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Erschienen in: Current Treatment Options in Neurology 3/2010

01.05.2010 | Neuroimmunology

Neuromyelitis Optica

verfasst von: MBBS, MD William M. Carroll, MD Kazuo Fujihara

Erschienen in: Current Treatment Options in Neurology | Ausgabe 3/2010

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Opinion statement

Neuromyelitis optica (NMO) or Devic’s disease typically involves the optic nerves and the spinal cord and is most often relapsing. The pathogenesis is one of an acute inflammatory process targeting astrocytes and resulting in demyelination, as well as axonal injury. In a high proportion of recognized cases of NMO, there is a highly specific autoantibody (NMO-IgG), which is directed to the common central nervous system water channel, aquaporin-4. NMO attacks usually result in severe residual visual impairment or myelopathy. Despite the publication of new diagnostic criteria for NMO, uncertainty at the time of the index event as to whether the attack is due to multiple sclerosis or NMO can cause therapeutic hesitancy. Nevertheless, whenever a reasonable degree of suspicion exists, therapies directed to limiting acute injury and to preventing subsequent further injury mediated by humoral mechanisms should be instituted immediately. Investigations can then be completed and the therapeutic direction confirmed.
For an acute attack, high-dose methylprednisolone and plasma exchange (generally given sequentially) are most useful.
For the prevention of further attacks, selective or nonselective immunosuppressive therapy directed to humoral mechanisms is preferred. Agents recommended are oral azathioprine or mycophenolate mofetil with or without low-dose prednisolone or rituximab. Therapy should be planned to continue for up to 5 years in all patients, including those with a single attack who are at high risk of further relapse.
Regrettably, there are no controlled trials for the treatment of either the classic manifestations of NMO or the so-called limited manifestations known as NMO spectrum disorders. The therapeutic opinions expressed in this article are therefore based on the current understanding of the pathogenesis of this disorder, reports of small series of patients receiving a range of treatments, and expert opinions.
Literatur
1.
Zurück zum Zitat Aoyama T: A case of myelitis and blindness. J Tokyo Med Assoc 1891, 5:827–830. Aoyama T: A case of myelitis and blindness. J Tokyo Med Assoc 1891, 5:827–830.
2.
Zurück zum Zitat Allbutt TC: On the ophthalmoscopic signs of spinal disease. Lancet 1870, 1:76–78. Allbutt TC: On the ophthalmoscopic signs of spinal disease. Lancet 1870, 1:76–78.
3.
Zurück zum Zitat Dévic E: Myélite aiguë dorso-lombaire avec névrite optique. Autopsie. Congrès Français de Médecine (Lyon) 1894, 1:434–439. Dévic E: Myélite aiguë dorso-lombaire avec névrite optique. Autopsie. Congrès Français de Médecine (Lyon) 1894, 1:434–439.
4.
Zurück zum Zitat Gault F: De la neuromyélite optique aiguë [thesis]. Lyon, 1894. Gault F: De la neuromyélite optique aiguë [thesis]. Lyon, 1894.
5.
Zurück zum Zitat Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG: The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology 1999, 53:1107–1114. PubMed Wingerchuk DM, Hogancamp WF, O’Brien PC, Weinshenker BG: The clinical course of neuromyelitis optica (Devic’s syndrome). Neurology 1999, 53:1107–1114. PubMed
6.•
Zurück zum Zitat Lotze TE, Northrop JL, Hutton GJ, et al.: Spectrum of pediatric neuromyelitis optica. Pediatrics 2008, 122:e1039–e1047. CrossRefPubMed Lotze TE, Northrop JL, Hutton GJ, et al.: Spectrum of pediatric neuromyelitis optica. Pediatrics 2008, 122:e1039–e1047. CrossRefPubMed
7.
Zurück zum Zitat Pittock SJ, Lennon VA, Krecke K, et al.: Brain abnormalities in neuromyelitis optica. Arch Neurol 2006, 63:390–396. CrossRefPubMed Pittock SJ, Lennon VA, Krecke K, et al.: Brain abnormalities in neuromyelitis optica. Arch Neurol 2006, 63:390–396. CrossRefPubMed
8.••
Zurück zum Zitat Matsushita T, Isobe N, Piao H, et al.: Reappraisal of brain MRI features in patients with multiple sclerosis and neuromyelitis optica according to anti-aquaporin-4 antibody status. J Neurol Sci 2010 Jan 30 (Epub ahead of print). This article describes the MRI features in AQP4-positive Japanese NMO patients and shows that ovoid lesions of MS are common and that those with prominent brain lesions have high risk of recurrent attacks. Matsushita T, Isobe N, Piao H, et al.: Reappraisal of brain MRI features in patients with multiple sclerosis and neuromyelitis optica according to anti-aquaporin-4 antibody status. J Neurol Sci 2010 Jan 30 (Epub ahead of print). This article describes the MRI features in AQP4-positive Japanese NMO patients and shows that ovoid lesions of MS are common and that those with prominent brain lesions have high risk of recurrent attacks.
9.
Zurück zum Zitat Scolding N: Devic’s disease and autoantibodies. Lancet Neurol 2005, 4:136–137. PubMed Scolding N: Devic’s disease and autoantibodies. Lancet Neurol 2005, 4:136–137. PubMed
10.
Zurück zum Zitat Wingerchuk DM, Lennon VA, Pittock SJ, et al.: Revised diagnostic criteria for neuromyelitis optica. Neurology 2006, 66:1485–1489. CrossRefPubMed Wingerchuk DM, Lennon VA, Pittock SJ, et al.: Revised diagnostic criteria for neuromyelitis optica. Neurology 2006, 66:1485–1489. CrossRefPubMed
11.
Zurück zum Zitat Lennon VA, Kryzer TJ, Pittock SJ, et al.: IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med 2005, 202:473–477. CrossRefPubMed Lennon VA, Kryzer TJ, Pittock SJ, et al.: IgG marker of optic-spinal multiple sclerosis binds to the aquaporin-4 water channel. J Exp Med 2005, 202:473–477. CrossRefPubMed
12.•
Zurück zum Zitat Matiello M, Lennon VA, Jacob A, et al.: NMO-IgG predicts the outcome of recurrent optic neuritis. Neurology 2008, 70:2197–2200. CrossRefPubMed Matiello M, Lennon VA, Jacob A, et al.: NMO-IgG predicts the outcome of recurrent optic neuritis. Neurology 2008, 70:2197–2200. CrossRefPubMed
13.••
Zurück zum Zitat Misu T, Fujihara K, Kakita A, et al.: Loss of aquaporin-4 in lesions in neuromyelitis optica: distinction from multiple sclerosis. Brain 2007, 130:1224–1234. CrossRefPubMed Misu T, Fujihara K, Kakita A, et al.: Loss of aquaporin-4 in lesions in neuromyelitis optica: distinction from multiple sclerosis. Brain 2007, 130:1224–1234. CrossRefPubMed
14.•
Zurück zum Zitat Roemer SF, Parisi JE, Lennon VA, et al.: Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis. Brain 2007, 130:1194–1205. CrossRefPubMed Roemer SF, Parisi JE, Lennon VA, et al.: Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis. Brain 2007, 130:1194–1205. CrossRefPubMed
15.••
Zurück zum Zitat Jarius S, Paul F, Franciotta D, et al.: Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis optica. Nat Clin Pract Neurol 2008, 4:202–214. PubMed Jarius S, Paul F, Franciotta D, et al.: Mechanisms of disease: aquaporin-4 antibodies in neuromyelitis optica. Nat Clin Pract Neurol 2008, 4:202–214. PubMed
16.•
Zurück zum Zitat Cree B: Neuromyelitis optica: diagnosis, pathogenesis, and treatment. Curr Neurol Neurosci Rep 2008, 8:427–433. CrossRefPubMed Cree B: Neuromyelitis optica: diagnosis, pathogenesis, and treatment. Curr Neurol Neurosci Rep 2008, 8:427–433. CrossRefPubMed
17.•
Zurück zum Zitat Bradl M, Misu T, Takahashi T, et al: Neuromyelitis optica: pathogenicity of patient immunoglobulin in vivo. Ann Neurol 2009, 66:630–643. CrossRefPubMed Bradl M, Misu T, Takahashi T, et al: Neuromyelitis optica: pathogenicity of patient immunoglobulin in vivo. Ann Neurol 2009, 66:630–643. CrossRefPubMed
18.••
Zurück zum Zitat Nicchia GP, Mastrototaro M, Rossi A, et al.: Aquaporin-4 orthogonal arrays of particles are the target for neuromyelitis optica autoantibodies. Glia 2009, 57:1363–1373. CrossRefPubMed Nicchia GP, Mastrototaro M, Rossi A, et al.: Aquaporin-4 orthogonal arrays of particles are the target for neuromyelitis optica autoantibodies. Glia 2009, 57:1363–1373. CrossRefPubMed
19.••
Zurück zum Zitat Bennett JL, Lam C, Kalluri SR, et al.: Intrathecal pathogenic anti-aquaporin-4 antibodies in early neuromyelitis optica. Ann Neurol 2009, 66:617–629. CrossRefPubMed Bennett JL, Lam C, Kalluri SR, et al.: Intrathecal pathogenic anti-aquaporin-4 antibodies in early neuromyelitis optica. Ann Neurol 2009, 66:617–629. CrossRefPubMed
20.
Zurück zum Zitat Sloka JS, Steffanelli M: The mechanism of action of methylprednisolone in the treatment of multiple sclerosis. Mult Scler 2005, 11( 4):425–432. CrossRefPubMed Sloka JS, Steffanelli M: The mechanism of action of methylprednisolone in the treatment of multiple sclerosis. Mult Scler 2005, 11( 4):425–432. CrossRefPubMed
21.
Zurück zum Zitat Braitch M, Harikrishnan S, Robins RA, et al.: Glucocorticoids increase CD4 + CD25 high cell percentage and Foxp3 expression in patients with multiple sclerosis. Acta Neurol Scand 2009, 119( 4):239–245. CrossRefPubMed Braitch M, Harikrishnan S, Robins RA, et al.: Glucocorticoids increase CD4 + CD25 high cell percentage and Foxp3 expression in patients with multiple sclerosis. Acta Neurol Scand 2009, 119( 4):239–245. CrossRefPubMed
22.
Zurück zum Zitat Goto H, Matsuo H, Nakane S, et al.: Plasmapheresis affects T helper type-1/T helper type-2 balance of circulating peripheral lymphocytes. Ther Apher 2001, 5:494–496. CrossRefPubMed Goto H, Matsuo H, Nakane S, et al.: Plasmapheresis affects T helper type-1/T helper type-2 balance of circulating peripheral lymphocytes. Ther Apher 2001, 5:494–496. CrossRefPubMed
23.
Zurück zum Zitat Lehmann HC, Hartung HP, Hetzel GR, et al.: Plasma exchange in neuroimmunological disorders. Part 1: Rationale and treatment of inflammatory central nervous system disorders. Arch Neurol 2006, 63:930–935. CrossRefPubMed Lehmann HC, Hartung HP, Hetzel GR, et al.: Plasma exchange in neuroimmunological disorders. Part 1: Rationale and treatment of inflammatory central nervous system disorders. Arch Neurol 2006, 63:930–935. CrossRefPubMed
24.
Zurück zum Zitat Weinshenker BG, O'Brien PC, Petterson TM, et al.: A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol 1999, 46:878–886. CrossRefPubMed Weinshenker BG, O'Brien PC, Petterson TM, et al.: A randomized trial of plasma exchange in acute central nervous system inflammatory demyelinating disease. Ann Neurol 1999, 46:878–886. CrossRefPubMed
25.
Zurück zum Zitat Keegan M, Pineda AA, McClelland RL, et al.: Plasma exchange for severe attacks of CNS demyelination: predictors of response. Neurology 2002, 58:143–146. PubMed Keegan M, Pineda AA, McClelland RL, et al.: Plasma exchange for severe attacks of CNS demyelination: predictors of response. Neurology 2002, 58:143–146. PubMed
26.•
Zurück zum Zitat Watanabe S, Nashima I, Misu T, et al.: Therapeutic efficacy of plasma exchange in NMO-IgG–positive patients with neuromyelitis optica. Mult Scler 2007, 13:128–132. CrossRefPubMed Watanabe S, Nashima I, Misu T, et al.: Therapeutic efficacy of plasma exchange in NMO-IgG–positive patients with neuromyelitis optica. Mult Scler 2007, 13:128–132. CrossRefPubMed
27.•
Zurück zum Zitat Bonnan M, Valentino R, Olindo S: Plasma exchange in severe spinal attacks associated with neuromyelitis optica spectrum disorder. Mult Scler 2009, 15:487–492. CrossRefPubMed Bonnan M, Valentino R, Olindo S: Plasma exchange in severe spinal attacks associated with neuromyelitis optica spectrum disorder. Mult Scler 2009, 15:487–492. CrossRefPubMed
28.
Zurück zum Zitat Llufriu S, Castillo J, Blanco Y, et al.: Plasma exchange for acute attacks of CNS demyelination: predictors of improvement. Neurology 2009, 73:949–953. CrossRefPubMed Llufriu S, Castillo J, Blanco Y, et al.: Plasma exchange for acute attacks of CNS demyelination: predictors of improvement. Neurology 2009, 73:949–953. CrossRefPubMed
29.
Zurück zum Zitat Aguilera AJ, Carlow TJ, Smith KJ, et al.: Lymphocytaplasmapheresis in Devic’s syndrome. Transfusion 1985, 25:54–56. CrossRefPubMed Aguilera AJ, Carlow TJ, Smith KJ, et al.: Lymphocytaplasmapheresis in Devic’s syndrome. Transfusion 1985, 25:54–56. CrossRefPubMed
30.
Zurück zum Zitat Nozaki I, Hamaguchi T, Komai K, et al.: Fulminant Devic disease successfully treated by lymphocytapheresis. J Neurol Neurosurg Psychiatry 2006, 77:1094–1095. CrossRefPubMed Nozaki I, Hamaguchi T, Komai K, et al.: Fulminant Devic disease successfully treated by lymphocytapheresis. J Neurol Neurosurg Psychiatry 2006, 77:1094–1095. CrossRefPubMed
31.
Zurück zum Zitat Papeix C, Vidal JS, De Seze J, et al.: Immunosuppressive therapy is more effective than interferon in neuromyelitis optica. Mult Scler 2007, 13:256–259. CrossRefPubMed Papeix C, Vidal JS, De Seze J, et al.: Immunosuppressive therapy is more effective than interferon in neuromyelitis optica. Mult Scler 2007, 13:256–259. CrossRefPubMed
32.•
Zurück zum Zitat Tanaka M, Tanaka K, Komori M: Interferonβ1b treatment in neuromyelitis optica. Eur Neurol 2009, 62:167–170. CrossRefPubMed Tanaka M, Tanaka K, Komori M: Interferonβ1b treatment in neuromyelitis optica. Eur Neurol 2009, 62:167–170. CrossRefPubMed
33.
Zurück zum Zitat Warabi Y, Matsumoto Y, Hayashi H: Interferon beta-1b exacerbates multiple sclerosis with severe optic nerve and spinal cord demyelination. J Neurol Sci 2007, 252:57–61. CrossRefPubMed Warabi Y, Matsumoto Y, Hayashi H: Interferon beta-1b exacerbates multiple sclerosis with severe optic nerve and spinal cord demyelination. J Neurol Sci 2007, 252:57–61. CrossRefPubMed
34.
Zurück zum Zitat Shimizu Y, Yokoyama K, Misu T, et al.: Development of extensive brain lesions following interferon beta therapy in relapsing neuromyelitis optica and longitudinally extensive myelitis. J Neurol 2008, 255:305–307. CrossRefPubMed Shimizu Y, Yokoyama K, Misu T, et al.: Development of extensive brain lesions following interferon beta therapy in relapsing neuromyelitis optica and longitudinally extensive myelitis. J Neurol 2008, 255:305–307. CrossRefPubMed
35.
Zurück zum Zitat Mandler RN, Ahmed W, Dencoff JE: Devic’s neuromyelitis optica: a prospective study of seven patients treated with prednisone and azathioprine. Neurology 1998, 51:1219–1220. PubMed Mandler RN, Ahmed W, Dencoff JE: Devic’s neuromyelitis optica: a prospective study of seven patients treated with prednisone and azathioprine. Neurology 1998, 51:1219–1220. PubMed
36.•
Zurück zum Zitat Jacob A, Matiello M, Weinshenker BG, Wingerchuk DM: Treatment of neuromyelitis optica with mycophenolate mofetil. Retrospective analysis of 24 patients. Arch Neurol 2009, 66:1128–1133. CrossRefPubMed Jacob A, Matiello M, Weinshenker BG, Wingerchuk DM: Treatment of neuromyelitis optica with mycophenolate mofetil. Retrospective analysis of 24 patients. Arch Neurol 2009, 66:1128–1133. CrossRefPubMed
37.•
Zurück zum Zitat Jacob A, Weinshenker B, Violich I, et al.: Treatment of neuromyelitis optica with rituximab: retrospective analysis of 25 patients. Arch Neurol 2008, 65:1443–1448. CrossRefPubMed Jacob A, Weinshenker B, Violich I, et al.: Treatment of neuromyelitis optica with rituximab: retrospective analysis of 25 patients. Arch Neurol 2008, 65:1443–1448. CrossRefPubMed
38.
Zurück zum Zitat Kim HJ, Kim W, Park MS, et al.: B cell depletion therapy using rituximab in neuromyelitis optica spectrum disorders. Mult Scler 2009, 15:S253. CrossRef Kim HJ, Kim W, Park MS, et al.: B cell depletion therapy using rituximab in neuromyelitis optica spectrum disorders. Mult Scler 2009, 15:S253. CrossRef
39.
Zurück zum Zitat 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. PubMed 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. PubMed
40.
Zurück zum Zitat Weinstock-Guttman B, Ramanathan M, Lincoff N, et al.: Study of mitoxantrone for the treatment of neuromyelitis optica (Devic disease). Arch Neurol 2006, 63:957–963. CrossRefPubMed Weinstock-Guttman B, Ramanathan M, Lincoff N, et al.: Study of mitoxantrone for the treatment of neuromyelitis optica (Devic disease). Arch Neurol 2006, 63:957–963. CrossRefPubMed
41.•
Zurück zum Zitat Watanabe S, Misu T, Miyazama I, et al.: Low-dose corticosteroids reduce relapses in neuromyelitis optica: a retrospective analysis. Mult Scler 2007, 13:968–974. CrossRefPubMed Watanabe S, Misu T, Miyazama I, et al.: Low-dose corticosteroids reduce relapses in neuromyelitis optica: a retrospective analysis. Mult Scler 2007, 13:968–974. CrossRefPubMed
42.
Zurück zum Zitat Okada K, Tsuji S, Takana K: Intermittent intravenous immunoglobulin successfully prevents relapses of neuromyelitis optica. Intern Med 2007, 46:1671–1672. CrossRefPubMed Okada K, Tsuji S, Takana K: Intermittent intravenous immunoglobulin successfully prevents relapses of neuromyelitis optica. Intern Med 2007, 46:1671–1672. CrossRefPubMed
43.
Zurück zum Zitat Bakker J, Metz L: Devic’s neuromyelitis optica treated with intravenous gamma globulin (IVIG). Can J Neurol Sci 2004, 31:265–267. PubMed Bakker J, Metz L: Devic’s neuromyelitis optica treated with intravenous gamma globulin (IVIG). Can J Neurol Sci 2004, 31:265–267. PubMed
44.
Zurück zum Zitat Mok CC, To CH, Mak A, Poon WL: Immunoablative cyclophosphamide for refractory lupus-related neuromyelitis optica. J Rheumatol 2008, 35:172–174. PubMed Mok CC, To CH, Mak A, Poon WL: Immunoablative cyclophosphamide for refractory lupus-related neuromyelitis optica. J Rheumatol 2008, 35:172–174. PubMed
45.
Zurück zum Zitat Nasir S, Kerr D, Birnbaum J: Nineteen episodes of recurrent myelitis in a woman with neuromyelitis optica and systemic lupus erythematosus. Arch Neurol 2009, 66:1160–1163. CrossRefPubMed Nasir S, Kerr D, Birnbaum J: Nineteen episodes of recurrent myelitis in a woman with neuromyelitis optica and systemic lupus erythematosus. Arch Neurol 2009, 66:1160–1163. CrossRefPubMed
46.
Zurück zum Zitat Petelin Gadze Z, Hajnsek S, Basic S, et al.: Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report. BMC Neurol 2009, 9:56. CrossRefPubMed Petelin Gadze Z, Hajnsek S, Basic S, et al.: Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report. BMC Neurol 2009, 9:56. CrossRefPubMed
48.
Zurück zum Zitat Meinl E, Krumbholz M, Hohlfeld R: B lineage cells in the inflammatory central nervous system environment: migration, maintenance, local antibody production and therapeutic modulation. Ann Neurol 2006, 59:880–892. CrossRefPubMed Meinl E, Krumbholz M, Hohlfeld R: B lineage cells in the inflammatory central nervous system environment: migration, maintenance, local antibody production and therapeutic modulation. Ann Neurol 2006, 59:880–892. CrossRefPubMed
50.
Zurück zum Zitat Warth A: Prevention of orthogonal array of particles formation as a treatment approach for neuromyelitis optica. Med Hypotheses 2009, 73:361–362. CrossRefPubMed Warth A: Prevention of orthogonal array of particles formation as a treatment approach for neuromyelitis optica. Med Hypotheses 2009, 73:361–362. CrossRefPubMed
51.••
Zurück zum Zitat Dale RC, Brilot F, Banwell B: Pediatric central nervous system inflammatory demyelination: acute disseminated encephalomyelitis, clinically isolated syndromes, neuromyelitis optica, and multiple sclerosis. Curr Opin Neurol 2009, 22:233–240. CrossRefPubMed Dale RC, Brilot F, Banwell B: Pediatric central nervous system inflammatory demyelination: acute disseminated encephalomyelitis, clinically isolated syndromes, neuromyelitis optica, and multiple sclerosis. Curr Opin Neurol 2009, 22:233–240. CrossRefPubMed
Metadaten
Titel
Neuromyelitis Optica
verfasst von
MBBS, MD William M. Carroll
MD Kazuo Fujihara
Publikationsdatum
01.05.2010
Verlag
Current Science Inc.
Erschienen in
Current Treatment Options in Neurology / Ausgabe 3/2010
Print ISSN: 1092-8480
Elektronische ISSN: 1534-3138
DOI
https://doi.org/10.1007/s11940-010-0071-z

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