This Article  • Full text  • PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (7)  • Contact me when this article is cited  Related Content  • Related letters  • Similar articles in this journal  Topic Collections  • Neuro-ophthalmology  • Neurology, Other  • Otolaryngology/ Head & Neck Surgery  • Inflammatory Disease of Head & Neck  • Musculoskeletal Syndromes (Chronic Fatigue, Gulf War)  • Rheumatology, Other  • Immunologic Disorders  • Alert me on articles by topic  Social Bookmarking   What's this?

Sean J. Pittock, MD; Vanda A. Lennon, MD, PhD; Jerome de Seze, MD; Patrick Vermersch, MD; Henry A. Homburger, MD; Dean M. Wingerchuk, MD; Claudia F. Lucchinetti, MD; Hélène Zéphir, MD; Kevin Moder, MD; Brian G. Weinshenker, MD

Arch Neurol. 2008;65(1):78-83.

Background  Neuromyelitis optica (NMO) is often associated with other clinical or serological markers of non–organ-specific autoimmunity.

Objective  To evaluate the relationship between NMO spectrum disorders (NMOSDs), including NMO, longitudinally extensive transverse myelitis, and recurrent optic neuritis, and autoimmune disease. We concentrated on the association with systemic lupus erythematosus (SLE), Sjögren syndrome (SS), or serological evidence of these disorders, which commonly is a source of diagnostic confusion.

Design  Retrospective blinded serological survey.

Setting  Mayo Clinic College of Medicine, Rochester, and Centre Hospitalier Régional Universitaire de Lille.

Methods  Group 1 included 153 US patients with NMOSDs (78 with NMO and 75 with longitudinally extensive transverse myelitis) and 33 control subjects with SS/SLE. Group 2 included 30 French patients with SS/SLE, 14 with NMOSDs (6 with NMO, 6 with longitudinally extensive transverse myelitis, and 2 with recurrent optic neuritis), 16 without NMOSDs, and 4 with NMO without SS/SLE.

Results  For group 1, NMO-IgG was detected in 66.7%, antinuclear antibodies in 43.8%, and Sjögren syndrome A (SSA) antibodies in 15.7% of patients with NMO and longitudinally extensive transverse myelitis. Five NMO-IgG–seropositive patients with NMOSDs had coexisting SLE, SS, or both. Antinuclear antibodies and SSA antibodies were more frequent in NMO-IgG–seropositive patients than in NMO-IgG–seronegative patients (P = .001). For group 2, NMO-IgG was detected in 5 of 14 patients (35.7%) with NMOSDs and SS/SLE and in 2 of 4 patients (50.0%) with NMO without SS/SLE (P = .59). We detected NMO-IgG only in patients with NMOSDs and not in 49 controls with SS/SLE but without optic neuritis or myelitis from the 2 cohorts (P = .01).

Conclusion  Neuromyelitis optica spectrum disorders with seropositive findings for NMO-IgG occurring with SS/SLE or non–organ-specific autoantibodies is an indication of coexisting NMO rather than a vasculopathic or other complication of SS/SLE.

Author Affiliations: Departments of Neurology (Drs Pittock, Lennon, Lucchinetti, and Weinshenker), Laboratory Medicine and Pathology (Drs Pittock, Lennon, and Homburger), Immunology (Dr Lennon), and Rheumatology (Dr Moder), Mayo Clinic, Rochester, Minnesota; and Departments of Neurology, Hospitale Universitaire de Strasbourg, Strasbourg, France (Dr de Seze); Centre Hospitalier Régional Universitaire de Lille, Lille, France (Drs Vermersch and Zéphir); and Mayo Clinic, Scottsdale, Arizona (Dr Wingerchuk).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

RELATED LETTERS

Neuromyelitis Optica With Coexisting Autoimmune Disorders
Konstantina G. Yiannopoulou, Athina K. Efthymiou, and Kleanthis D. Karydakis
Arch Neurol. 2008;65(7):995.
EXTRACT | FULL TEXT  

Neuromyelitis Optica and Autoimmune Diseases
Jorge C. Kattah and Meena Gujrati
Arch Neurol. 2008;65(7):995-996.
EXTRACT | FULL TEXT  

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Sjogren's syndrome myelopathy: spinal cord involvement in Sjogren's syndrome might be a manifestation of neuromyelitis optica
Kim et al.
Mult Scler 2009;15:1062-1068.
ABSTRACT  

Brain abnormalities in Sjogren syndrome with recurrent CNS manifestations: association with neuromyelitis optica
Min et al.
Mult Scler 2009;15:1069-1076.
ABSTRACT  

Neuromyelitis optica positive antibodies confer a worse course in relapsing-neuromyelitis optica in Cuba and French West Indies
Cabrera-Gomez et al.
Mult Scler 2009;15:828-833.
ABSTRACT  

Neuromyelitis optica in Brazil: a study on clinical and prognostic factors
Bichuetti et al.
Mult Scler 2009;15:613-619.
ABSTRACT  

Differential diagnosis of suspected multiple sclerosis: a consensus approach
Miller et al.
Mult Scler 2008;14:1157-1174.
ABSTRACT  

Aquaporin-4-binding autoantibodies in patients with neuromyelitis optica impair glutamate transport by down-regulating EAAT2
Hinson et al.
JEM 2008;205:2473-2481.
ABSTRACT | FULL TEXT  

Neuromyelitis optica in patients with gluten sensitivity associated with antibodies to aquaporin-4
Jarius et al.
J. Neurol. Neurosurg. Psychiatry 2008;79:1084-1084.
FULL TEXT  

CNS aquaporin-4 autoimmunity in children
McKeon et al.
Neurology 2008;71:93-100.
ABSTRACT | FULL TEXT  

Neuromyelitis Optica With Coexisting Autoimmune Disorders
Yiannopoulou et al.
Arch Neurol 2008;65:995-995.
FULL TEXT  

Neuromyelitis Optica and Autoimmune Diseases
Kattah and Gujrati
Arch Neurol 2008;65:995-996.
FULL TEXT  

A devilishly interesting case
Hewett and Coleman
PN 2008;8:186-190.
FULL TEXT  

Aquaporin-4 Autoantibodies in a Paraneoplastic Context
Pittock and Lennon
Arch Neurol 2008;65:629-632.
ABSTRACT | FULL TEXT