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Volume 66, Number 10, May 23, 2006
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NEUROLOGY 2006;66:1485-1489
© 2006 American Academy of Neurology

Revised diagnostic criteria for neuromyelitis optica

D. M. Wingerchuk, MD, FRCP(C), V. A. Lennon, MD, PhD, S. J. Pittock, MD, C. F. Lucchinetti, MD and B. G. Weinshenker, MD, FRCP(C)

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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