Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Long segments of spinal cord infl ammation (myelitis), severe optic neuritis, and/or bouts of intractable vomiting and hiccoughs (area postrema syndrome) are classic presentations of the disease and may alert the clinician to the diagnosis. Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their fi rst attack. Unlike multiple sclerosis, a progressive clinical course is very unusual and the accrual of disability is related to relapses. Approximately 75% of patients have antibodies against aquaporin-4, a water channel expressed on astrocytes. Relapses are treated aggressively to prevent residual disability with highdose steroids and often plasma exchange. Relapse prevention is crucial and achieved with long-term immuno suppression. In this article we review the pathogenesis, clinical features, diagnosis and management of NMOSD.
Patients without VF deficits or whose visual deficits are stable or improving can be managed expectantly without negative impact on outcomes. Clinical severity based on a PAS ≥ 4 appeared to influence management towards emergency surgical intervention.
Aim There are numerous treatment options for intermittent distance exotropia. The aim of this study is to evaluate the use of overcorrecting minus lenses as a primary treatment option for intermittent distance exotropia (IDEX) and determine ocular alignment status after 5 years from commencement of the study. Methods Prospective nonrandomised longitudinal cohort study in which 21 patients were recruited with a diagnosis of IDEX. Treatment was instigated with the minimum minus lens required to achieve control of the manifest deviation. The strength of lenses was reduced over time while monitoring the results of orthoptic measurements. The results were evaluated by nonparametric (Wilcoxon) and parametric (paired t-test) analysis. Results Thirteen female and eight male patients were recruited with a mean age of 5 years at the start of treatment. There was a significant reduction in angle of deviation after treatment. Overcorrecting lenses did not appear to induce myopia. Twenty-four per cent of the patients had a successful outcome, 28% had a good outcome, and 33% required surgery at a later date. Conclusions Fifty-two per cent of the patients achieved a successful or good outcome with overcorrecting minus lenses alone, and this was maintained for at least 1 year follow-up. Those requiring surgery had been unable to wean out of lenses but had a successful outcome after one procedure. We recommend overcorrecting minus lenses as a primary treatment option for IDEX with the knowledge that surgery, if subsequently required, is safely delayed to an older age without prior loss of binocular vision.
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