Abstract
PurposeTo 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). MethodsBased 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. ResultsFemale 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 seronegative group, and a mean period between 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.ConclusionAs 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.
Highlights
In 1894, Eugène Devic reported atypical cases of multiple sclerosis (MS) characterized by an inflammatory lesion of both optic nerves and spinal cord, a condition which he dubbed ‘neuromyelitis optica’ (NMO) [1, 2]
Antibodies against conformational myelin oligodendrocyte glycoprotein (MOG) epitopes detected by cell-based assay (CBA) were later causally implicated in acute disseminated encephalomyelitis (ADEM) and NMO spectrum disorders’ (NMOSD)
Neurological syndromes associated with MOG- and AQP4antibodies appear as two separate demyelinating diseases, characterized by distinct pathophysiology: AQP4NMOSD is an astrocytopathy while MOG-associated disorders’ (MOGAD) currently appears as an oligodendrogliopathy [18]
Summary
In 1894, Eugène Devic reported atypical cases of multiple sclerosis (MS) characterized by an inflammatory lesion of both optic nerves and spinal cord, a condition which he dubbed ‘neuromyelitis optica’ (NMO) [1, 2]. NMO was considered as a subtype of MS until the discovery of causal antibodies against AQP4 protein [3, 4] This objective biomarker allowed for a broadening of NMO phenotype to isolated myelitis without optic neuritis (ON) or even central nervous system (CNS) involvement without a spinal cord or optic nerve damage, e.g., area postrema syndrome [5]. This pleomorphic phenotype led to the concept of ‘NMO spectrum disorders’ (NMOSD) [6]. Antibodies against conformational MOG epitopes detected by cell-based assay (CBA) were later causally implicated in acute disseminated encephalomyelitis (ADEM) and NMOSD
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