Abstract
Neuromyelitis optica or Devic syndrome is a severe demyelinating syndrome. This entity although of low prevalence represents a diagnostic and therapeutic challenge, especially at the moment of differentiation with multiple sclerosis. With the discovery of aquaporin-4 specific serum autoantibody, in the pathophysiology of this syndrome, we have expanded the tools to make a diagnosis with greater certainty and identify options for treatment. Here is an update of the literature regarding its role in the diagnosis and treatment of neuromyelitis optica.
References
SELLNER J, BOGGILD M, CLANET M ET AL. EFNS guidelines on diagnosis and management of neuromyelitis Optica. European Journal of Neurology 2010;17:1019-1032.
WINGERCHUK DM, HOGANCAMP WF, O_BRIEN PC, ET AL. The clinical course of neuromy-elitis optica (Devic_s syndrome). Neurology 1999;53:1107-1114.
LANA-PEIXOTO M. Devic's neuromyelitis optica. Arq Neuropsiquiatr 2008;66:120-138.
GRABER DJ, LEVY M, KERR D, ET AL. Neuromyelitis optica pathogenesis and aquaporin 4. J Neuroinflammation 2008;5:22-80.
TAIT MJ, SAADOUN S, BELL BA, ET AL. Water movements in the brain:role of aquaporins. Trends Neurosci 2008;31:37-43.
NAGGELHUS EA, HORIO Y, INANOBE A, ET AL. Immunogold evidence suggests that coupling of K+ siphoning and water transport in rat retinal Müller cells is mediated by a coenrichment of Kir4.1 and AQP4 in specific membrane domains. Glia 1999;26:47-54.
ZENG XN, SUN XL, GAO L, FAN Y, DING JH, HU G. Aquaporin-4 deficien-cy down-regulates glutamate uptake and GLT-1 expression in astrocytes. Mol Cell Neurosci 2007;34:34-39.
BENARROCH EE. Aquaporin-4, homeostasis, and neurologic disease. Neurology 2007;69:2266-2268.
GRIESDALE DE, HONEY CR. Aquaporins and brain edema. Surg Neurol 2004;61:34-39.
MERLE H, OLINDO S, BONNAN M, ET AL. Natural history of the visual impairment of relapsing neuromyelitis optica. Ophthalmology 2007;114:810-815.
WINGERCHUK DM, WEINSHENKER BG. Neuromyelitis optica:clinical predictors of a relapsing course and survival. Neurology 2003;60:848-853.
MANDLER RN, DAVIS LE, JEFFERY DR, ET AL. Devic_s neuromyelitis optica:a clinicopathologi-cal study of 8 patients. Ann Neurol 1993;34:162-168.
FARDET L, GENEREAU T, MIKAELOFF Y, ET AL. Devic_s neuromyelitis optica:study of nine cases. Acta NeurolScand 2003;108:193-200.
ORIORDAN JI, GALLAGHER HL, THOMPSON AJ, ET AL. Clinical, CSF, and MRI findings in Devic_s neuromielitis optica. J Neurol Neurosurg Psychiatry 1996;60:382-387.
POPPE AY, LAPIERRE Y, MELANCON D, ET AL. Neuromyelitis optica with hypothalamic involvement. Mult Sckr 2005;11:617-621.
VERNANT JC, CABRE P, SMADJA D, ET AL. Recurrent optic neuromyelitis with endocrinopathies:a new syndrome. Neurology 1997;48:58-64.
SEZE J, STOJKOVIC T, FERRIBY D, ET AL. Devic_s neuromyelitis optica:clinical, laboratory, MRI and outcome profile. J Neurol Sci 2002;197:57-61.
CABRERA-GOMEZ JA, KURTZKE JF, GON-ZALEZ-QUEVEDO A, ET AL. An epidemiological study of neuromyelitis optica in Cuba. J Neurol 2009;256:35-44.
KANZAKI M, MOCHIZUKI H, OGAWA G, ET AL. Clinical features of opticospinal multiple sclerosis with anti-aquaporin 4 antibody. Eur Neurol 2008;60:37-42.
JOHNSON G, MILLER DH, MACMANUS D, ET AL. STIR sequences in NMR imaging of the optic nerve. Neuroradiology 1987;29:238-245.
KUPERSMITH MJ, ALBAN T, ZEIFFER B, ET AL. Contrastenhanced MRI in acute optic neuritis:relationship to visual performance. Brain 2002;125:812-822.
WINGERCHUK DM, LENNON VA, PITTOCK SJ, ET AL. Revised diagnostic criteria for neuromy-elitis optica. Neurology 2006;66:1485-1489.
FILIPPI M, ROCCA MA, MOIOLA L, ET AL. MRI and magnetization transfer imaging changes in the brain and cervical cord of patients with Devic_s neuromyelitis optica. Neurology 1999;53:1705-1710.
PITTOCK SJ, LENNON VA, KRECKE K, ET AL. Brain abnormalities in neuromyelitis optica. Arch Neurol 2006;63:390-396.
FAZIO R, MALOSIO ML, LAMPASONA V, ET AL. Antiacquaporin 4 antibodies detection by different techniques in neuromyelitis optica patients. Mult Scler 2009;15:1153-1163.
WATERS P, VINCENT A. Detection of antiaquaporin-4 antibodies in neuromyelitis optica: current status of the assays. Int MS J 2008;15:99-105.
CABRE P, GONZALEZ-QUEVEDO A, BONNAN M, ET AL. Relapsing neuromyelitis optica:long term history and predictors of death. J Neurol Neurosurg Psychiatry 2009;80:1162-1164.
PAPAIS-ALVARENGA RM, MIRANDA-SANTOS CM, PUCCIONI-SOHLER M, ET AL. Optic neuromyelitis syndrome in Brazilian patients. J Neurol Neu-rosurg Psychiatry 2002;73:429-435.
WEINSHENKER BG, WINGERCHUK DM, VUKUSIC S, ET AL. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol 2006;59:566-569.
DE SE-ZE J, ARNDT C, JEANJEAN L, ET AL. Relapsing inflammatory optic neuritis:is it neuromyelitis optica?. Neurology 2008;70:2075-2076.
MARIGNIER R, DE SEZE J, VUKUSIC S, ET AL. NMO-IgG and Devic_s neuromyelitis optica:a French experience. Mult Scler 2008;14:440-445.
JARIUS S, ABOUL-ENEIN F, WATERS P, ET AL. Antibody to aquaporin-4 in the long-term course of neuromyelitis optica. Brain 2008;131:3072-3080.
LALIVE PH, MENGE T, BARMAN I, CREE BA, GENAIN CP. Identification of new serum autoantibodies in neuromyelitis optica using protein microar-rays. Neurology 2006;67:176-177.
MARIGNIER R, GIRAUDON P, VUKUSIC S, CONFAVREUX C, HONNORAT J. Anti-aquapo-rin-4 antibodies in Devic's neuromyelitis optica:therapeutic implications. Ther Adv Neurol Disord 2010;3:311-321.
MATIELLO M, LENNON VA, JACOB A, ET AL. NMO-IgG predicts the outcome of recurrent optic neuritis. Neurology 2008;70:2197-2200.
WEINSHENKER BG, WINGERCHUK DM, VUKUSIC S, ET AL. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol 2006;59:566-569.
PITTOCK SJ, LENNON VA, DE SEZE J, ET AL. Neuromyelitis optica and non organ-specific autoimmunity. Arch Neurol 2008;65:78-83.
MCKEON A, LENNON VA, JACOB A, ET AL. Coexistence of myasthenia gravis and serological markers of neurological autoimmunity in neuromyelitis optica. Muscle Nerve 2009;39: 87-90.
KIRA J. Neuromyelitis optica and asian phenotype of multiple sclerosis. Ann N Y Acad Sci 2008;1142:58-71.
MILLER DH, WEINSHENKER BG, FILIPPI M, ET AL. Differential diagnosis of suspected multiple sclerosis:a consensus approach. Mult Scler 2008;14:1157-1174.
LLUFRIU S, CASTILLO J, BLANCO Y, ET AL. Plasma exchange for acute attacks of CNS demy-elination:predictors of improvement at 6 months. Neurology 2009;73:949-953.
WEINSHENKER BG, OBRIEN PC, PETTER-SON TM, ET AL. A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol 1999; 46:878-886.
KEEGAN M, PINEDA AA, MCCLELLAND RL, ET AL. Plasma exchange for severe attacks of CNS demyelination:predictors of response. Neurology 2002;58:143- 146.
WATANABE S, NAKASHIMA I, MISU T, ET AL. Therapeutic efficacy of plasma exchange In NMO-IgG-positive patients with neuromyelitis optica. Mult Scler 2007;13:128-132.
RUPRECHT K, KLINKER E, DINTELMANN T, ET AL. Plasma exchange for severe optic neuritis:treatment of 10 patients. Neurology 2004;63:1081-1083.
BONNAN M, VALENTINO R, OLINDO S, ET AL. Plasma exchange in severe spinal attacks associated with neuromyelitis optica spectrum disorder. Mult Scler 2009;15:487-492.
MIYAMOTO K, KUSUNOKI S. Intermittent plasmapheresis prevents recurrence in neuromyelitis optica. Ther Apher Dial 2009; 13: 505-508.
MANDLER RN, AHMED W, DENCOFF JE. Devic_s neuromyelitis optica:a prospective study of seven patients treated with prednisone and azathio-prine. Neurology 1998;51:1219-1220.
BICHUETTI DB, LOBATO DE OLIVEIRA EM, OLIVEIRA DM, AMORIN DE SOUZA N, GABBAI AA. Neuromyelitis Optica Treatment:Analysis of 36 Patients. Arch Neurol 2010;67:1131-1136.
WINGERCHUK DM, WEINSHENKER BG. Neuromyelitis optica. Curr Treat Options Neurol 2005;7:173-182.
JACOB A, WEINSHENKER BG, VIOLICH I, ET AL. Treatment of neuromyelitis optica with rituximab:retrospective analysis of 25 patients. Arch Neurol 2008;65:1443-1448.
CREE BA, LAMB S, MORGAN K, ET AL. An open label study of the effects of rituximab in neuromyelitis optica. Neurology 2005;64:1270-1272.
KIMBY E. Tolerability and safety of rituximab (MabThera). Cancer Treat Rev 2005;31:456-473.
MOK CC, TO CH, MAK A, ET AL. Immunoablative cyclophosphamide for refractory lupus-related neuromyelitis optica. J Rheumatol 2008;35:172-174.
WEINSTOCK-GUTTMAN B, RAMANATHAN M, LINCOFF N, ET AL. Study of mitoxantrone for the treatment of recurrent neuromyelitis optica (Devic disease). Arch Neurol 2006;63:957-963.
JACOB A, MATIELLO M, WEINSHENKER BG, ET AL. Treatment of neuromyelitis optica with mycophenolate mofetil:retrospective analysis of 24 patients. Arch Neurol 2009;66:1128-1133.

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
