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 (85)  • Contact me when this article is cited  Related Content  • Related letters  • Similar articles in this journal  Topic Collections  • Neuro-ophthalmology  • Alert me on articles by topic  Social Bookmarking   What's this?

Sean J. Pittock, MD; Vanda A. Lennon, MD, PhD; Karl Krecke, MD; Dean M. Wingerchuk, MD; Claudia F. Lucchinetti, MD; Brian G. Weinshenker, MD

Arch Neurol. 2006;63:390-396.

Background  Neuromyelitis optica (NMO) is a severe demyelinating disease defined principally by its tendency to selectively affect optic nerves and the spinal cord causing recurrent attacks of blindness and paralysis. Contemporary diagnostic criteria require absence of clinical disease outside the optic nerve or spinal cord. We have, however, frequently encountered patients with a well-established diagnosis of NMO in whom either asymptomatic or symptomatic brain lesions develop suggesting that the diagnostic criteria for NMO should be revised.

Objective  To describe the magnetic resonance image (MRI) brain findings in NMO.

Design  Observational, retrospective case series.

Patients  We ascertained patients through a clinical biospecimens database of individuals with definite or suspected NMO. We included patients who (1) satisfied the 1999 criteria of Wingerchuk et al for NMO except for the absolute criterion of lacking symptoms beyond the optic nerve and spinal cord and the supportive criterion of having a normal brain MRI at onset; (2) had MRI evidence of a spinal cord lesion extending 3 vertebral segments or more (the most specific nonserological feature to differentiate NMO from MS); and (3) were evaluated neurologically and by brain MRI at the Mayo Clinic.

Main Outcome Measures  Magnetic resonance images were classified as normal or as abnormal with either nonspecific, multiple sclerosis–like or atypical abnormalities. We evaluated whether brain lesions were symptomatic and analyzed the neuropathologic features of a single brain biopsy specimen.

Results  Sixty patients (53 women [88%]) fulfilled these inclusion criteria. The mean ± SD age at onset was 37.2 ± 18.4 years and the mean ± SD duration of follow-up was 6.0 ± 5.6 years. Neuromyelitis optica–IgG was detected in 41 patients (68%). Brain MRI lesions were detected in 36 patients (60%). Most were nonspecific, but 6 patients (10%) had multiple sclerosis–like lesions, usually asymptomatic. Another 5 patients (8%), mostly children, had diencephalic, brainstem or cerebral lesions, atypical for multiple sclerosis. When present, symptoms of brain involvement were subtle, except in 1 patient who was comatose and had large cerebral lesions.

Conclusions  Asymptomatic brain lesions are common in NMO, and symptomatic brain lesions do not exclude the diagnosis of NMO. These observations justify revision of diagnostic criteria for NMO to allow for brain involvement.

Author Affiliations: From the Departments of Neurology (Drs Pittock, Lennon, Lucchinetti, and Weinshenker), Laboratory Medicine and Pathology (Drs Pittock and Lennon), Radiology (Dr Krecke), and Immunology (Dr Lennon), Mayo Clinic College of Medicine, Rochester, Minn; and the Department of Neurology, Mayo Clinic College of Medicine, Scottsdale, Ariz (Dr Wingerchuk).

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

RELATED LETTERS

Devic Disease With Abnormal Brain Magnetic Resonance Image Findings: The First Portuguese Case
Joana Guimarães and Maria José Sá
Arch Neurol. 2007;64(2):290-291.
EXTRACT | FULL TEXT  

Devic Disease With Abnormal Brain Magnetic Resonance Image Findings: The First Portuguese Case—Reply
Brian G. Weinshenker
Arch Neurol. 2007;64(2):291.
EXTRACT | FULL TEXT  

A Case of Neuromyelitis Optica With Gadolinium-Enhancing Brain Lesions and Parinaud Syndrome—Reply
Marc Gotkine
Arch Neurol. 2009;66(1):140-141.
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  

Extensive vasogenic edema of anti-aquaporin-4 antibody-related brain lesions
Matsushita et al.
Mult Scler 2009;15:1113-1117.
ABSTRACT  

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

Aquaporin-4 autoimmune syndrome and anti-aquaporin-4 antibody-negative opticospinal multiple sclerosis in Japanese
Matsushita et al.
Mult Scler 2009;15:834-847.
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  

Symptomatic, radiological and pathological involvement of the hypothalamus in neuromyelitis optica
Viegas et al.
J. Neurol. Neurosurg. Psychiatry 2009;80:679-682.
ABSTRACT | FULL TEXT  

Occurrence of acute large and edematous callosal lesions in neuromyelitis optica
Nakamura et al.
Mult Scler 2009;15:695-700.
ABSTRACT  

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

Is neuromyelitis optica associated with human leukocyte antigen?
Zephir et al.
Mult Scler 2009;15:571-579.
ABSTRACT  

Hypercomplementemia at relapse in patients with anti-aquaporin-4 antibody
Doi et al.
Mult Scler 2009;15:304-310.
ABSTRACT  

What insights have new imaging techniques given into aggressive forms of MS - different forms of MS or different from MS?
Pichiecchio et al.
Mult Scler 2009;15:285-293.
ABSTRACT  

Posterior reversible encephalopathy syndrome in neuromyelitis optica spectrum disorders
Magana et al.
Neurology 2009;72:712-717.
ABSTRACT | FULL TEXT  

Hypocretinergic dysfunction in neuromyelitis optica with coma-like episodes
Carlander et al.
BMJ Case Reports 2009;2009:bcr0820080709-bcr0820080709.
ABSTRACT | FULL TEXT  

Neuromyelitis Optica and Concentric Rings of Balo in the Brainstem
Graber et al.
Arch Neurol 2009;66:274-275.
FULL TEXT  

A Case of Neuromyelitis Optica With Gadolinium-Enhancing Brain Lesions and Parinaud Syndrome--Reply
Gotkine
Arch Neurol 2009;66:140-141.
FULL TEXT  

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

Spectrum of Pediatric Neuromyelitis Optica
Lotze et al.
Pediatrics 2008;122:e1039-e1047.
ABSTRACT | FULL TEXT  

Characterisation of the spectrum of demyelinating disease in Western Australia
Wu et al.
J. Neurol. Neurosurg. Psychiatry 2008;79:1022-1026.
ABSTRACT | FULL TEXT  

Neuromyelitis optica immunoglobulins as a marker of disease activity and response to therapy in patients with neuromyelitis optica
Weinstock-Guttman et al.
Mult Scler 2008;14:1061-1067.
ABSTRACT  

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

Minor salivary gland inflammation in Devic's disease and longitudinally extensive myelitis
Javed et al.
Mult Scler 2008;14:809-814.
ABSTRACT  

Anterior periventricular linear lesions in optic-spinal multiple sclerosis: a combined neuroimaging and neuropathological study
Matsushita et al.
Mult Scler 2008;14:343-353.
ABSTRACT  

NMO Antibody-Positive Recurrent Optic Neuritis Without Clear Evidence of Transverse Myelitis
Dinkin et al.
Arch Ophthalmol 2008;126:566-570.
FULL TEXT  

Hypocretinergic dysfunction in neuromyelitis optica with coma-like episodes
Carlander et al.
J. Neurol. Neurosurg. Psychiatry 2008;79:333-334.
FULL TEXT  

Neuromyelitis optica (Devic's disease) in a patient with syphilis
Wilcox et al.
Mult Scler 2008;14:268-271.
ABSTRACT  

Acute Disseminating Encephalomyelitis in Neuromyelitis Optica: Closing the Floodgates
Eichel et al.
Arch Neurol 2008;65:267-271.
ABSTRACT | FULL TEXT  

Neuromyelitis optica-IgG in childhood inflammatory demyelinating CNS disorders
Banwell et al.
Neurology 2008;70:344-352.
ABSTRACT | FULL TEXT  

Neuromyelitis Optica and Non Organ-Specific Autoimmunity
Pittock et al.
Arch Neurol 2008;65:78-83.
ABSTRACT | FULL TEXT  

Cognitive Functions in Neuromyelitis Optica
Blanc et al.
Arch Neurol 2008;65:84-88.
ABSTRACT | FULL TEXT  

Pathogenic potential of IgG binding to water channel extracellular domain in neuromyelitis optica
Hinson et al.
Neurology 2007;69:2221-2231.
ABSTRACT | FULL TEXT  

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
Tanaka et al.
J. Neurol. Neurosurg. Psychiatry 2007;78:990-992.
ABSTRACT | FULL TEXT  

Neuromyelitis Optica in a Mother and Daughter
Braley and Mikol
Arch Neurol 2007;64:1189-1192.
ABSTRACT | FULL TEXT  

Neuromyelitis Optica Is Distinct From Multiple Sclerosis
Weinshenker
Arch Neurol 2007;64:899-901.
FULL TEXT  

Neuromyelitis Optica Is a Variant of Multiple Sclerosis
Galetta and Bennett
Arch Neurol 2007;64:901-903.
FULL TEXT  

Is Neuromyelitis Optica Distinct From Multiple Sclerosis?: Something for "Lumpers" and "Splitters"
Frohman and Kerr
Arch Neurol 2007;64:903-905.
FULL TEXT  

Letter To the Editor
Jacob and Pittock
Mult Scler 2007;13:686-686.
 

Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis
Roemer et al.
Brain 2007;130:1194-1205.
ABSTRACT | FULL TEXT  

Loss of aquaporin 4 in lesions of neuromyelitis optica: distinction from multiple sclerosis
Misu et al.
Brain 2007;130:1224-1234.
ABSTRACT | FULL TEXT  

Heterogeneity of aquaporin-4 autoimmunity and spinal cord lesions in multiple sclerosis in Japanese
Matsuoka et al.
Brain 2007;130:1206-1223.
ABSTRACT | FULL TEXT  

Anti-aquaporin-4 antibody is involved in the pathogenesis of NMO: a study on antibody titre
Takahashi et al.
Brain 2007;130:1235-1243.
ABSTRACT | FULL TEXT  

Devic Disease With Abnormal Brain Magnetic Resonance Image Findings: The First Portuguese Case
Guimaraes and Sa
Arch Neurol 2007;64:290-291.
FULL TEXT  

Neuromyelitis optica and anti-aquaporin-4 antibodies: widening the clinical phenotype
Giovannoni
J. Neurol. Neurosurg. Psychiatry 2006;77:1001-1002.
FULL TEXT  

Neuromyelitis optica brain lesions localized at sites of high aquaporin 4 expression.
Pittock et al.
Arch Neurol 2006;63:964-968.
ABSTRACT | FULL TEXT  

Neuromyelitis optica
Jacob and Boggild
PN 2006;6:180-184.
FULL TEXT  

The lumping and splitting of inflammatory CNS diseases
Kerr
Neurology 2006;66:1466-1467.
FULL TEXT  

Revised diagnostic criteria for neuromyelitis optica
Wingerchuk et al.
Neurology 2006;66:1485-1489.
ABSTRACT | FULL TEXT