The clinical features of combined central and peripheral demyelination and antibodies against the node of Ranvier

被引:9
作者
Hou, Xiaodan [1 ]
Liang, Yan [1 ,2 ]
Cui, Pan [1 ,2 ]
Hao, Junwei [1 ,2 ]
机构
[1] Capital Med Univ, Xuanwu Hosp, Dept Neurol, Changchun Ave 45, Beijing 100032, Peoples R China
[2] Tianjin Med Univ, Tianjin Neurol Inst, Dept Neurol, Gen Hosp, Tianjin, Peoples R China
基金
中国国家自然科学基金;
关键词
Combined central and peripheral demyelination; neurofascin; myelin-associated glycoprotein; inflammatory demyelinating disease; antibodies; NERVOUS-SYSTEM DEMYELINATION;
D O I
10.1177/13524585211028126
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Background: Combined central and peripheral demyelination (CCPD) is a disease of inflammatory demyelination that affects central and peripheral nerves simultaneously or temporally separated. Objectives: This study evaluated the clinical characteristics and the existence of antinodal/paranodal antibodies in patients with CCPD. Methods: We reviewed the clinical manifestations, laboratory tests, electrophysiological examinations, neuroimaging findings, treatment, and prognosis of 31 patients with CCPD. Using a live cell-based assay, we tested antinodal/paranodal antibodies. Results: The most common symptoms were motor weakness (83.3%), hyporeflexia (63.3%), and sphincter disturbance (58.1%). In total, 16.6% of patients had impaired vision symptoms, whereas 33.3% of patients had abnormal visual-evoked potentials (VEPs). A total of 21.1% (4/19) of patients were positive for anti-AQP4 (aquaporin 4) antibodies, 20.0% (2/10) of patients were positive for anti-NF155 (neurofascin-155) antibodies, and 10.0% (1/10) of patients were positive for anti-MAG (myelin-associated glycoprotein) antibodies. The effective rates of intravenous corticosteroids, intravenous immunoglobulins, and rituximab were 72.2%, 37.5%, and 100%, respectively. At the illness peak, 75% of patients with CCPD had an mRS (modified Rankin Scale) score of 4 or greater. In remission, 37.5% had an mRS score of 4 or greater. Conclusion: The clinical manifestations of patients with CCPD are highly heterogeneous. We recommend testing antinodal/paranodal antibodies for patients with CCPD.
引用
收藏
页码:453 / 462
页数:10
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