Abstract
Neuromyelitis optica spectrum disorder (NMOSD) and multiple sclerosis (MS) are inflammatory diseases of the CNS. Overlap in the clinical and MRI features of NMOSD and MS means that distinguishing these conditions can be difficult. With the aim of evaluating the diagnostic utility of MRI features in distinguishing NMOSD from MS, we have conducted a cross-sectional analysis of imaging data and developed predictive models to distinguish the two conditions. NMOSD and MS MRI lesions were identified and defined through a literature search. Aquaporin-4 (AQP4) antibody positive NMOSD cases and age- and sex-matched MS cases were collected. MRI of orbits, brain and spine were reported by at least two blinded reviewers. MRI brain or spine was available for 166/168 (99%) of cases. Longitudinally extensive (OR = 203), “bright spotty” (OR = 93.8), whole (axial; OR = 57.8) or gadolinium (Gd) enhancing (OR = 28.6) spinal cord lesions, bilateral (OR = 31.3) or Gd-enhancing (OR = 15.4) optic nerve lesions, and nucleus tractus solitarius (OR = 19.2), periaqueductal (OR = 16.8) or hypothalamic (OR = 7.2) brain lesions were associated with NMOSD. Ovoid (OR = 0.029), Dawson's fingers (OR = 0.031), pyramidal corpus callosum (OR = 0.058), periventricular (OR = 0.136), temporal lobe (OR = 0.137) and T1 black holes (OR = 0.154) brain lesions were associated with MS. A score-based algorithm and a decision tree determined by machine learning accurately predicted more than 85% of both diagnoses using first available imaging alone. We have confirmed NMOSD and MS specific MRI features and combined these in predictive models that can accurately identify more than 85% of cases as either AQP4 seropositive NMOSD or MS.
Highlights
Neuromyelitis optica spectrum disorder (NMOSD) is an antibody-mediated autoimmune disease of the CNS in which the primary target for inflammation is aquaporin 4 (AQP4), a water channel found on the foot processes of astrocytes [1]
For this study we were only concerned with AQP4 antibody associated NMOSD and not MOG antibody associated demyelination (MOGAD)
The initial literature search identified 426 potential articles relating to MRI features in NMOSD, of which 118 were selected for full review
Summary
Neuromyelitis optica spectrum disorder (NMOSD) is an antibody-mediated autoimmune disease of the CNS in which the primary target for inflammation is aquaporin 4 (AQP4), a water channel found on the foot processes of astrocytes [1]. NMOSD is frequently a disabling condition when untreated, with high relapse rates and a high risk of permanent neurological deficits following an attack [2]. Acute treatment with plasma exchange appears to be effective in NMOSD [4]. The risk of NMOSD relapses is diminished by immunosuppressive therapy, anti-B-cell therapies (e.g., rituximab, inebilizumab), anti-IL6 receptor and anticomplement component 5 monoclonal antibodies [5,6,7,8]. It is important to recognise NMOSD early and distinguish it from MS prior to commencing disease modifying therapy
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