Giaccari Luca Gregorio, Mastria Donatella, Barbieri Rosella, De Maglio Rossella, Madaro Francesca, Paiano Gianfranco, Pace Maria Caterina, Sansone Pasquale, Pulito Giuseppe, Mascia Luciana
Department of Anesthesia and Intensive Care, "Vito Fazzi" Hospital, Lecce, Italy.
Department of Women, Child, General and Specialist Surgery, University of Campania "L. Vanvitelli", Naples, Italy.
Front Neurol. 2024 Mar 12;15:1387505. doi: 10.3389/fneur.2024.1387505. eCollection 2024.
Bickerstaff brainstem encephalitis (BBE) is a rare autoimmune disease characterized by the subacute onset of bilateral external ophthalmoplegia, ataxia, and decreased level of consciousness. BBE is part of a group of rare autoimmune diseases in children that can affect the nervous system at any level. The onset of neurological deficits is often sudden and nonspecific. The diagnosis is based on clinical findings and abnormal findings on cerebrospinal fluid (CSF), electroencephalography (EEG), electromyography (EMG), and magnetic resonance imaging (MRI). BBE is associated with the presence of the antiganglioside antibody, anti-GQ1b and anti-GM1. Intravenous immunoglobulin (IVIg) and plasma exchange are often used as treatments for these patients. We conducted a review on clinical presentation, diagnosis, treatment and outcome of reported cases of BBE. 74 cases are reported in the literature from the first cases described in 1951 to today. The prevalence is unknown while the incidence is higher in males. In 50% of cases, BBE occurs following respiratory or gastrointestinal tract infections. The most frequent initial symptoms were consciousness disturbance, headache, vomiting, diplopia, gait disturbance, dysarthria and fever. During illness course, almost all the patients developed consciousness disturbance, external ophthalmoplegia, and ataxia. Lumbar puncture showed pleocytosis or cytoalbuminological dissociation. Abnormal EEG and MRI studies revealed abnormalities in most cases. Anti-GQ1b antibodies were detected in more than half of the patients; anti-GM1 antibodies were detected in almost 40% of patients. Treatment guidelines are missing. In our analysis, steroids and IVIg were administered alone or in combination; as last option, plasmapheresis was used. BBE has a good prognosis and recovery in childhood is faster than in adulthood; 70% of patients reported no sequelae in our analysis. Future studies need to investigate pathogenesis and possible triggers, and therapeutic possibilities.
比克斯特费德脑干脑炎（BBE）是一种罕见的自身免疫性疾病，其特征为双侧外展神经麻痹、共济失调和意识水平下降的亚急性发作。BBE是一组可影响儿童神经系统任何水平的罕见自身免疫性疾病的一部分。神经功能缺损的发作通常是突然且非特异性的。诊断基于临床表现以及脑脊液（CSF）、脑电图（EEG）、肌电图（EMG）和磁共振成像（MRI）的异常发现。BBE与抗神经节苷脂抗体、抗GQ1b和抗GM1的存在有关。静脉注射免疫球蛋白（IVIg）和血浆置换常被用作这些患者的治疗方法。我们对已报道的BBE病例的临床表现、诊断、治疗和结局进行了综述。从1951年描述的首例病例到如今，文献中共报道了74例。患病率未知，而男性发病率更高。在50%的病例中，BBE发生在呼吸道或胃肠道感染之后。最常见的初始症状为意识障碍、头痛、呕吐、复视、步态障碍、构音障碍和发热。在病程中，几乎所有患者都会出现意识障碍、外展神经麻痹和共济失调。腰椎穿刺显示细胞增多或蛋白细胞分离。异常的EEG和MRI研究在大多数病例中显示出异常。超过一半的患者检测到抗GQ1b抗体；近40%的患者检测到抗GM1抗体。缺乏治疗指南。在我们的分析中，单独或联合使用了类固醇和IVIg；作为最后的选择，使用了血浆置换。BBE预后良好，儿童期的恢复比成年期更快；在我们的分析中，70%的患者报告无后遗症。未来的研究需要调查发病机制和可能的触发因素以及治疗可能性。
