Enedina Maria Lobato de Oliveira

Lack of Response to Pulse Cyclophosphamide in Neuromyelitis Optica: Evaluation of 7 Patients

T he effect of immunosuppression with corticosteroids, azathioprine, mycophenolate, or rituximab for preventing relapses in patients with neuromyelitis optica (NMO) has been demonstrated in case series and retrospective studies. These drugs are currently considered the mainstay treatment for preventing neurologic worsening in NMO. Herein, we describe our experience with pulse cyclophosphamide for treating patients with NMO, which was used prior to azathioprine and had its use interrupted owing to lack of efficacy.

Neuromyelitis optica: brain abnormalities in a Brazilian cohort

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.

Cognitive profile of patients with relapsing remitting multiple sclerosis

Multiple sclerosis (MS) is a common disease in Western countries of temperate/cold climate, but in tropical countries an increasing number of cases have been diagnosticated. Moved by the lack of information about cognitive dysfunction of Brazilian MS patients, the present study attempted to describe features of neuropsychological alterations in patients with relapsing remitting MS living in the city of São Paulo. They were compared to healthy volunteers, matched for age and education. In the absence of global intellectual deterioration, the patients had a deficit: a) in learning and verbal long-term memory tasks and in visual long-term memory of complex figure; b) in timed tasks, accounted for by a slowness of mental processes; c) in tasks with a motor component. Tendency to depression was observed; anxiety levels were normal.

Esclerose múltipla: estudo clínico de 50 pacientes acompanhados no Ambulatório de Neurologia UNIFESP-EPM

Multiple sclerosis, seems to be a rare disease however in the population herein studied it is similar to the one described by others, in Brazil and abroad. We studied 50 patients classified according Posers criteria that were followed at the Department of Neurology UNIFESP-EPM from 1983 to 1995. The clinical findings of these 50 patients were similar to those described in other series. We found a high prevalence among female young patients who presented relapsing-remitting evolution. The most common symptoms were those related to pyramidal and cerebellar dysfunctions. The EDSS score seems to be worse in patients with specific cerebellar and pyramidal signs, higher number of relapses and longer time of disease but it is not related to the number of white matter lesions found at MRI.

Patients with neuromyelitis optica have a more severe disease than patients with relapsingremitting multiple sclerosis, including higher risk of dying of a demyelinating disease

UNLABELLED Although 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.

The real-life experience with cardiovascular complications in the first dose of fingolimod for multiple sclerosis

Fingolimod is a new and efficient treatment for multiple sclerosis (MS). The drug administration requires special attention to the first dose, since cardiovascular adverse events can be observed during the initial six hours of fingolimod ingestion. The present study consisted of a review of cardiovascular data on 180 patients with MS receiving the first dose of fingolimod. The rate of bradycardia in these patients was higher than that observed in clinical trials with very strict inclusion criteria for patients. There were less than 10% of cases requiring special attention, but no fatal cases. All but one patient continued the treatment after this initial dose. This is the first report on real-life administration of fingolimod to Brazilian patients with MS, and one of the few studies with these characteristics in the world.

Semantic Relations and Repetition of Items Enhance the Free Recall of Words by Multiple Sclerosis Patients

We compared 25 patients with multiple sclerosis (MS) and 24 normal controls on a test of free recall of words. Some lists contained words that were all unrelated, while in others the intermediary words were semantically related. In another set, the mid-list words were repeated across the lists, or, in addition to the repetition, were semantically associated. Immediate recall was assessed using these lists. Delayed recall was assessed using different lists (delay-unrelated and delay-related) after distractor tasks. Recency was not affected in MS patients, but the primacy effect was lower than in controls, this effect being interpreted as due to a deficiency in articulatory rehearsal. The delay interval after each list abolished recency in both groups and resulted in impaired recall in MS patients. However the patients, like the controls, benefited from semantic relations in the middle of the lists and from spaced repetition of words across the lists, in either immediate and delayed recall. The enhancing effects of word relatedness and of spaced repetition are seen as being due to automatic processes preserved in MS patients.

Neuromyelitis optica with onset in childhood and adolescence.

BACKGROUND Neuromyelitis optica with onset before the age of 18 years is a relatively rare, yet potentially devastating condition. The objective of the present study was to contribute to the study of early-onset neuromyelitis optica with a case series. PATIENTS Data were collected from medical records of Brazilian neurologists caring for patients with neuromyelitis optica occurring in childhood and adolescence. RESULTS Twenty-nine patients with neuromyelitis optica occurring before the age of 18 years and fulfilling the diagnostic criteria were identified. The average age at disease onset was 13 years and the patients had had an average disease duration of 6 years. The expanded disability scale score at the latest consultation was, on average, 4.7, and one patient had died from the disease. The 29 patients had had an average 4.5 relapses during the disease, accounting for 0.75 relapses per year, irrespective of the medication used. All patients were using one or more of the following medications: azathioprine, prednisone, immunoglobulin, and glatiramer acetate. CONCLUSIONS Neuromyelitis optica with onset in childhood and adolescence is a poorly understood condition that is often disabling and difficult to manage.

European Ancestry Predominates in Neuromyelitis Optica and Multiple Sclerosis Patients from Brazil

Background Neuromyelitis optica (NMO) is considered relatively more common in non-Whites, whereas multiple sclerosis (MS) presents a high prevalence rate, particularly in Whites from Western countries populations. However, no study has used ancestry informative markers (AIMs) to estimate the genetic ancestry contribution to NMO patients. Methods Twelve AIMs were selected based on the large allele frequency differences among European, African, and Amerindian populations, in order to investigate the genetic contribution of each ancestral group in 236 patients with MS and NMO, diagnosed using the McDonald and Wingerchuck criteria, respectively. All 128 MS patients were recruited at the Faculty of Medicine of Ribeirão Preto (MS-RP), Southeastern Brazil, as well as 108 healthy bone marrow donors considered as healthy controls. A total of 108 NMO patients were recruited from five Neurology centers from different Brazilian regions, including Ribeirão Preto (NMO-RP). Principal Findings European ancestry contribution was higher in MS-RP than in NMO-RP (78.5% vs. 68.7%) patients. In contrast, African ancestry estimates were higher in NMO-RP than in MS-RP (20.5% vs. 12.5%) patients. Moreover, principal component analyses showed that groups of NMO patients from different Brazilian regions were clustered close to the European ancestral population. Conclusions Our findings demonstrate that European genetic contribution predominates in NMO and MS patients from Brazil.

Diffusion tensor imaging of the cervical spinal cord of patients with Neuromyelitis Optica.

BACKGROUND Previous studies have demonstrated a correlation between Expanded Disability Status Scale (EDSS) and Diffusion Tensor Imaging (DTI) metrics, but the conclusions were based on evaluations of the entire cervical spinal cord. OBJECTIVES The purpose of this study was to quantify the FA and MD values in the spinal cord of NMO patients, separating the lesion sites from the preserved sites, which has not been previously preformed. In addition, we attempted to identify a correlation with EDSS. METHODS DTI was performed in 11 NMO patients and 11 healthy individuals using a 1.5-T MRI scanner. We measured the FA and MD at ROIs positioned along the cervical spinal cord. The mean values of FA and MD at lesion, preserved and spinal cord sites were compared with those of a control group. We tested the correlations between the mean FA and MD with EDSS. RESULTS FA in NMO patients was significantly reduced in lesion sites (0.44 vs. 0.55, p=0.0046), preserved sites (0.46 vs. 0.55, p=0.0015), and all sites (0.45 vs 0.55, p=0.0013) while MD increased only in lesion sites (1.03×10(-3)mm(2)/s vs. 0.90×10(-3)mm(2)/s, p=0.009). The FA demonstrated the best correlation with EDSS (r=-0.7603, p=0.0086), particularly at lesion sites. CONCLUSIONS The results reinforce the importance of the FA index and confirm the hypothesis that NMO is a diffuse disease.