与治疗反应

Acute Bickerstaff Brainstem Encephalitis in a Middle-Aged Male: Diagnostic Challenges and Therapeutic Responses.

作者信息

Bawwab Ameed, Snoubar Issa, Hajjeh Orabi, Khatib Lana, Aiti Danny

机构信息

Department of Internal Medicine, Cleveland Clinic Mercy Hospital, Canton, USA.

Department of Internal Medicine, Cleveland Medical Center, University Hospitals, Cleveland, USA.

出版信息

Cureus. 2025 Jul 15;17(7):e87964. doi: 10.7759/cureus.87964. eCollection 2025 Jul.

Abstract

Bickerstaff brainstem encephalitis (BBE) is a rare, post-infectious autoimmune disorder characterized by ophthalmoplegia, ataxia, and altered consciousness. It shares overlapping features with Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS), and diagnosis is often clinical, supported by anti-GQ1b antibodies. A 40-year-old previously healthy male presented with dizziness, diplopia, gait instability, and a recent viral prodrome. Neurologic examination revealed bilateral sixth cranial nerve (CN VI) palsy, partial involvement of the third cranial nerve (CN III), and bilateral dysmetria. Brain Imaging was unremarkable. Cerebrospinal fluid (CSF) analysis revealed lymphocytic pleocytosis, and the infectious workup was negative. Serum Anti-GQ1b antibody levels were elevated. The patient was treated with intravenous immunoglobulin (IVIG), resulting in marked improvement over four days; however, mild deficits persisted at the time of discharge to a rehabilitation facility. BBE remains a diagnostic challenge due to its rarity and nonspecific findings. Brain magnetic resonance imaging (MRI) may be normal, and anti-GQ1b antibodies, though not always present, are valuable for diagnosis. Prompt treatment with IVIG improves prognosis. This case exemplifies the clinical overlap within the Fisher-Bickerstaff spectrum and the importance of early recognition. Early identification of BBE is crucial for timely treatment and improved outcomes. This case underscores the need to consider autoimmune encephalitis in patients with acute brainstem symptoms and recent viral illness, even when imaging is normal.

摘要

比克斯特费尔德脑干脑炎(BBE)是一种罕见的感染后自身免疫性疾病,其特征为眼肌麻痹、共济失调和意识改变。它与吉兰-巴雷综合征(GBS)和米勒-费希尔综合征(MFS)有重叠特征,诊断通常基于临床,并由抗GQ1b抗体支持。一名40岁既往健康男性出现头晕、复视、步态不稳,近期有病毒前驱症状。神经系统检查发现双侧第六颅神经(CN VI)麻痹、第三颅神经(CN III)部分受累以及双侧辨距不良。脑部影像学检查无异常。脑脊液(CSF)分析显示淋巴细胞增多,感染相关检查结果为阴性。血清抗GQ1b抗体水平升高。患者接受静脉注射免疫球蛋白(IVIG)治疗,4天内有明显改善;然而,出院前往康复机构时仍有轻度功能缺损。由于其罕见性和非特异性表现,BBE仍然是一个诊断难题。脑磁共振成像(MRI)可能正常,抗GQ1b抗体虽然并非总是存在,但对诊断有重要价值。及时使用IVIG治疗可改善预后。该病例体现了费希尔-比克斯特费尔德谱系内的临床重叠以及早期识别的重要性。早期识别BBE对于及时治疗和改善预后至关重要。该病例强调,对于有急性脑干症状和近期病毒感染病史的患者,即使影像学检查正常,也需要考虑自身免疫性脑炎。

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