Department of Neurology, Wakayama Medical University, 811-1 Kimiidera, Wakayama, Wakayama 641-8510, Japan.
J Clin Neurosci. 2013 Dec;20(12):1799-801. doi: 10.1016/j.jocn.2012.12.033. Epub 2013 Jul 31.
We report a patient with chronic inflammatory demyelinating polyneuropathy associated with primary biliary cirrhosis (PBC). Except for minimal biochemical abnormalities, clinical symptoms of PBC were not observed, and we diagnosed our patient with asymptomatic PBC from the results of a liver biopsy. Although the patient noticed little muscle weakness, an electrophysiological study demonstrated slow conduction velocities and prolonged distal latencies, with definite conduction blocks in the median, ulnar, and tibial nerves. The disturbed sensory pattern was asymmetrical, and sensory nerve action potentials were not evoked. From these observations, we diagnosed this patient with chronic inflammatory demyelinating polyneuropathy. Neuropathy associated with PBC is very rare. We must differentiate demyelinating neuropathy with PBC in patients with asymmetrical sensory dominant neuropathy with high immunoglobulin M titers, and investigate for the presence of anti-mitochondrial antibodies to rule out a complication of asymptomatic PBC.
我们报告了一例伴有原发性胆汁性肝硬化（PBC）的慢性炎症性脱髓鞘性多发性神经病患者。除了轻微的生化异常外，未观察到 PBC 的临床症状，我们根据肝活检结果诊断患者为无症状 PBC。尽管患者注意到肌肉无力，但电生理研究显示正中神经、尺神经和胫神经的传导速度缓慢，远端潜伏期延长，并存在明确的传导阻滞。感觉模式紊乱呈不对称性，感觉神经动作电位无法引出。根据这些观察结果，我们诊断该患者患有慢性炎症性脱髓鞘性多发性神经病。与 PBC 相关的神经病非常罕见。我们必须在具有高免疫球蛋白 M 滴度的不对称感觉优势神经病患者中鉴别 PBC 相关脱髓鞘神经病，并检查抗线粒体抗体以排除无症状 PBC 的并发症。
