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Published on: August 21, 2017
Revised diagnostic criteria for neuromyelitis optica
D M Wingerchuk1, V A Lennon, S J Pittock
1Department of Neurology, Mayo Clinic College of Medicine, Scottsdale, AZ 85259, USA. wingerchuk.dean@mayo.edu
Neurology
|May 24, 2006
Summary
Revised diagnostic criteria for neuromyelitis optica (NMO) now incorporate NMO-IgG seropositivity, improving accuracy. These updated guidelines enhance the distinction between NMO and multiple sclerosis (MS).
Area of Science:
- Neurology
- Immunology
- Diagnostic Criteria Development
Background:
- Previous neuromyelitis optica (NMO) diagnostic criteria had limitations in distinguishing NMO from multiple sclerosis (MS).
- Some NMO patients exhibit symptoms beyond optic nerve and spinal cord inflammation or present with MS-like brain MRI lesions.
- A highly specific serum autoantibody, NMO-IgG, has emerged as a key marker for NMO.
Purpose of the Study:
- To propose revised diagnostic criteria for definite neuromyelitis optica (NMO).
- To integrate NMO-IgG serostatus into diagnostic criteria for improved accuracy.
- To enhance the differentiation between NMO and multiple sclerosis (MS).
Main Methods:
- Sensitivity and specificity were calculated for individual criteria and combinations.
- A reference standard of final clinical diagnosis (NMO or MS) was used.
- Likelihood ratios and logistic regression analysis informed the diagnostic model development using data from 96 NMO and 33 MS patients.
Main Results:
- NMO-IgG seropositivity demonstrated 76% sensitivity and 94% specificity for NMO.
- Fourteen NMO patients (14.6%) presented with extra-optic-spinal central nervous system (CNS) symptoms.
- The optimal diagnostic combination (99% sensitive, 90% specific for NMO) included at least two of: longitudinally extensive cord lesion, onset brain MRI nondiagnostic for MS, or NMO-IgG seropositivity.
Conclusions:
- Revised criteria for definite NMO require optic neuritis, myelitis, and at least two supportive criteria.
- Supportive criteria include: MRI evidence of a contiguous spinal cord lesion ≥3 segments, onset brain MRI nondiagnostic for MS, or NMO-IgG seropositivity.
- Central nervous system involvement beyond optic nerves and spinal cord is compatible with NMO.
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