Severe structural and functional visual system damage leads to profound loss of vision-related quality of life in patients with neuromyelitis optica spectrum disorders
Introduction
Neuromyelitis optica spectrum disorders (NMOSD) are autoimmune CNS conditions primarily presenting with longitudinally extensive transverse myelitis (LETM) and optic neuritis (ON), and to a lesser extent with area postrema, acute brainstem, diencephalic and cerebral syndromes (Wingerchuk et al., 2015). Initially thought to be a variant of multiple sclerosis (MS), the detection of serum autoantibodies targeting the water channel aquaporin-4 (AQP4-IgG) in up to 80% of cases established NMOSD as an own disease entity distinct from MS (Jarius et al., 2014, Zekeridou and Lennon, 2015). Recently, serum antibodies directed against myelin oligodendrocyte glycoprotein (MOG-IgG) were found in a subset of AQP4-IgG negative NMOSD patients and in patients with recurrent optic neuritis, further extending the range of NMOSD (Jarius et al., 2016a, Jarius et al., 2016b, Jarius et al., 2016c, Pache et al., 2016).
Although pathogenically distinct, NMOSD and MS show a considerable overlap in clinical and paraclinical presentation, including involvement of the afferent visual system by acute ON (Wingerchuk et al., 2015). ON occurs in approximately 80% of NMOSD patients, in approximately 20% of the cases bilaterally, often involving the optic chiasm (Kleiter et al., 2016, Ramanathan et al., 2016). In contrast, ON in MS occurs in up to 70% of patients, is mainly unilateral and chiasm involving optic nerve lesions are rare (Costello, 2016, Toosy et al., 2014). In most cases, ON-associated damage is more severe in NMOSD patients than in MS patients, leading to more severe residual visual loss and structural damage (Bennett et al., 2015).
Visual function has great relevance for quality of life, affecting everyday tasks and vision-dependent activities like driving or social and role functioning. Consequently, in a previous study, MS patients rated vision as the second most important bodily function following lower limb function (Heesen et al., 2008). Further impact of vision-related quality of life has been previously investigated in MS patients and its association with retinal axonal and neuronal damage and reduced visual function shown (Mowry et al., 2009, Schinzel et al., 2014, Walter et al., 2012).
However, although NMOSD patients suffer from greater visual function loss than MS patients, vision-related quality of life has hitherto not been investigated in this condition. Thus, the goals of our study were to investigate vision-related quality of life in NMOSD compared to MS patients and to relate vision-related quality of life to structural damage and dysfunction of the visual system in patients with NMOSD.
Section snippets
Study participants and controls
Data for this cross-sectional study was derived from two on-going longitudinal observational studies following patients with NMOSD and MS. Thirty-six patients from the outpatient clinics of NeuroCure Clinical Research Center and from the Department of Neurology, Charité – Universitätsmedizin Berlin were screened for eligibility. Inclusion criteria were NMOSD according to the current diagnostic criteria (Wingerchuk et al., 2015) or MOG-IgG associated recurrent ON and age ≥18 and ≤70 years.
Results
Vision-related quality of life represented by the NEI-VFQ composite score was significantly lower in NMOSD patients (77.4±19.2) compared to MS patients (86.6±6.7, p=0.01) (Fig. 1). Patients with unilateral ON regularly displayed high vision-related quality of life, whereas patients with ON episodes in both eyes reported the strongest loss: A multivariate linear regression model showed that the severity of vision-related quality of life loss was driven by a higher incidence of bilateral in
Discussion
In this cross-sectional study we show that vision-related quality of life is more severely impaired in NMOSD than in MS patients. Key findings are, a) NEI-VFQ score was significantly reduced in NMOSD compared to MS patients; b) This effect is mostly attributable to the higher occurrence and stronger severity of ON episodes in both eyes in NMOSD patients whereas patients with unilateral ON showed only little loss of vision-related quality of life despite often-times severe structural and
Funding
This work was supported by Bundesministerium für Bildung und Forschung (Competence Network Multiple Sclerosis and N2-ADVISIMS), Deutsche Forschungsgemeinschaft (DFG Exc 257 to FrP), a limited research grant from Novartis Pharma, and the BIH-Charité Medical Student Research Program at Charité-Universitätsmedizin Berlin (to FCO).
Conflict of interest
The authors declare that they have no conflict of interest.
Declaration of interest
F. Schmidt reports personal fees from Genzyme, outside the submitted work; H. Zimmermann reports personal fees from Teva, personal fees from Bayer, outside the submitted work; J. Mikolajczak reports personal fees from TEVA, personal fees from Biogen, outside the submitted work; . F. Oertel has nothing to disclose. F. Pache reports personal fees from Genzyme, outside the submitted work; . M. Weinhold reports personal fees from TEVA, outside the submitted work; J. Schinzel has nothing to
Acknowledgements
We thank Ivonne Hinz and Brian Dommisch for excellent technical assistance.
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