Comparative study of AQP4-NMOSD, MOGAD and seronegative NMOSD: a single-center Belgian cohort

Abstract

To emphasize physio-pathological, clinical and prognosis differences between conditions causing serious and sometimes very similar clinical manifestations: anti-aquaporin-4 (AQP4) and anti-myelin oligodendrocyte glycoprotein (MOG) antibodies related diseases, and seronegative NMOSD (neuromyelitis optica spectrum disorders). Based on Wingerchuk et al. (Neurology 85:177–189, 2015) criteria for NMOSD and on those more recently proposed by Jarius et al. (J Neuroinflammation 15:134, 2018) for MOGAD (MOG associated disorders), we retrospectively surveyed 10 AQP4-NMOSD, 8 MOGAD and 2 seronegative NMOSD, followed at the specialized neuroimmunology unit of the CHU Liège. Female predominance was only observed in AQP4 group. Age at onset was 37.8 and 27.7 years old for AQP4-NMOSD and MOGAD, respectively. In both groups, the first clinical event most often consisted of optic neuritis (ON), followed by isolated myelitis. Fifteen of our 20 patients encountered a relapsing course with 90% relapses in AQP4-NMOSD, 62.5% in MOGAD and 50% in the seronegative group, and a mean period between the first and second clinical event of 7.1 and 4.8 months for AQP4-NMOSD and MOGAD, respectively. In total, we counted 54 ON, with more ON per patient in MOGAD. MOG-associated ON mainly affected the anterior part of the optic nerve with a papilledema in 79.2% of cases. Despite a fairly good visual outcome after MOG-associated ON, retinal nerve fibre layer (RNFL) thickness decreased, suggesting a fragility of the optic nerve toward further attacks. As observed in larger cohorts, our MOGAD and AQP4-NMOSD cases differ by clinical and prognostic features. A better understanding of these diseases should encourage prompt biological screening and hasten proper diagnosis and treatment.

Risk Factors and Nomogram for Predicting Relapse Risk in Pediatric Neuromyelitis Optica Spectrum Disorders

“…These findings suggest that the initial presentation had a higher predictive value for the clinical presentation of the second attack. Additionally, in the AQP4 antibody-positive children, younger patients were more likely to relapse with brain/brainstem onset than older patients (14), and this was clinically distinct from adult patients in that ON attack had more frequency at sequential attack (37,38). Also, our study demonstrated that TM onset was a potential predictor for TM recurrence at the second attack, and this phenomenon was observed in brain/brainstem onset as well but not found in the children with ON and mixed-lesion onset.…”

Neuro-ophthalmological Presentation of Optic Neuritis in Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease

“…A systematic review and meta-analysis has shown that eyes of AQP4-IgG + NMOSD patients with history of ON have worse visual acuity outcome when compared with those of MOGAD and MS patients [26], with other studies showing similar results [62][63][64][65][66]. In eyes with comparable pRNFL and GCIPL thinning, the degree of visual impairment in MOGAD patients is worse than that of MS patients but better than that of AQP4-IgG + NMOSD patients [64].…”

Optical Coherence Tomography in Chronic Relapsing Inflammatory Optic Neuropathy, Neuromyelitis Optica and Multiple Sclerosis: A Comparative Study

“…Corticosteroids are used for acute attacks, while different other modalities, such as immunosuppressive medications, are used off-label as maintenance therapy. Although diagnostic criteria for the disease have been defined, CRION essentially remains a diagnosis of exclusion, as other demyelinating, autoimmune, and systemic causes should be ruled out first [ 1 , 2 , 3 , 4 , 5 ]. Despite the distinct clinical features characterizing CRION and NMOSD, such as a younger age of onset in CRION and female predominance in NMOSD, diagnosis remains challenging [ 5 ].…”

“…Although diagnostic criteria for the disease have been defined, CRION essentially remains a diagnosis of exclusion, as other demyelinating, autoimmune, and systemic causes should be ruled out first [ 1 , 2 , 3 , 4 , 5 ]. Despite the distinct clinical features characterizing CRION and NMOSD, such as a younger age of onset in CRION and female predominance in NMOSD, diagnosis remains challenging [ 5 ]. CRION patients may be incorrectly diagnosed with relapsing-remitting multiple sclerosis (RRMS), neuromyelitis optica spectrum disorder (NMOSD), or multiple sclerosis (MS)-mimics.…”