Myelin oligodendrocyte glycoprotein-IgG-positive, steroid-responsive combined central and peripheral demyelination with
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Myelin oligodendrocyte glycoprotein-IgG-positive, steroid-responsive combined central and peripheral demyelination with recurrent peripheral neuropathy Takaaki Nakamura 1 & Kimihiko Kaneko 2,3 & Genya Watanabe 1 & Shogo Harashima 1 & Emiko Kawasaki 1 & Kenichi Tsukita 1 & Toshiyuki Takahashi 2,4 & Ichiro Nakashima 5 & Tatsuro Misu 2 & Yasushi Suzuki 1 Received: 15 May 2020 / Accepted: 10 October 2020 # Fondazione Società Italiana di Neurologia 2020
Abstract Myelin oligodendrocyte glycoprotein (MOG)-IgG detected by the cell-based assay is associated with demyelinating diseases of the central nervous system, such as optic neuritis, myelitis, and acute disseminated encephalomyelitis, but rarely with peripheral neuropathy. Here, we describe the case of a 32-year-old MOG-IgG+ woman who developed central and peripheral demyelinating lesions. In contrast to previous similar cases, she uniquely presented with repeated subsequent relapses in the peripheral nerve, mimicking chronic inflammatory demyelinating polyneuropathy. Possible pathogenic implications of MOG-IgG in combined central and peripheral nervous system diseases are considered. Keywords Combined central and peripheral demyelination . Delivery . Myelin oligodendrocyte glycoprotein . Myelitis . Radiculitis . Relapse
Background Myelin oligodendrocyte glycoprotein (MOG) is a structural CNS protein localized at the outermost layers of myelin sheath. Recently, a cell-based assay was developed to detect antibodies against the conformational extracellular epitopes in patients with CNS demyelinating diseases, such as acute disseminated encephalomyelitis, optic neuritis, seronegative neuromyelitis optica spectrum disorders, and cortical encephalitis
* Takaaki Nakamura [email protected] 1
Department of Neurology, National Hospital Organization Sendai Medical Center, Sendai, Miyagi, Japan
2
Department of Neurology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan
3
Department of Neurology, National Hospital Organization Miyagi National Hospital, Watari, Miyagi, Japan
4
Department of Neurology, National Hospital Organization Yonezawa National Hospital, Yonezawa, Yamagata, Japan
5
Department of Neurology, Tohoku Medical and Pharmaceutical University, Sendai, Miyagi, Japan
[1]. However, three recent cases of MOG-IgG+-associated diseases were possibly associated with peripheral neuropathy in addition to CNS demyelinating lesions [2–4]. Here, we describe a case of a young MOG-IgG+ woman whose symptoms fulfilled the proposed criteria of combined central and peripheral demyelination (CCPD) [5]. Her unique clinical course included repeated relapses in the peripheral nerve, thus fulfilling the criteria for chronic inflammatory demyelinating polyneuropathy (CIDP) in addition to CCPD.
Patient description A 32-year-old healthy woman, without preceding infection or vaccination, noticed gradually worsening numbness in both hands 3 months after normal delivery of the second baby. One month later, she also noticed numbness in bo
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