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

• Published in: Multiple sclerosis Vol. 28; no. 3; p. 453
• Main Authors: Hou, Xiaodan, Liang, Yan, Cui, Pan, Hao, Junwei
• Format: Journal Article
• Language: English
• Published: England 01.03.2022
• Subjects: Autoantibodies; Demyelinating Diseases - drug therapy; Humans; Immunoglobulins, Intravenous - therapeutic use; Prognosis; Rituximab; inflammatory demyelinating disease; Combined central and peripheral demyelination; myelin-associated glycoprotein; neurofascin; antibodies
• ISSN: 1477-0970
• DOI: 10.1177/13524585211028126

Combined central and peripheral demyelination (CCPD) is a disease of inflammatory demyelination that affects central and peripheral nerves simultaneously or temporally separated. This study evaluated the clinical characteristics and the existence of antinodal/paranodal antibodies in patients with CCPD. 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. 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. The clinical manifestations of patients with CCPD are highly heterogeneous. We recommend testing antinodal/paranodal antibodies for patients with CCPD.