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From the Department of Neurology (D.M.W.), Mayo Clinic College of Medicine, Scottsdale, AZ; and Departments of Neurology (V.A.L., S.J.P., C.F.L., B.G.W.), Laboratory Medicine and Pathology (V.A.L., S.J.P.), and Immunology (V.A.L.), Mayo Clinic College of Medicine, Rochester, MN.
Address correspondence and reprint requests to Dr. Dean M. Wingerchuk, Department of Neurology, Mayo Clinic College of Medicine, 13400 East Shea Boulevard, Scottsdale, AZ 85259; e-mail: wingerchuk.dean{at}mayo.edu
Background: The authors previously proposed diagnostic criteria for neuromyelitis optica (NMO) that facilitate its distinction from prototypic multiple sclerosis (MS). However, some patients with otherwise typical NMO have additional symptoms not attributable to optic nerve or spinal cord inflammation or have MS-like brain MRI lesions. Furthermore, some patients are misclassified as NMO by the authors’ earlier proposed criteria despite having a subsequent course indistinguishable from prototypic MS. A serum autoantibody marker, NMO-IgG, is highly specific for NMO. The authors propose revised NMO diagnostic criteria that incorporate NMO-IgG status.
Methods: Using final clinical diagnosis (NMO or MS) as the reference standard, the authors calculated sensitivity and specificity for each criterion and various combinations using a sample of 96 patients with NMO and 33 with MS. The authors used likelihood ratios and logistic regression analysis to develop the most practical and informative diagnostic model.
Results: Fourteen patients with NMO (14.6%) had extra-optic-spinal CNS symptoms. NMO-IgG seropositivity was 76% sensitive and 94% specific for NMO. The best diagnostic combination was 99% sensitive and 90% specific for NMO and consisted of at least two of three elements: longitudinally extensive cord lesion, onset brain MRI nondiagnostic for MS, or NMO-IgG seropositivity.
Conclusions: The authors propose revised diagnostic criteria for definite neuromyelitis optica (NMO) that require optic neuritis, myelitis, and at least two of three supportive criteria: MRI evidence of a contiguous spinal cord lesion 3 or more segments in length, onset brain MRI nondiagnostic for multiple sclerosis, or NMO-IgG seropositivity. CNS involvement beyond the optic nerves and spinal cord is compatible with NMO.
Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the May 23 issue to find the title link for this article.
Editorial, see page 1466
See also page 1568
Disclosure: The authors report no conflicts of interest.
Received October 25, 2005. Accepted in final form January 13, 2006.
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F. Blanc, H. Zephir, C. Lebrun, P. Labauge, G. Castelnovo, M. Fleury, F. Sellal, C. Tranchant, K. Dujardin, P. Vermersch, et al. Cognitive Functions in Neuromyelitis Optica Arch Neurol, January 1, 2008; 65(1): 84 - 88. [Abstract] [Full Text] [PDF]
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R. M. Papais-Alvarenga, S. C. Carellos, M. P. Alvarenga, C. Holander, R. P. Bichara, and L. C. S. Thuler Clinical Course of Optic Neuritis in Patients With Relapsing Neuromyelitis Optica Arch Ophthalmol, January 1, 2008; 126(1): 12 - 16. [Abstract] [Full Text] [PDF]
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C. C. Glisson and S. L. Galetta Is Neuromyelitis Optica Eyeing a Distinct Path From Multiple Sclerosis? Arch Ophthalmol, January 1, 2008; 126(1): 128 - 129. [Full Text] [PDF]
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E. E. Benarroch Aquaporin-4, homeostasis, and neurologic disease Neurology, December 11, 2007; 69(24): 2266 - 2268. [Full Text] [PDF]
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M. Tanaka, K. Tanaka, M. Komori, and T. Saida Anti-aquaporin 4 antibody in Japanese multiple sclerosis: the presence of optic spinal multiple sclerosis without long spinal cord lesions and anti-aquaporin 4 antibody J. Neurol. Neurosurg. Psychiatry, September 1, 2007; 78(9): 990 - 992. [Abstract] [Full Text] [PDF]
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D. Yuksel, N. Senbil, D. Yilmaz, and Y.K. Yavuz Gurer Devic's Neuromyelitis Optica in an Infant Case J Child Neurol, September 1, 2007; 22(9): 1143 - 1146. [Abstract] [PDF]
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S. Watanabe, T. Misu, I. Miyazawa, I. Nakashima, Y. Shiga, K. Fujihara, and Y. Itoyama Low-dose corticosteroids reduce relapses in neuromyelitis optica: a retrospective analysis Multiple Sclerosis, September 1, 2007; 13(8): 968 - 974. [Abstract] [PDF]
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K. Tanaka, T. Tani, M. Tanaka, T. Saida, J. Idezuka, M. Yamazaki, M. Tsujita, T. Nakada, K. Sakimura, and M. Nishizawa Anti-aquaporin 4 antibody in selected Japanese multiple sclerosis patients with long spinal cord lesions Multiple Sclerosis, August 1, 2007; 13(7): 850 - 855. [Abstract] [PDF]
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T. Braley and D. D. Mikol Neuromyelitis Optica in a Mother and Daughter Arch Neurol, August 1, 2007; 64(8): 1189 - 1192. [Abstract] [Full Text] [PDF]
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B. G. Weinshenker Neuromyelitis Optica Is Distinct From Multiple Sclerosis Arch Neurol, June 1, 2007; 64(6): 899 - 901. [Full Text] [PDF]
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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. [Full Text] [PDF]
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E. M. Frohman and D. Kerr Is Neuromyelitis Optica Distinct From Multiple Sclerosis?: Something for "Lumpers" and "Splitters" Arch Neurol, June 1, 2007; 64(6): 903 - 905. [Full Text] [PDF]
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A. Jacob and S. J. Pittock Letter To the Editor Multiple Sclerosis, June 1, 2007; 13(5): 686 - 686. [PDF]
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A. Compston Complexity and heterogeneity in demyelinating disease Brain, May 1, 2007; 130(5): 1178 - 1180. [Full Text] [PDF]
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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. [Abstract] [Full Text] [PDF]
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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. [Abstract] [Full Text] [PDF]
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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. [Abstract] [Full Text] [PDF]
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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. [Full Text] [PDF]
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B. G. Weinshenker, A. Jacob, I. Kister, J. Herbert, M. L. Swerdlow, R. Bergamaschi, G. Piccolo, J. Oger, K. Ikeda, Y. Araki, et al. Occurrence of CNS demyelinating disease in patients with myasthenia gravis. Neurology, April 17, 2007; 68(16): 1326 - 1326. [Full Text] [PDF]
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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. [Abstract] [Full Text] [PDF]
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B. Banwell, M. Shroff, J. M. Ness, D. Jeffery, S. Schwid, B. Weinstock-Guttman, and for the International Pediatric MS Study Group MRI features of pediatric multiple sclerosis Neurology, April 17, 2007; 68(16_suppl_2): S46 - S53. [Abstract] [Full Text] [PDF]
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