Clinical characteristics and response to treatment of patients with MOGAD: a multicenter case series

Authors: Hayet Boudjani, Giulia Fadda, Gabrielle Dufort, Jack Antel, Paul Giacomini, Myriam Levesque-Roy, Maryam Oskoui, Pierre Duquette, Alexandre Prat, Rose-Marie Rebillard, Inge Meijer, Elana Pinchefsky, Cam-Tu Emilie Nguyen, Elsa Rossignol, Jacinthe Rouleau, Oliver Blanchard, Nicole Khairallah, Philippe Beauchemin, Anne-Marie Trudelle, Emmanuelle Lapointe, Catherine Larochelle, Alexander Saveriano. 
Author Disclosure Block: H. Boudjani: Membership on advisory boards or speakers’ bureaus; Name of for-profit or not-for-profit organization(s); Biogen, Alexion. Membership on advisory boards or speakers’ bureaus; Description of relationship(s); Has served on scientific advisory board. G. Fadda: Any direct financial payments including receipt of honoraria; Name of for-profit or not-for-profit organization(s); Sanofi Genzyme, American Academy of Neurology. Any direct financial payments including receipt of honoraria; Description of relationship(s); Has received salary support from these institutions. G. Dufort: None. J. Antel: None. P. Giacomini: Any direct financial payments including receipt of honoraria; Name of for-profit or not-for-profit organization(s); Actelion, Alexion, Allergan, Biogen Idec, Bristol Myers Squibb-Celgene, EMD Serono, Genzyme-Sanofi, Innoderm Neurosciences, Merz, Novartis, Pendopharm, Roche, Teva Neurosciences. Any direct financial payments including receipt of honoraria; Description of relationship(s); Has received honoraria for consulting, speaking and advisory board participation for these institutions. M. Levesque-Roy: None. M. Oskoui: Funded grants or clinical trials; Name of for-profit or not-for-profit organization(s); Biogen, Roche. Funded grants or clinical trials; Description of relationship(s); Has received research support as Site investigator for clinical trials. P. Duquette: None. A. Prat: None. R. Rebillard: Any direct financial payments including receipt of honoraria; Name of for-profit or not-for-profit organization(s); Biogen, MS Society of Canada, Fonds de Recherche du Québec en Santé. Any direct financial payments including receipt of honoraria; Description of relationship(s); Has received salary support from these institutions. I. Meijer: None. E. Pinchefsky: None. C. Nguyen: None. E. Rossignol: None. J. Rouleau: None. O. Blanchard: None. N. Khairallah: Membership on advisory boards or speakers’ bureaus; Name of for-profit or not-for-profit organization(s); Sanofi, Roche, Biogen, Ally, Novartis. P. Beauchemin: Membership on advisory boards or speakers’ bureaus; Name of for-profit or not-for-profit organization(s); Alexion, Biogen, EMD-Serono, Pendopharm, Roche. All other investments or relationships that could be seen by a reasonable, well-informed participant as having the potential to influence the content of the educational activity; Name of for-profit or not-for-profit organization(s); Roche, Novartis. All other investments or relationships that could be seen by a reasonable, well-informed participant as having the potential to influence the content of the educational activity; Description of relationship(s); Served as a speaker for these institutions. A. Trudelle: Any direct financial payments including receipt of honoraria; Name of for-profit or not-for-profit organization(s); Alexion, Biogen, BMS, EMD-Serono, Hoffmann-La Roche, Novartis, Sanofi Genzyme. Any direct financial payments including receipt of honoraria; Description of relationship(s); Has received consulting and/or speaker honorarium from these institutions. E. Lapointe: Membership on advisory boards or speakers’ bureaus; Name of for-profit or not-for-profit organization(s); Novartis, Alexion, EMD Serono, Biogen, Hoffmann-La Roche, BMS, Sanofi-Genzyme. Membership on advisory boards or speakers’ bureaus; Description of relationship(s); Has served on scientific advisory boards and as a speaker for these institutions. C. Larochelle: Membership on advisory boards or speakers’ bureaus; Name of for-profit or not-for-profit organization(s); EMD-Serono, Biogen, Bristol-Myers Squibb, Roche, Novartis, Teva, Celgene, Actelion, Sanofi-Genzyme. Membership on advisory boards or speakers’ bureaus; Description of relationship(s); Has served of scientific advisory boards and as a speaker for these institutions. Funded grants or clinical trials; Name of for-profit or not-for-profit organization(s); EMD Serono/Merck. Funded grants or clinical trials; Description of relationship(s); Holds a Grant for Multiple Sclerosis Innovation (GMSI) from these institutions. All other investments or relationships that could be seen by a reasonable, well-informed participant as having the potential to influence the content of the educational activity; Name of for-profit or not-for-profit organization(s); Sanofi-Genzyme. All other investments or relationships that could be seen by a reasonable, well-informed participant as having the potential to influence the content of the educational activity; Description of relationship(s); Has received travel support. A. Saveriano: Membership on advisory boards or speakers’ bureaus; Name of for-profit or not-for-profit organization(s); EMD-Serono, Biogen, Roche, Novartis, Teva, Alexion, Celgene, Actelion, Sanofi-Genzyme. Membership on advisory boards or speakers’ bureaus; Description of relationship(s); Has served on scientific advisory boards and as a speaker for these institutions. All other investments or relationships that could be seen by a reasonable, well-informed participant as having the potential to influence the content of the educational activity; Name of for-profit or not-for-profit organization(s); EMD-Serono. All other investments or relationships that could be seen by a reasonable, well-informed participant as having the potential to influence the content of the educational activity; Description of relationship(s); Has received travel support from this institution.

Purpose: MOG antibody-associated disease (MOGAD) is a rare demyelinating disease distinct from multiple sclerosis (MS) and neuromyelitis optica spectrum disorder (NMOSD). We describe the clinical and paraclinical features of 45 patients with MOGAD, and their response to treatment. 

Study Design: This is a retrospective multicenter study. Methods: Patients with positive anti-MOG IgG were identified in different academic centers across the province of Quebec. 

Results: We identified 45 patients with MOGAD (20 Females, 44%). Median age at first presentation was 32 years (1-71). The most frequent ethnic groups were Caucasian (60%) and Asian (16%). The median time to diagnosis was 4 months (0-265). A preceding event was reported in 20 (44%) patients, with infections (65%, 13/20), vaccination (20%, 4/20) and a surgery/procedure (15%, 3/20) being the most frequently reported. Optic neuritis (ON) (55%, 25/45), of which 56% were unilateral, and transverse myelitis (TM) (27%, 21/45) were the most frequent first clinical presentation. ADEM was reported in 20% (9/45), and seizures in 7% (3/45). Papilledema was noted in 12/25 of patients with ON, and 63% (14/25) of them had severe visual impairment at presentation (≤ 20/200). MRI was abnormal in 38/45 of patients at presentation (brain and/or spine), and 47% (18/38) of these lesions showed complete resolution on subsequent MRIs. The mean WBC count in the cerebrospinal fluid was 10 X 106/L (0-384), with mostly lymphocytic predominance. Oligoclonal bands were absent in 90% of patients. IV methylprednisolone was used in 35/45 of patients as an acute treatment, followed in 25/45 by a course of steroid taper. After the first attack, 38% (17/45) of patients had a complete recovery. For those with a relapsing course (60%, 27/45) complete recovery occurred in 42% (40/94) of subsequent relapses. Median follow-up period was 2.6 years (0.25-24). Maintenance therapy was started in 33/45 patients (73%). Oral prednisone (21/33), azathioprine (13/33), IV immunoglobulin (12/33) and rituximab (8/33), alone or in combination were most frequently used. The median time to the first relapse was 3 months (0-121). The mean number of relapses per patient was 2.0 (1-11). Residual deficits were present in 31/45 69% of patients on last follow up, of which 47% (21/45) were visual. 

Conclusions: Our case series highlights the characteristics of Quebec’s multiethnic MOGAD patients. MOGAD is not always a benign disease, and best long-term management of this disease is still uncertain.