Neuromyelitis optica spectrum disorders (NMOSD) is currently recognised as an inflammatory central nervous system (CNS) disorder that most frequently involves the spinal cord and optic nerve. Many studies have provided sufficient evidence for the importance of the AQP4 antibody in the pathogenesis of NMOSD [
- Jarius S.
- Aboul-Enein F.
- Waters P.
- et al.
Antibody to aquaporin-4 in the long-term course of neuromyelitis optica.
Brain. 2008; 131: 3072-3080
]. In addition, other autoantibodies such as the AQP-1 and myelin oligodendrocyte glycoprotein (MOG) antibodies have also been found in NMOSD patients and have significant effects in the immune process of the disease [
- Papadopoulos M.C.
- Verkman A.S.
Aquaporin 4 and neuromyelitis optica.
Lancet Neurol. 2012; 11: 535-544
]. Neurofascin-155 (NF155) is a protein expressed in both CNS and peripheral nervous system (PNS) myelin sheath. Studies have shown that the NF155 antibody plays an important role in chronic inflammatory demyelinating polyradiculopathy (CIDP) and combined central and peripheral demyelination disease [
- Jasiak-Zatonska M.
- Kalinowska-Lyszczarz A.
- Michalak S.
- Kozubski W.
The immunology of neuromyelitis optica-current knowledge, clinical implications, controversies and future perspectives.
Int. J. Mol. Sci. 2016; 17: 273
- Querol L.
- Nogales-Gadea G.
- Rojas-Garcia R.
- et al.
Neurofascin IgG4 antibodies in CIDP associate with disabling tremor and poor response to IVIg.
Neurology. 2014; 82: 879-886
]. In contrast, there have so far been no previous reports of concurrent NF155 and AQP4 antibody involvement in NMOSD. We herein present the cases of two patients with seropositive NMOSD, who carried of the NF155 antibody, which are mainly IgG1 and IgG2 subclasses.
- Kawamura N.
- Yamasaki R.
- Yonekawa T.
- et al.
Anti-neurofascin antibody in patients with combined central and peripheral demyelination.
Neurology. 2013; 81: 714-722
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- Antibody to aquaporin-4 in the long-term course of neuromyelitis optica.Brain. 2008; 131: 3072-3080
- Aquaporin 4 and neuromyelitis optica.Lancet Neurol. 2012; 11: 535-544
- The immunology of neuromyelitis optica-current knowledge, clinical implications, controversies and future perspectives.Int. J. Mol. Sci. 2016; 17: 273
- Neurofascin IgG4 antibodies in CIDP associate with disabling tremor and poor response to IVIg.Neurology. 2014; 82: 879-886
- Anti-neurofascin antibody in patients with combined central and peripheral demyelination.Neurology. 2013; 81: 714-722
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- Diagnosis of Sjogren's syndrome: American-European and the American College of Rheumatology classification criteria.Oral Maxillofac. Surg. Clin. North Am. 2014; 26: 13-22
- Disruption of neurofascin localization reveals early changes preceding demyelination and remyelination in multiple sclerosis.Brain. 2006; 129: 3173-3185
- Reduced raft-association of NF155 in active MS-lesions is accompanied by the disruption of the paranodal junction.Glia. 2007; 55: 885-895
- Characterization of IgG4 anti-neurofascin 155 antibody-positive polyneuropathy.Ann. Clin. Transl. Neurol. 2015; 2: 960-971
Published online: January 16, 2019
Accepted: January 15, 2019
Received in revised form: January 11, 2019
Received: November 12, 2018
© 2019 Elsevier B.V. All rights reserved.