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Neurology 1999;53:1107
© 1999 American Academy of Neurology
Articles

The clinical course of neuromyelitis optica (Devic’s syndrome)

Dean M. Wingerchuk, MD, FRCP(C), William F. Hogancamp, MD, Peter C. O’Brien, PhD and Brian G. Weinshenker, MD, FRCP(C)

From the Department of Neurology (Drs. Wingerchuk, Hogancamp, and Weinshenker) and Health Sciences Research (Dr. O’Brien), Mayo Clinic and Foundation, Rochester, MN.

Address correspondence and reprint requests to Dr. Brian G. Weinshenker, Dept. of Neurology, West 8A, Mayo Clinic, 200 First Street SW, Rochester, MN 55905.

OBJECTIVES: To evaluate the spectrum of neuromyelitis optica (NMO), including characteristics of the index events (optic neuritis [ON]) and myelitis), neuroimaging, CSF, and serologic studies, and to evaluate the long-term course.

METHODS: Review of 71 patients with NMO evaluated at the Mayo Clinic between 1950 and 1997.

RESULTS: NMO was either monophasic or relapsing. Patients with a monophasic course (n = 23) usually presented with rapidly sequential index events (median 5 days) with moderate recovery. Most with a relapsing course (n = 48) had an extended interval between index events (median 166 days) followed within 3 years by clusters of severe relapses isolated to the optic nerves and spinal cord. Most relapsing patients developed severe disability in a stepwise manner, and one-third died because of respiratory failure. Features of NMO distinct from "typical" MS included >50 cells/mm3 in CSF (often polymorphonuclear), normal initial brain MRI, and lesions extending over three or more vertebral segments on spinal cord MRI.

CONCLUSIONS: Clinical, laboratory, and imaging features generally distinguish neuromyelitis optica from MS. Patients with relapsing optic neuritis and myelitis may have neuromyelitis optica rather than MS. Patients with a relapsing course of neuromyelitis optica have a poor prognosis and frequently develop respiratory failure during attacks of cervical myelitis.

Key words: Neuromyelitis optica—Devic’s syndrome—Prognosis—Optic neuritis—Myelitis—MS.




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S. L. Galetta and J. Bennett
Neuromyelitis Optica Is a Variant of Multiple Sclerosis
Arch Neurol, June 1, 2007; 64(6): 901 - 903.
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G.J.D. Hengstman, P. Wesseling, C.W.G.M. Frenken, and P.J.H. Jongen
Neuromyelitis optica with clinical and histopathological involvement of the brain
Multiple Sclerosis, June 1, 2007; 13(5): 679 - 682.
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A. Compston
Complexity and heterogeneity in demyelinating disease
Brain, May 1, 2007; 130(5): 1178 - 1180.
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S. F. Roemer, J. E. Parisi, V. A. Lennon, E. E. Benarroch, H. Lassmann, W. Bruck, R. N. Mandler, B. G. Weinshenker, S. J. Pittock, D. M. Wingerchuk, et al.
Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis
Brain, May 1, 2007; 130(5): 1194 - 1205.
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T. Misu, K. Fujihara, A. Kakita, H. Konno, M. Nakamura, S. Watanabe, T. Takahashi, I. Nakashima, H. Takahashi, and Y. Itoyama
Loss of aquaporin 4 in lesions of neuromyelitis optica: distinction from multiple sclerosis
Brain, May 1, 2007; 130(5): 1224 - 1234.
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T. Matsuoka, T. Matsushita, Y. Kawano, M. Osoegawa, H. Ochi, T. Ishizu, M. Minohara, H. Kikuchi, F. Mihara, Y. Ohyagi, et al.
Heterogeneity of aquaporin-4 autoimmunity and spinal cord lesions in multiple sclerosis in Japanese
Brain, May 1, 2007; 130(5): 1206 - 1223.
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T. Takahashi, K. Fujihara, I. Nakashima, T. Misu, I. Miyazawa, M. Nakamura, S. Watanabe, Y. Shiga, C. Kanaoka, J. Fujimori, et al.
Anti-aquaporin-4 antibody is involved in the pathogenesis of NMO: a study on antibody titre
Brain, May 1, 2007; 130(5): 1235 - 1243.
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B. L. Banwell
The long (-itudinally extensive) and the short of it: Transverse myelitis in children
Neurology, May 1, 2007; 68(18): 1447 - 1449.
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J. M. Ness, D. Chabas, A. D. Sadovnick, D. Pohl, B. Banwell, B. Weinstock-Guttman, and for the International Pediatric MS Study Group
Clinical features of children and adolescents with multiple sclerosis
Neurology, April 17, 2007; 68(16_suppl_2): S37 - S45.
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M. Nakamura, I. Nakashima, S. Sato, I. Miyazawa, K. Fujihara, and Y. Itoyama
Clinical and laboratory features of neuromyelitis optica with oligoclonal IgG bands
Multiple Sclerosis, April 1, 2007; 13(3): 332 - 335.
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J.A. Cabrera-Gomez, J. Galarraga-Inza, R.M. Coro-Antich, Y. Real-Gonzalez, M. Cristofol-Corominas, H. Gomez, K. Romero-Garcia, M.X. Gil-Gil, and A. Gonzalez-Quevedo
Down's syndrome and neuromyelitis optica (Devic's disease). An autopsy-proven case
Multiple Sclerosis, April 1, 2007; 13(3): 433 - 436.
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S. Jarius, D. Franciotta, R. Bergamaschi, H. Wright, E. Littleton, J. Palace, R. Hohlfeld, and A. Vincent
NMO-IgG in the diagnosis of neuromyelitis optica
Neurology, March 27, 2007; 68(13): 1076 - 1077.
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I. Miyazawa, I. Nakashima, A. Petzold, K. Fujihara, S. Sato, and Y. Itoyama
High CSF neurofilament heavy chain levels in neuromyelitis optica
Neurology, March 13, 2007; 68(11): 865 - 867.
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C Papeix, J-S Vidal, J de Seze, C Pierrot-Deseilligny, A Tourbah, B Stankoff, C Lebrun, T Moreau, P Vermersch, B Fontaine, et al.
Immunosuppressive therapy is more effective than interferon in neuromyelitis optica
Multiple Sclerosis, March 1, 2007; 13(2): 256 - 259.
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T Fukazawa and S Kikuchi
A three-dimensional approach for understanding the spectrum of idiopathic inflammatory demyelinating disorders: importance of the 'attack-related severity' axis
Multiple Sclerosis, March 1, 2007; 13(2): 199 - 207.
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J. A Cabrera-Gomez, L Quevedo-Sotolongo, A Gonzalez-Quevedo, S Lima, Y Real-Gonzalez, M Cristofol-Corominas, K Romero-Garcia, C Ugarte-Sanchez, J Jordan-Gonzalez, J E G. de la Nuez, et al.
Brain magnetic resonance imaging findings in relapsing neuromyelitis optica
Multiple Sclerosis, March 1, 2007; 13(2): 186 - 192.
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D. M. Wingerchuk, S. J. Pittock, C. F. Lucchinetti, V. A. Lennon, and B. G. Weinshenker
A secondary progressive clinical course is uncommon in neuromyelitis optica
Neurology, February 20, 2007; 68(8): 603 - 605.
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S Watanabe, I Nakashima, T Misu, I Miyazawa, Y Shiga, K Fujihara, and Y Itoyama
Therapeutic efficacy of plasma exchange in NMO-IgG-positive patients with neuromyelitis optica
Multiple Sclerosis, January 1, 2007; 13(1): 128 - 132.
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T. F. Scott, S. L. Kassab, and S. J. Pittock
Neuromyelitis optica IgG status in acute partial transverse myelitis.
Arch Neurol, October 1, 2006; 63(10): 1398 - 1400.
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D Petravic, M Habek, S Supe, and V V Brinar
Recurrent optic neuromyelitis with endocrinopathies: a new syndrome or just a coincidence?
Multiple Sclerosis, September 1, 2006; 12(5): 670 - 673.
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I Nakashima, K Fujihara, I Miyazawa, T Misu, K Narikawa, M Nakamura, S Watanabe, T Takahashi, S Nishiyama, Y Shiga, et al.
Clinical and MRI features of Japanese patients with multiple sclerosis positive for NMO-IgG
J. Neurol. Neurosurg. Psychiatry, September 1, 2006; 77(9): 1073 - 1075.
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G Giovannoni
Neuromyelitis optica and anti-aquaporin-4 antibodies: widening the clinical phenotype
J. Neurol. Neurosurg. Psychiatry, September 1, 2006; 77(9): 1001 - 1002.
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