Clinical Radiology xxx (2015) e1ee8
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Neuromyelitis optica spectrum disorders: beyond longitudinally extensive transverse myelitis M.D. Lemos a, *, G.B.S. Carvalho a, R.S. Carvalho a, D.B. Bichuetti b, E.M.L. de Oliveira b, N. Abdala a a b
~o Paulo, Brazil stico por Imagem, Universidade Federal de Sa Departamento de Diagno ~o Paulo, Brazil Disciplina de Neurologia, Universidade Federal de Sa
art icl e i nformat ion Article history: Received 22 October 2014 Received in revised form 23 October 2014 Accepted 20 February 2015
AIM: To describe the neuroradiological features and their prevalence in patients with neuromyelitis optica (NMO). MATERIALS AND METHODS: Two neuroradiologists independently reviewed 35 spinal cord and 37 brain MRI studies from patients with NMO. The examinations were analysed for the presence of lesion, topography, enhancement, and brain lesions suggestive of multiple sclerosis and/or NMO. RESULTS: Seventy percent of the spinal cord lesions involved over three or more vertebral segments. Seventy-eight percent of brain scans were abnormal, and the most prevalent ﬁndings were non-speciﬁc foci of T2 hyperintensities in the cerebral white matter (55%) and brainstem lesions (52%). One patient had lesions disseminated in space compatible with multiple sclerosis according to 2010 revised McDonald criteria. Brain lesions suggestive of NMO occurred at least once in 17 (59%) patients. CONCLUSION: Spinal cord lesions were often longitudinally extensive and brain lesions were common, with the majority of patients having at least one distinctive NMO lesion. Ó 2015 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.
Introduction Neuromyelitis optica (NMO) is an autoimmune, inﬂammatory, and demyelinating disease of the central nervous system with predominant involvement of the optic nerves and spinal cord, associated with anti-aquaporin-4 antibodies (NMO-IgG).1 Most patients are female, with a mean age of onset in the fourth to ﬁfth decade.2 Patients with NMO have a higher risk of dying compared to multiple
* Guarantor and correspondent: M. Delboni Lemos, Department of Diag~o Paulo, Rua Napole~ nostic Imaging, Universidade Federal de Sa ao de Barros, ~o Paulo e SP, 04024-002, Brazil. Tel.: 800, Vila Clementino, Sa þ551159087900. E-mail address: [email protected]
sclerosis (MS),3 and long-term therapy is different for both diseases, thus prompt differentiation between NMO and MS is necessary. Acute NMO events are treated with high-dose intravenous methylprednisolone, intravenous immunoglobulin, or plasmapheresis in resistant cases, and longterm remission is achieved with oral corticosteroids and immunosuppressants.4 MRI from patients with NMO generally demonstrates contiguous spinal cord lesions extending over three or more vertebral segments (longitudinally extensive transverse myelitis).5 In addition, brain lesions, considered rare before, are now recognized as part of the NMO spectrum. These lesions affect predominantly sites of high aquaporin-4 expression in the brain, mainly the periependymal regions, but also the hemispheres and corticospinal tracts.7,8
http://dx.doi.org/10.1016/j.crad.2015.02.016 0009-9260/Ó 2015 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: M. Delboni Lemos, Balthazar da Silveira Carvalho G, et al., Neuromyelitis optica spectrum disorders: beyond longitudinally extensive transverse myelitis, Clinical Radiology (2015), http://dx.doi.org/10.1016/j.crad.2015.02.016
M.D. Lemos et al. / Clinical Radiology xxx (2015) e1ee8
Considering this scenario of rapid changes, the aim of the present study is to describe the radiological features in Brazilian patients with NMO and their prevalence in both brain and spinal cord MRI, and to present a practical approach to evaluating the images from these patients for the general radiologist and general neurologist.
Materials and methods This study was approved by the institutional ethics committee. All available MRI studies performed in our ~o Paulo) from patients followed at the hospital (Hospital Sa neurology department from January 2004 to December 2012, diagnosed according to Wingerchuck 1999 and/or 2006 criteria,6 were retrieved. For patients that had more than one available MRI examination, the examination in which lesions were more conspicuous was selected. Clinical and demographic data were obtained from medical records, neurological status was measured by the Expanded Disability Status Scale (EDSS), and NMO-IgG serology was obtained by indirect immunoﬂuorescence from a commercial test. Exclusion criteria were paediatric cases (