Peripheral neuropathy and MOG-IgG: A clinical and neuropathological retrospective study

Abstract

Background: Myelin oligodendrocyte glycoprotein antibodies (MOG-Abs) may rarely be associated with peripheral nervous system involvement. We aimed to test MOG-Abs in patients with undetermined peripheral neuropathy (PN).

Methods: Consecutive patients with available sural nerve biopsy and paired serum sample were retrospectively identified (January, 1st 2016-November, 1st 2021) and tested for MOG-Abs with live cell-based assay (CBA). Patients with antibody titre ≥1:160 (secondary H + L antibody) and selective MOG-IgG presence (IgG-Fc predominance) were considered MOG-IgG positive. All positive samples were analysed with immunohistochemistry and CBAs for antibodies against Neurofascin-155 and Contactin-1. Clinical and neuropathological data were collected through clinical reports.

Results: Among 163 patients, 5 (3%) resulted positive for predominantly IgG MOG-Abs (median titer 1:320, range 1:160-1:5120), none showed other concomitant antibodies. Median age was 74 years-old (range 55-81), median disease duration was 60 months (range 1-167), 60% of patients were female. Of these, 4/5 cases had clinical features suggestive of acute (n = 1) or chronic (n = 3) inflammatory demyelinating neuropathy, 2/5 fulfilled the criteria of combined central and peripheral demyelination (CCPD) whilst 3/5 had isolated PNS involvement. Neuropathological findings showed mixed axonal-demyelinating features in 2/5, predominant demyelination in 3/5 cases. Other neuropathological hallmarks included paranodal demyelination (n = 3), myelin outfoldings (n = 4), slight inflammatory infiltrates (n = 3), onion bulbs (n = 3), and clusters of regeneration (n = 4).

Discussion: MOG-IgG can be detected in patients with isolated PN or CCPD. Clinical and neuropathological features are suggestive for demyelination and slight inflammation. Further studies should include larger cohorts of patients to elucidate the utility of MOG-Abs testing in PN.

Keywords: Chronic inflammatory demyelinating polyneuropathy (CIDP); Myelin oligodendrocyte glycoprotein related disease (MOGAD); Nerve biopsy; Neuropathy.