In this series, we did not find a statistical difference of disease progression between patients with and without brain lesions, suggesting that the presence of brain abnormalities is not a marker of disease severity.

Patients: Thirty-six patients with relapsing-remitting optic-spinal disease; long, extending spinal cord lesions; and brain magnetic resonance images not meeting Barkhof criteria for multiple sclerosis, thus fulfilling the 1999 and 2006 criteria for neuromyelitis optica. Patients were followed up from 1994 to 2007.Main Outcome Measures: Relapses and accumulation of disability.Results: Mean follow-up time was 47.2 months and mean ageatonsetwas32.3years.Sixty-fourtreatmentswereimple-mentedin36patients,whichincludedinterferonbeta,methotrexate, cyclophosphamide, prednisone, and azathioprine solely or plus prednisone. Patients who were treated with azathioprine or azathioprine with prednisone had a reduction in the occurrence of relapses and Expanded Disability Severity Scale score stabilization, as opposed to patients who received other treatments. Of the 4 patients who died, only 1 had received azathioprine treatment. Conclusion:Azathioprine as monotherapy or with prednisone seems to have reduced the relapse frequency and halted disability progression in the majority of patients treated, with minor and manageable adverse effects.

NAWM was found to have a normal Naa/Cr in patients with NMO, reinforcing the concept that the white matter is not primarily affected in this disease.

-Neuromyelitis optica (NMO) is a demyelinating disease consisting of relapsing-remitting optic neuritis and myelitis with a more severe course than Multiple Sclerosis. Recently, it has been shown that almost 50% of patients with NMO can have brain magnetic resonance imaging (MRI) abnormalities. We report on six Brazilian patients with NMO, fulfilling the 1999 Wingerchuck criteria for this disease, with abnormal brain MRI and discuss their clinical and radiological features.Key WORdS: neuromyelitis optica, brain abnormalities, MRI. Neuromielite óptica: alterações encefálicas em pacientes brasileirosResumo -Neuromielite óptica (NMO) é doença desmielinizante, remitente-recorrente, com acometimento predominante dos nervos ópticos e medula espinal e uma evolução mais grave comparada à esclerose múltipla. estudos recentes demonstraram que até 50% dos pacientes com NMO podem apresentar lesões encefálicas à ressonância magnética (RM). Relatamos seis pacientes brasileiros com NMO, que satisfazem os critérios diagnósticos de Wingerchuck (1999) para NMO, com alterações encefálicas em RM de encéfalo e discutimos seus dados clínicos e de imagem.PALAVRAS-CHAVe: neuromielite óptica, alterações encefálicas, ressonância magnética. Neuromyelitis optica (NMO), also known as devic's syndrome or devic's disease, is a demyelinating disease with predilection for the optic nerve and spinal cord 1,2 . It was first considered to have a monophasic course and to be a multiple sclerosis (MS) variant, but data gathered in the past few years have shown NMO to be a relapsing disease with clinical behavior and pathology distinctive from MS 1,3 . The first Brazilian reports on NMO have also disclosed extensive demyelination in the optic nerves and spinal cord 4,5 . The first attempt to establish diagnostic criteria for NMO demanded the occurrence of optic neuritis (uni or bilateral) and acute myelitis with no restriction on the timeframe over which the first attacks of optic neuritis and myelitis occur (index event), and no evidence of disease outside the optic nerve and spinal cord 2 . Neurologists have thus been reluctant to diagnose NMO in someone with brain scan abnormalities, even though these abnormalities do not fulfill the criteria for MS 6 . Therefore, we sought to survey our NMO patient's records in search of cases with brain MRI abnormalities and discuss their disease course. methodWe retrospectively reviewed records of 63 patients attended at the Federal University of São Paulo Hospital Neuroimmunology Clinic, Brazil, from 1994 to 2006, who presented with a recurrent idiopathic demyelinating disease, predominantly affecting the optic nerves and spinal cord. Apart from the clinical course they had spinal cord lesions longer than three vertebral segments and brain magnetic resonance imaging (MRI) abnormalities not fulfilling MS criteria, thus meeting the 1999 criteria for NMO 2 . Based solely on the records notes, 50% had some form of unspecific brain MRI abnormality. We selected six of these patients whose brain MRI were avai...

Neuromyelitis optica (NMO) is an inflammatory autoimmune disease that affects the central nervous system (CNS) associated to significant neurologic disability 1,2 . For many decades, NMO was considered a variant of multiple sclerosis (MS), characterized by recurrent bilateral optic neuritis and transverse myelitis occurring concomitantly or apart in time, and without evidence of disease outside the optic nerves and the spinal cord 3 . Nowadays, studies ABSTRACTAlthough neuromyelitis optica (NMO) is known to be a more severe disease than relapsing-remitting multiple sclerosis (RRMS), few studies comparing both conditions in a single center have been done. Methods: Comparison of our previously published cohort of 41 NMO patients with 177 RRMS patients followed in the same center, from 1994 to 2007. Results: Mean age of onset was 32.6 for NMO and 30.2 for RRMS (p=0.2062) with mean disease duration of 7.4 years for NMO and 10.3 years for RRMS. Patients with NMO had a higher annualized relapse rate (1.0 versus 0.8, p=0.0013) and progression index (0.9 versus 0.6, p<0.0001), with more patients reaching expanded disability status scale (EDSS) 6.0 (39 versus 17%, p=0.0036). The odds ratio for reaching EDSS 6.0 and being deceased due to NMO in comparison to RRMS were, respectively, 3.14 and 12.15. Conclusion: Patients with NMO have a more severe disease than patients with RRMS, including higher risk of dying of a demyelinating disease.Key words: neuromyelitis optica, multiple sclerosis, demyelinating diseases, risk factors. RESUMOEmbora a neuromielite óptica (NMO) seja reconhecida como mais grave que a esclerose múltipla remitente recorrente (EMRR), existem poucos estudos comparando as duas doenças em um único centro. Métodos: Comparação de nossa coorte publicada de 41 pacientes com NMO com 177 pacientes com EMRR seguidos no mesmo centro, de 1994 a 2007. Resultados: A média de idade inicial foi de 32,6 anos em NMO e 30,2 anos em EMRR (p=0,2062), com tempo médio de doença de 7,4 anos para NMO e 10,3 anos EMRR. Pacientes com NMO apresentaram maior taxa anualizada de surtos (1,0 versus 0,8, p=0,0013) e índice de progressão (0,9 versus 0,6, p<0,0001), com mais pacientes atingindo EDSS 6,0 (39 versus 17%, p=0,0036). Os riscos relativos de se alcançar 6,0 EDSS e falecer em decorrência de NMO em comparação com EMRR, foram, respectivamente, 3,14 e 12,15. Conclusão: Pacientes com NMO têm uma doença mais grave do que os pacientes com EMRR, incluindo maior risco de morrer de uma doença desmielinizante.Palavras-Chave: neuromielite óptica, esclerose múltipla, doenças desmielinizantes, fatores de risco.indicate that NMO is a distinct disease from MS 4 , with nearly 90% of patients presenting a relapsing remitting course and half of them exhibiting brain lesions on magnetic resonance imaging 2,5 . Although NMO is frequently cited to have a more severe clinical course than relapsing-remitting MS (RRMS), few direct head-to-head studies involving both diseases in a single center scenario have been done 6,7 . We recently published