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.

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...

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Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a neurodegenerative disorder characterized by late-infantile onset spastic ataxia and other neurological features. ARSACS has a high prevalence in northeastern Quebec, Canada. Several ARSACS cases have been reported outside Canada in recent decades. This is the first report of typical clinical and neuroimaging features in a Brazilian family with probable diagnosis of ARSACS.