与慢跳阅Neurochondrin自身免疫:一个案例报告

客观的N / A。

背景Neurochondrin细胞质神经元抗原,可以有针对性的特定的抗体,导致抗原特异性t细胞反应和自身免疫小脑和脑干变性。1Previously reported cases of neurochondrin antibody positivity have been predominantly associated with rapidly-progressive rhombencephalitis, but various other neurological symptoms and signs may be present.1,2

设计/方法N / A。

结果34岁的女人面对2年持续的在她的脚趾麻木,逐渐恶化涉及整个上肢。她还报道构音障碍、不平衡和腿痉挛状态。神经系统检查显示缓慢,wide-based步态、快反应双边、和双边伸肌足底的反应。MRI大脑是正常和CSF分析显示7寡克隆乐队。病人被诊断为多发性硬化症和类固醇可能发起的,但导致没有症状的改善。血浆置换也是无效的。3个月后,MRI显示中央线巧妙地增加T2信号主要包括灰质的C5、C6。CSF分析显示15寡克隆乐队和免疫球蛋白升高指数为1.39。测试是不反应的。AQP4 HTLV, ANCA, CSF VZ PCR, HSV PCR和HHV6 PCR都是负面的。 EMG showed mild length-dependent axonal sensory motor peripheral neuropathy. On neuro-ophthalmic examination, the patient showed slowed saccades with some preservation of optokinetic nystagmus (OKN) fast phases. Repeat MR showed abnormal T2 hyperintensity in the anterior horns of the gray matter from C1-2 to T1. Serum testing at that time showed a positive neurochondrin antibody. She was then started on cyclophosphamide and mycophenolate mofetil. 6 months after initiation of cyclophosphamide, dysarthria and upper limb mobility improved, and she had no further neurological deterioration at that time.

结论Neurochondrin IgG-related自身免疫是罕见的,可以导致多种神经系统症状和体征。眼蠕动障碍,包括跳阅放缓,可能存在。