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

Mult Scler. 2022 Mar;28(3):453-462. doi: 10.1177/13524585211028126. Epub 2021 Oct 1.


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.

Keywords: Combined central and peripheral demyelination; antibodies; inflammatory demyelinating disease; myelin-associated glycoprotein; neurofascin.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies*
  • Demyelinating Diseases* / drug therapy
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Prognosis
  • Rituximab


  • Autoantibodies
  • Immunoglobulins, Intravenous
  • Rituximab