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はじめに
Bickerstaff型脳幹脳炎(Bickerstaff's brainstem encephalitis:BBE)は眼筋麻痺,小脳性運動失調に加え,意識障害,錘体路症状など多彩な症状を呈するものの,一般的に予後の良好な疾患と考えられている1,2)。現在,BBEは病因論的にGuillain-Barre症候群やFisher症候群と連続する疾患単位とみなされており1-3),IgG抗GQ1b抗体が陽性を示すことが診断に有用とされる4)。また,BBEは頭部MRIで異常像として描出されることは比較的稀(30%)である2)。
われわれは,頭部MRIで脳幹部に明瞭な異常信号を認め,BBEと診断した1例を経験したので報告する。
Abstract
We report a case of Bickerstaff's brainstem encephalitis (BBE) with positive findings on head MRI. Abnormal lesions on head MRI were detected in approximately 30% cases of BBE. An 81-year-old female presented with disturbance of consciousness (Japan Coma Scale 200), ophthalmoplegia (downward deviation) and tetraplegia. Cerebrospinal fluid examination revealed mild pleocytosis 244 cells in 3 μl of CSF with normal glycorrhachia. Head MRI showed high-intensity abnormalities extending over the brain stem. The patient was diagnosed with BBE. It was thought that her symptoms were believed to arise from the lesion in reticular formation, rostral interstitial nucleus of the medial longitudinal fasciculus, and the pyramidal tract. At discharged after 1 month, she had no sequela.
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