This medical condition has either been superseded or has become inactive
Specific Conditions
  • Devic disease (neuromyelitis optica)
Level of Evidence Insufficient data (Category 4a)
Devic disease is an idiopathic inflammatory demyelinating disorder of the central nervous system characterised by recurrent bouts of optic neuritis and myelitis. It is distinct from multiple sclerosis and evidence of B-cell autoimmunity has been found. A circulatory antibody to aquaporin-4 is found in many patients, providing further evidence of B-cell autoimmunity in its pathogenesis and suggestive of a role for intravenous immunoglobulin (IVIg) therapy. Single case reports of various therapies, including IVIg, have shown variable benefit in this otherwise devastating disorder.

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The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.

 
Bibliography
Lennon, VA, Wingerchuk, DM, Kryzer, TJ, et al 2004, ‘A serum autoantibody marker of neuromyelitis optica: distinction from multiple sclerosis’, Lancet, vol. 364, no. 9451, pp. 2106–12.

Lucchinetti, CF, Mandler, RN, McGavern, D, et al 2002, ‘A role for humoral mechanisms in the pathogenesis of Devic’s neuromyelitis optica’, Brain, vol. 125, pp. 1450–61.

Minagar, A, Alexander, JS, Fowler, MR, et al 2002, ‘Devic disease: clinical course, pathophysiology, and management’, Pathophysiology, vol. 9, no. 1, p. 33.