Yamaji Kenji, Atsumi Masahiko, Saigoh Kazumasa, Kuzumoto Yoshimasa, Sada Masami, Hirakawa Minako, Morita Daiji, Mitsui Yoshiyuki
Department of Neurology, Kinki University of Medicine, Osaka, Japan.
No To Shinkei. 2005 Jun;57(6):523-6.
A patient with acute oropharyngeal palsy associated with internal ophthalmoplegia was reported. A 13-year-old boy had fever and diarrhea for two days. Ten days after resolution of these symptoms, he noticed difficulty in speaking (day 1). Neurological findings on day 4 included bilateral mydriasis, right abducens nerve palsy, nasal voice with absent pharyngeal reflex. Although superficial sensation was preserved, vibratory sensation was reduced in distal limbs. Tendon reflexes were generally absent. Neither ataxia nor dysautonomia was seen. Serum anti-glycolipid antibody assay on day 4 disclosed elevated IgG antibodies to GQ1b and GT1a. His cerebrospinal fluid on day 21 contained 6 mononuclear cells/microl with 137 mg/dl of total protein. Nerve conduction study on day 5 showed minimal sensory nerve involvement. Quantitative sudomotor axon reflex test was normal in the lower extremities. Low dose pilocarpine eyedrops dilated his pupils. Although mild cerebellar-like ataxia appeared on day 5, intravenous immunoglobulin (0.4 g/kg/day for four days) rapidly improved his neurological abnormalities. IgG anti-GQ1b antibody might contribute not only oropharyngeal weakness but also internal ophthalmoplegia in this patient.
报告了一名患有急性口咽麻痹伴内眼肌麻痹的患者。一名13岁男孩有两天的发热和腹泻症状。这些症状缓解十天后，他注意到说话困难（第1天）。第4天的神经系统检查结果包括双侧瞳孔散大、右侧展神经麻痹、鼻音且咽反射消失。虽然浅感觉保留，但远端肢体的振动觉减退。腱反射通常消失。未发现共济失调或自主神经功能障碍。第4天的血清抗糖脂抗体检测显示抗GQ1b和GT1a的IgG抗体升高。第21天他的脑脊液中每微升含有6个单核细胞，总蛋白为137毫克/分升。第5天的神经传导研究显示感觉神经受累轻微。下肢定量汗腺轴突反射试验正常。低剂量毛果芸香碱滴眼液使他的瞳孔散大。虽然第5天出现了轻度小脑样共济失调，但静脉注射免疫球蛋白（0.4克/千克/天，共四天）迅速改善了他的神经异常。IgG抗GQ1b抗体可能不仅导致该患者的口咽肌无力，还导致内眼肌麻痹。
