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The spectrum of neuromyelitis optica in Singapore: an 18-year retrospective study

Background: Neuromyelitis Optica (NMO) and its spectrum disorders (NMOSD), which include recurrent transverse myelitis (rTM) and recurrent optic neuritis (rON) are demyelinating diseases of the central nervous system (CNS). NMO is characterised by severe and selective involvement of the optic nerve and spinal cord and may be distinct from multiple sclerosis (MS) in pathogenesis, prognosis and response to treatment. At present, the NMO and NMOSD patients in Singapore have yet to be characterised. Objective: To describe the demographic, clinical, radiological and serological features and current treatment of NMOSD patients in Singapore. Methods: We retrospectively reviewed all patients with NMO and NMOSD from the National Neuroscience Institute (NNI) Neuroimmunology Database and Tissue Repository seen between 1992 and 2009. Results: Twenty-eight out of 158 patients fulfilled inclusion criteria (4 male:24 female; mean age of onset 46.4 years [range 19 – 77]). Seventeen patients were diagnosed with NMO (12 fulfilled 2006 Wingerchuk criteria), 7 had rTM and 4 had rON. Initial presentation were TM (n=10), ON (n=12), simultaneous opticospinal involvement (n=3) and brainstem involvement (n=3). No patient had a positive family history for CNS demyelinating disease. Five patients had initial brain MRI fulfilling Barkhof criteria for MS and 21 had longitudinally extensive spinal cord lesions (>=3 vertebral segments) at some point. Cerebrospinal fluid analysis (n=24) showed mean nucleated cell count of 42.5 cells/ul (range 0 – 360) and oligoclonal bands present in 2/18 patients. A significant number of patients were seropositive for anti-nuclear antibody (13/28), anti-Ro (6/27), and anti-double stranded DNA (4/26); 1 had lip biopsy suggestive of Sjogren’s syndrome. Mean duration of follow up was 64 months (range 6 – 219). At last follow-up, mean relapse rate was 0.63/year (range 0 – 2.4) and median Expanded Disability Status Score (EDSS) was 3.0; with 3 patients deceased and 1 lost to follow-up. Current treatments included azathioprine (n=11), methotrexate (n=5), mycophenolate mofetil (n=2) and Interferon-beta (n=1); 5 were not on disease modifying treatment. Conclusions: The results are consistent with published data for NMO from other cohorBilateral Internuclear Ophthalmoplegia in a Patient with Devic’s Neuromyelitis Optica
ts. We are conducting further analysis comparing MS and NMO patients in the local population.

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