Shimizu Y, Takeuchi M, Ota K, Hara Y, Iwata M
Department of Neurology, Tokyo Women's Medical College.
Rinsho Shinkeigaku. 1997 Nov;37(11):976-81.
We presented a case of acute inflammatory demyelinating polyneuropathy associated with autoimmune chronic active hepatitis (AI-CAH). This is the third case report of neuropathy in AI-CAH. A 64-year-old male with chronic liver dysfunction was admitted to the hospital because of high fever, distal weakness and sensory disturbance of all extremities, bilateral facial weakness and dysphagia. On neurologic examination, there was bilateral weakness of the upper and lower facial muscles, bulbar palsy and severe distal weakness of all extremities. The deep tendon reflexes were absent and the sensation of touch, pinprick, temperature, and vibration was impaired bilaterally symmetrically in all extremities. Serum biochemistry revealed hyperproteinemia, hypergammaglobulinemia and elevated liver enzymes. Rheumatoid factor, antinuclear antibody anti-smooth muscle antibody were positive. Serological tests for hepatitis B surface antigen and its antibody hepatitis B core antibody, and hepatitis C antibody were all negative. Serum anti-GM1, anti-GD1b, anti-GQ1b and anti-MAG antibodies were negative. Liver biopsy findings were consistent with AI-CAH with marked lymphocytic infiltration in the portal tracts. Albuminocytologic dissociation was noted in CSF. Motorconduction velocity of the median, ulnar and facial nerves were markedly reduced with temporal dispersion. No motor response was evoked in the lower extremities. Needle electromyography revealed denervation and reinnervation potentials in the arm and leg. The sural nerve biopsy showed segmental de- and re-myelination and deposition of IgG components in endoneurium. Neurological symptoms and liver dysfunction improved with corticosteroid treatment. In this case, hypergammaglobulinemia associated with an exacerbation of AI-CAH may be responsible for the acute inflammatory demyelinating neuropathy through an unknown autoimmune mechanism.
我们报告了一例与自身免疫性慢性活动性肝炎（AI-CAH）相关的急性炎性脱髓鞘性多发性神经病。这是AI-CAH中神经病的第三例病例报告。一名64岁患有慢性肝功能障碍的男性因高热、四肢远端无力和感觉障碍、双侧面部无力及吞咽困难入院。神经系统检查发现双侧上下面部肌肉无力、延髓麻痹以及四肢严重远端无力。所有肢体的深腱反射消失，双侧对称的触觉、针刺觉、温度觉和振动觉均受损。血清生化检查显示高蛋白血症、高球蛋白血症及肝酶升高。类风湿因子、抗核抗体、抗平滑肌抗体均呈阳性。乙肝表面抗原及其抗体、乙肝核心抗体以及丙肝抗体的血清学检测均为阴性。血清抗GM1、抗GD1b、抗GQ1b和抗MAG抗体均为阴性。肝活检结果与AI-CAH相符，门脉区有明显淋巴细胞浸润。脑脊液中发现蛋白细胞分离。正中神经、尺神经和面部神经的运动传导速度明显降低并伴有时间离散。下肢未引出运动反应。针极肌电图显示手臂和腿部有失神经和再支配电位。腓肠神经活检显示节段性脱髓鞘和再髓鞘形成以及IgG成分在内膜沉积。经皮质类固醇治疗后神经症状和肝功能障碍有所改善。在该病例中，与AI-CAH加重相关的高球蛋白血症可能通过未知的自身免疫机制导致急性炎性脱髓鞘性神经病。
