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Fulminant Devic disease successfully treated by lymphocytapheresis
  1. I Nozaki,
  2. T Hamaguchi,
  3. K Komai,
  4. M Yamada
  1. Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, Kanazawa, Ishikawa, Japan
  1. Correspondence to:
 Ichiro Nozaki
 Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Science, 13-1 Takara-machi, Kanazawa, Ishikawa 920-8640, Japan; ichi51{at}med.kanazawa-u.ac.jp

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Devic disease is a demyelinating disease characterised by acute optic neuritis and transverse myelitis. Although there are differences in the pathogenesis of Devic disease and multiple sclerosis, the treatment for Devic disease is the same as that for multiple sclerosis. We report a patient with Devic disease who was resistant to high-dose intravenous methylprednisolone and intravenous immunoglobulin (Ig) treatments, but was effectively treated with lymphocytapheresis (LCP). The profiles of cytokines during the clinical course are discussed.

Case report

In August 2001, a 27-year-old woman developed acute loss of vision and sensory disturbance in both lower extremities after coughing. The next morning (day 2), she was totally blind and was admitted to our hospital. Neurological examination showed total blindness, papilloedema, moderate muscle weakness of the hip and thigh muscles, positive pathological reflexes of the limbs, hyper-reflexia and sensory loss below the T4–5 level. Loss of vesicorectal function and perspiration below the chest were also observed.

Laboratory examination disclosed a normal C-reactive protein level, positive antinuclear (titre 1/160) and antithyroglobulin antibodies (0.8 U/ml (normal range <0.3)) in serum, normal CD4:CD8 ratio (0.90 (normal range 0.69−1.74)) and an increased percentage of CD8 T cells (45% of total T cells (normal range 12−30%)). Anti-neutrophil cytoplasmic antibodies, anti-phospholipid antibodies and rheumatoid factor were …

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