Shimozono Koji, Shimono Kenshin, Kusunoki Susumu
Department of Internal Medicine, Ootemachi Hospital.
Rinsho Shinkeigaku. 2012;52(9):656-9. doi: 10.5692/clinicalneurol.52.656.
A 25-years-old man experienced fever and diarrhea. Ten days later he noticed difficulty walking (day 1). On admission neurological examination revealed lethargy, dysarthria and weakness of limbs. Oculocephalic response was not be elicited and extensor toe signs were positive. In spite of treatment with aciclovir and methylprednisolone, he continued to show progressive deterioration developing to coma with decorticate posture. Autonomic symptoms (hyperhidrosis, hypersalivation and fever) and groaning were observed. Brain magnetic resonance image and brainstem evoked potential presented no abnormality, but electroencephalographic study showed a spindle pattern indicating spindle coma. Laboratory tests including cerebrospinal fluids showed no specific results. High-dose immunoglobulin was administered from day 6, and his consciousness level improved. External ophthalomoplegia and ataxic gait were observed after he became more alert. Because he had IgG type anti-GQ1b antibodies in the serum, a diagnosis was made of Bickerstaff's brainstem encephalitis (BBE). Six months after discharge he had complete resolution of his symptoms. This is the first report of spindle coma observed in a case of serologically confirmed BBE.
一名25岁男性出现发热和腹泻。10天后，他开始出现行走困难（第1天）。入院时神经学检查发现嗜睡、构音障碍和肢体无力。无法引出眼前庭反射，巴宾斯基征阳性。尽管接受了阿昔洛韦和甲泼尼龙治疗，但他仍持续病情恶化，发展为去皮质姿势昏迷。观察到自主神经症状（多汗、流涎和发热）及呻吟。脑磁共振成像和脑干诱发电位未见异常，但脑电图显示纺锤波模式，提示纺锤波昏迷。包括脑脊液在内的实验室检查未得出特异性结果。从第6天开始给予大剂量免疫球蛋白治疗，他的意识水平有所改善。在他意识更清醒后，观察到眼球外展麻痹和共济失调步态。由于他血清中存在IgG型抗GQ1b抗体，诊断为比克斯特法夫脑干脑炎（BBE）。出院6个月后，他的症状完全消失。这是血清学确诊的BBE病例中观察到纺锤波昏迷的首例报告。
