Grézis G, Tamion F, Lamia B, Girault C, Delangre T, Bonmarchand G
Service de réanimation médicale, CHU Charles-Nicolle, 1, rue de Germont, 76031 Rouen, France.
Rev Med Interne. 2005 Sep;26(9):748-50. doi: 10.1016/j.revmed.2005.05.016.
Bickerstaff's brainstem encephalitis represents a differential diagnostic that must be discuss with meningoencephalitis with ophtalmoplegia, ataxia and confusion.
A 26 year-old woman presented a Bickerstaff syndrome. A severe disturbance of consciousness had lead to admission in intensive care unit with mechanical ventilation. Electrophysiological tests and the brain magnetic resonance were normal. We have not observed systemic anti-G1Qb antibody in our patient. Nevertheless, 66% of patients with Bickerstaff syndrome have anti-GQ1b antibody during the acute phase period. We observed a beneficial effect of IV Ig treatment although no significant improvement was observed with corticosteroids.
Controlled clinical trials are needed to established the efficacy of IV Ig or plasmapheresis as a specific therapy for this pathology.
比克斯特费尔德脑干脑炎是一种鉴别诊断，必须与伴有眼肌麻痹、共济失调和意识模糊的脑膜脑炎进行讨论。
一名26岁女性患有比克斯特费尔德综合征。严重的意识障碍导致其入住重症监护病房并接受机械通气。电生理检查和脑部磁共振成像均正常。我们的患者未检测到系统性抗G1Qb抗体。然而，66%的比克斯特费尔德综合征患者在急性期有抗GQ1b抗体。我们观察到静脉注射免疫球蛋白治疗有有益效果，尽管使用皮质类固醇未观察到显著改善。
需要进行对照临床试验来确定静脉注射免疫球蛋白或血浆置换作为这种疾病的特异性治疗方法的疗效。
