Nagakane Y, Ijichi T, Akiyama K, Mori S, Shimamura O, Nakajima K
Department of Neurology, Kyoto Prefectural University of Medicine.
Rinsho Shinkeigaku. 2000 Jun;40(6):591-5.
A 75-year-old man developed subacute progressive muscle weakness and painful paresthesia of the left upper and right lower limbs. The patient had no history of diabetes mellitus. On physical examination, there was no evidence of icterus or hepatosplenomegaly. Palmar erythema without rash was noted. Neurologic examination revealed muscle atrophy and weakness in the left upper limb and mild muscle weakness in the right proximal lower limb. Dysesthesia, severe hypesthesia, and hypalgesia were found in the left upper limb. The tendon reflexes were decreased in the left upper limb and absent in the lower limbs. The cranial nerves were preserved on the day of admission, followed by the involvement of the right oculomotor nerve. Serological examination revealed a mixed IgG/IgM cryoglobulinemia and hepatitis C virus (HCV) infection with evidence of HCV virus replication by PCR for HCV RNA. The patient was diagnosed as having a mixed cryoglobulinemic neuropathy associated with HCV infection. Interferon-alpha therapy with 3 million units subcutaneously was initiated three times per week; however, there was no clinical improvement, although cryoglobulins became undetectable and the level of serum HCV RNA decreased remarkably. Intravenous immunoglobulin therapy 20 g per day for 5 days was also ineffective. The patient developed right facial nerve palsy, followed by right abducens nerve palsy. Treatment with prednisolone 40 mg per day improved and stabilized neurologic symptoms. Although interferon-alpha is considered to be a promising therapy for neurologic complications of HCV infection with mixed cryoglobulinemia, the optimal treatment remains unestablished.
一名75岁男性出现亚急性进行性肌肉无力以及左上肢和右下肢疼痛性感觉异常。该患者无糖尿病病史。体格检查时,未发现黄疸或肝脾肿大的迹象。注意到有手掌红斑但无皮疹。神经系统检查发现左上肢肌肉萎缩和无力,右下肢近端轻度肌肉无力。左上肢存在感觉障碍、严重感觉减退和痛觉减退。左上肢腱反射减弱,下肢腱反射消失。入院当天颅神经未受累,随后右侧动眼神经受累。血清学检查显示混合性IgG/IgM冷球蛋白血症以及丙型肝炎病毒(HCV)感染,通过HCV RNA的PCR检测有HCV病毒复制的证据。该患者被诊断为与HCV感染相关的混合性冷球蛋白血症性神经病。开始每周3次皮下注射300万单位的α干扰素治疗;然而,尽管冷球蛋白检测不到且血清HCV RNA水平显著下降,但临床症状并无改善。每天静脉注射20 g免疫球蛋白,共5天,也无效。患者出现右侧面神经麻痹,随后出现右侧展神经麻痹。每天使用40 mg泼尼松龙治疗使神经症状得到改善并稳定。尽管α干扰素被认为是治疗HCV感染合并混合性冷球蛋白血症神经并发症的一种有前景的疗法,但最佳治疗方案仍未确定。