Department of Neurology and Clinical Neuroscience, Yamaguchi University Graduate School of Medicine, Minami-Kogushi 1-1-1, Ube, Yamaguchi 755-8505, Japan.
J Neurol Neurosurg Psychiatry. 2012 Dec;83(12):1210-5. doi: 10.1136/jnnp-2012-303060. Epub 2012 Jul 31.
Information regarding the epidemiological background of Bickerstaff brainstem encephalitis (BBE) is limited.
We conducted a nationwide survey of BBE in the Japanese population in two steps: the first aimed to identify patients with brainstem encephalitis for the specified 3 year period and the second to evaluate whether the clinical picture met our diagnostic criteria for BBE.
The number of patients with brainstem encephalitis was estimated as 704 (95% CI 478 to 930) over the 3 years. The annual onset of BBE was roughly estimated as 100 cases, which accounted for 43% of brainstem encephalitis. BBE was slightly male predominant and often young onset. Among brainstem encephalitis patients, BBE was characterised by antecedent infectious symptoms, oropharyngeal palsy and sensory disturbance at the distal extremities with absent or decreased tendon reflexes, in addition to a triad of symptoms (external ophthalmoplegia, ataxia and impaired level of consciousness) and shorter duration to the peak, with good outcome. Anti-GQ1b antibodies were present in 75% of cases. Several BBE patients with atypical clinical features or without anti-GQ1b antibodies were also identified. These cases often had marked CSF pleocytosis, abnormal brain MRI findings and a longer duration to peak symptoms, sometimes with considerable residual deficits.
BBE is a rare disorder but accounts for a major proportion of brainstem encephalitis. BBE consists of typical and atypical cases. Typical BBE has similar neurological and serological features to Fisher syndrome and shows good recovery whereas atypical BBE is characterised by delayed recovery, negative anti-GQ1b antibodies, and abnormal CSF and brain MRI findings with other possible pathogeneses.
关于 Bickerstaff 脑干脑炎（BBE）的流行病学背景信息有限。
我们分两步在日本人群中进行了 BBE 的全国性调查：第一步旨在确定指定 3 年期间的脑干脑炎患者，第二步评估临床图像是否符合我们的 BBE 诊断标准。
3 年内估计脑干脑炎患者人数为 704 例（95%CI 478 至 930）。BBE 的年发病数估计约为 100 例，占脑干脑炎的 43%。BBE 略为男性多发，且常为青年起病。在脑干脑炎患者中，BBE 的特征为前驱感染症状、口咽部瘫痪和远端肢体感觉障碍伴腱反射消失，此外还有三联征（外展神经麻痹、共济失调和意识水平下降）和较短的高峰病程，预后良好。75%的病例存在抗 GQ1b 抗体。还发现了一些具有不典型临床特征或无抗 GQ1b 抗体的 BBE 患者。这些病例常伴有明显的 CSF 细胞增多、异常的脑 MRI 表现和较长的高峰症状持续时间，有时伴有相当大的残留缺陷。
BBE 虽然罕见，但占脑干脑炎的很大比例。BBE 包括典型和不典型病例。典型 BBE 的神经和血清学特征与 Fisher 综合征相似，恢复良好，而不典型 BBE 的特征为恢复延迟、抗 GQ1b 抗体阴性、CSF 和脑 MRI 异常，可能有其他发病机制。
