Japanese variant multiple sclerosis or neuromyelitis optica?

TitleJapanese variant multiple sclerosis or neuromyelitis optica?
Publication TypeJournal Article
AuthorsWundes A, Hamilton S, Mayadev A, Kraft GH
JournalInternational Journal of MS Care

Background: Because our understanding of neuromyelitis optica (NMO)/Devic’s has improved, the diagnostic criteria have been recently revised. In the past, NMO was considered a variant within the spectrum of multiple sclerosis (MS), whereas now it is believed to be distinct from MS in terms of pathology, prognosis, and treatment options. Previously, brain magnetic resonance imaging (MRI) abnormalities were incompatible with a diagnosis of NMO; the revised criteria no longer exclude per se abnormalities on brain MRI. The controversy about whether Japanese variant MS is truly MS or NMO remains unresolved. Methods: Presented is a case presentation and literature review. Discussion: The patient is a 33-year-old Japanese woman initially diagnosed with Japanese variant MS based on clinical course (optic neuritis February 2001, transverse myelitis April 2001), abnormalities on cervical and thoracic spine MRI, and cerebrospinal fluid with pleocytosis but negative oligoclonal band and normal brain MRI. The patient had been clinically relatively stable on interferon ß-1a despite mild relapses and worsening of her brain MRI with >40 new lesions atypical but not inconsistent with MS. In September 2006, NMO-antibody testing was positive, and treatment was switched to mycophenolate mofetil. In March, September, and October 2007, the patient experienced severe exacerbations. Additionally, Sjogren’s syndrome was diagnosed (laboratory studies, dry eye syndrome) and symptomatic treatment initiated. This case represents a diagnostic and therapeutic challenge in the context of differentiation of MS versus NMO, especially because of brain involvement and concomitant autoimmune disease. Classification of disease plus optimal treatment for the acute relapses and long-term maintenance were considered. The patient is now regarded as a severe case of NMO with marked brain involvement. She takes an oral regimen of azathioprine/ steroids and received four doses of rituximab. She is slowly recovering some but overall has significantly worsened in her neurological and functional status. The case and relevant literature in this evolving area of NMO will be presented.


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