Author Of 2 Presentations
P0708 - Differential MRI biomarkers between MOGAD, AQP4-NMOSD and RRMS: a MAGNIMS multicenter study (ID 1335)
Clinical and imaging features of myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) may overlap with those of aquaporin 4-neuromyelitis optica spectrum disorder (AQP4-NMOSD) and relapsing remitting multiple sclerosis (RRMS). There is an unmet need for MRI biomarkers which reflect biological mechanisms involved in MOGAD and can help in the differential diagnosis.
We aim to identify imaging features able to differentiate between non-acute MOG-antibody disease, AQP4-NMOSD and RRMS.
In this ongoing retrospective, cross-sectional MAGNIMS study, we analyzed data collected from 8 centers. All subjects (n=352) had brain and cervical cord 3T MRI. Quantification of MRI biomarkers included brain white matter lesions (WMLs), cortical lesions (CL), brain parenchymal fraction (BPF), white matter fraction (WMF), cortical and deep grey matter fractions (GMF) and cross-sectional cervical cord area (CSA) at C1-C2. Linear regression models were used to compare MRI measures between groups, corrected for age, sex, and centre. Statistical significance was considered when p was <0.05.
91 patients with MOGAD (50F, mean age: 41yrs [±15]), 85 with AQP4-NMOSD (68F, 49yrs [±14]), 90 with RRMS (56F, 41yrs [±11]) and 87 healthy controls (HCs) (54F, 36yrs [±11.6]) were collected. The most common phenotypes at onset were optic neuritis and transverse myelitis in MOGAD (93%) and AQP4-NMOSD (87%). WMLs were detected in 57% MOGAD, 79% AQP4-NMOSD, all RRMS (100%) patients, and in 15% HCs. The mean lesion load and number of lesions were higher in RRMS than both MOGAD (p=0.007, p<0.001) and AQP4-NMOSD (p=0.001, p<0.001). At least one CL was seen in 8% patients with MOGAD (total n=8), 10% patients with AQP4-NMOSD (n=7), and in 69% patients with RRMS (n=150). All patient groups showed lower BPF than HCs, with lower WMF in MOGAD and RRMS than HCs (all p<0.01). Between groups, deep GMF was lower in RRMS than MOGAD (p<0.001) and AQP4-NMOSD (p=0.001). CSA was reduced in all disease groups when compared to HCs (all p<0.01) and lower in AQP4-NMOSD than RRMS (p=0.01).
This ongoing study indicates that MOGAD and AQP4-NMOSD share similar MRI features, and no specific MRI biomarker can distinguish between them. Patients with AQP4-NMOSD showed greater spinal cord atrophy than RRMS, and RRMS patients had a higher number of cortical lesions, and greater deep GM atrophy than AQP4-NMOSD and MOGAD. The next step is to investigate whether lesion distribution differs between the two antibody-mediated disease.
P0761 - What is seronegative neuromyelitis optica spectrum disorder? (ID 1160)
Most but not all cases of Neuromyelitis Optica Spectrum Disorder (NMOSD) are associated with Aquaporin-4 (AQP4) or myelin oligodendrocyte glycoprotein (MOG) antibodies.
To determine the core clinical characteristics of seronegative NMOSD patients fulfilling 2015 International Diagnostic consensus criteria, treated in the National NMOSD service.
Retrospective review of patient databases at The Walton Centre for Neurology and Neurosurgery and The John Radcliffe Hospital (Neuromyelitis Optica National Referral Centres) between 1st January 2010 - 17th January 2020.
Of NMOSD=727, 49(7%) were seronegative. The male to female ratio was 1:2.5 and median age at onset was 36(5-57) years. In 2/3 of patients the index presentation was myelitis=22 or myelitis+optic neuritis=11. In 26/33 (79%), longitudinally extensive myelitis was present. Optic neuritis=9 (4 bilateral) and brain involvement=7 were also seen. Relapsing disease was observed in 39/49(80%) of patients. The median annualised attack rate was 0.58 over a median disease duration of 78 (3-258) months. Unmatched CSF oligoclonal bands (CSF-OCBs) were detected in 4/38(11%) and 31/49(63%) fulfilled multiple sclerosis (MS) diagnostic criteria. Immunosuppression (typically Mycophenolate and Rituximab) was used in 34/49(69%). Median last EDSS was 4 (1-10) with death recorded in 5/49 (10%) patients.
Seronegative NMOSD is uncommon. Longitudinal myelitis with/without optic neuritis is a common initial presentation. Similar to AQP4-IgG, NMOSD disability and mortality rates are high. Absence of unmatched CSF-OCB and typical brain lesions help to distinguish this disease from MS.