Valor pronóstico del glutamato en el líquido cefalorraquídeo en esclerosis múltiple / Prognostic value of cerebrospinal fluid glutamate in multiple sclerosis

Resumen El objetivo del trabajo fue evaluar la asociación entre el nivel de glutamato en el líquido cefalorraquídeo (LCR) al inicio de la enfermedad y la progresión de la enfermedad durante el seguimiento en una cohorte de pacientes con esclerosis múltiple (EM). Se determinaron niveles de glutamato (Glu) en LCR al inicio de la enfermedad. Se realizó una resonancia basal y durante el seguimiento cada 12 meses con el objeto de determinar el porcentaje de cambio de volumen cerebral (PCVC), grosor cortical (GC) y volumen le sional cerebral en secuencia T2 (VLT2). Los predictores primarios de interés fueron los niveles basales de Glu en LCR, PCVC Y GC, así como la progresión clínica de la enfermedad [medida por Expanded Disability Status Scale (EDSS) y tasa anual de recaídas]. Un total de 26 pacientes fueron incluidos. La concentración media de Glu fue de 5.3 ± 0.4 μM/l. Se encontró una asociación significativa entre concentraciones basales elevadas de Glu y la progresión del EDSS (b = 1.06, IC 95% 0.47-1.66, p = 0.003), así como también el PCVC (b = -0.71, IC 95% -0.56-1.38, p = 0.002) y CG (b = -0.15, IC 95% -0.06-0.33, p = 0.01). No se encontró asociación entre los niveles de Glu y la tasa anual de recaídas como tampoco el VLT2 (b = 0.08, IC 95% -0.11-0.43, p = 0.11 y b = 195, IC -39-330, p = 0.22, respectivamente). Los niveles aumentados de Glu se asociaron con un mayor cambio en el PCVC y progresión del EDSS durante el seguimiento.

Abstract. The objective of this study was to evaluate the association between glutamate (Glu) levels in cerebrospinal fluid (CSF) at disease onset and disease progression during follow up in a cohort of multiple sclerosis (MS) patients. Glu level was measured at disease onset (first relapse). MRI was obtained at baseline and follow-up (every 12 months) to determine the percent of brain volume change (PBVC), cortical thickness (CT), and T2 lesion volume (T2LV). The primary predictors of interest were baseline CSF Glu levels, PBVC and CT, as well as clinical disease progression [measured by Expanded Disability Status Scale (EDSS) and annualized relapse rate] during follow-up. A total of 26 MS patients were included. Mean concentration of Glu in CSF at diagnosis was 5.3 ± 0.4 μM/l. A significant association was observed between higher baseline levels of Glu and an increase in EDSS during follow up (b = 1.06, 95%CI 0.47-1.66, p = 0.003) as well as PBVC (b = -0.71 95%CI -0.56-1.38, p = 0.002) and CT (b = -0.15, 95%CI -0.06-0.33, p = 0.01). We did not observe an association between baseline Glu levels and relapse rate or T2LV during follow-up (b = 0.08, 95%CI -0.11-0.43, p = 0.11 and b = 195, 95%CI -39-330, p = 0.22, respectively). Higher Glu concentrations at disease onset were associated with an increase in PBVC and EDSS progression during follow-up in MS patients.

Prevalence of multiple sclerosis in key cities of Brazil: a study in Passo Fundo, Southern Brazil / Prevalência de esclerose múltipla em cidades-chave do Brasil: um estudo em Passo Fundo, no sul do Brasil

ABSTRACT Background: To improve the comparability of multiple sclerosis (MS) prevalence across Brazilian regions, the Brazilian Committee for Treatment and Research in MS has implemented a standardized approach to assess the prevalence of the disease in five key cities, which were deemed representative of their regions in terms of socio-geographical features and where in-person revision of each case was feasible. Objective: To report the point-prevalence of MS in Passo Fundo, one of the key cities in Southern Brazil. Methods: We sought to identify all MS patients who were living in Passo Fundo on July 1st, 2015. The primary source for case ascertainment was records from the offices of neurologists and neurosurgeons practicing in the city. Multiple secondary sources were used to maximize identification of cases. All patients underwent in-person review of the diagnosis by a panel of neurologists with experience in MS. Results: We identified 52 MS patients living in Passo Fundo on July 1st, 2015. The point-prevalence rate for MS was 26.4/100,000 population (95% confidence interval, 19.7 to 34.6/100,000). Among the MS cases, 42 (80.8%) were female, for a sex ratio of 4.2:1. Forty-six cases (88.5%) were categorized as relapsing-remitting MS, and the remaining 6 cases, as secondary progressive MS (11.5%). Other epidemiological and clinical features were comparable to national and international MS populations. Conclusions: The prevalence of MS in Passo Fundo is one of the highest reported in Brazil so far. Studies in other key Brazilian cities, using the same methodology, are currently being carried out.

RESUMO Introdução: Para melhor comparar a prevalência de esclerose múltipla (EM) nas diferentes regiões do Brasil, o Comitê Brasileiro para Tratamento e Pesquisa em Esclerose Múltipla implementou uma abordagem padronizada para avaliar a prevalência da doença em 5 cidades-chave, consideradas representativas de suas regiões em termos de características sociogeográficas e nas quais seria viável revisar cada caso pessoalmente. Objetivos: Descrever a prevalência pontual de EM em Passo Fundo, uma das cidades-chave, localizada no Sul do Brasil. Métodos: Buscamos identificar todos os pacientes com EM que viviam em Passo Fundo no dia 1( de julho de 2015. A fonte primária para identificação de casos foi os registros de consultórios de neurologistas e neurocirurgiões da cidade. Múltiplas fontes secundárias foram usadas para maximizar a identificação de casos. Todos os pacientes tiveram o diagnóstico revisado pessoalmente por um painel de neurologistas com experiência em EM. Resultados: Identificamos 52 pacientes com EM que viviam em Passo Fundo em 1( de julho de 2015. Assim, a prevalência pontual bruta de EM foi 26,4/100.000 habitantes (intervalo de confiança de 95%, 19,7 a 34,6/100.000). Entre os casos de EM, 42 (80,8%) eram mulheres, (razão de sexos: 4,2:1). Quarenta e seis casos (88,5%) foram categorizados como EM remitente-recorrente, e os 6 casos restantes como EM secundariamente progressiva (11,5%). As demais características epidemiológicas e clínicas foram comparáveis a populações de EM internacionais. Conclusões: A prevalência de EM em Passo Fundo é uma das maiores já relatadas no Brasil. Estudos em outras cidades-chave brasileiras, usando a mesma metodologia, estão em andamento.

Determinants of disability development in patients with multiple sclerosis / Determinantes do desenvolvimento de deficiência em pacientes com esclerose múltipla

Abstract Background: Multiple sclerosis (MS) is one of the most common chronic neurological diseases affecting the central nervous system in young adults. Objective: To investigate demographic and clinical factors that are effective in the development of irreversible disability from the onset of MS, and to identify factors that affect the transformation from the relapse-remitting MS (RRMS) phase to the progressive MS (PMS) phase. Methods: Retrospective study on 741 patients who were diagnosed with RRMS and PMS according to the McDonald criteria, and were enrolled into the Turkish MS database of the Department of Neurology MS Polyclinic, at the Faculty of Medicine, Karadeniz Technical University, in Trabzon, Turkey. Kaplan-Meier analysis was used to evaluate the time taken to reach EDSS 4 and EDSS 6 from the onset of disease, and the time taken between EDSS 4 and EDSS 6. Results: Age of onset >40 years; having polysymptomatic-type onset, pyramidal or bladder-intestinal system-related first episode; ≥7 episodes in the first 5 years; and <2 years between the first two episodes were found to be effective for MS patients to reach EDSS 4 and EDSS 6. The demographic and clinical parameters that were effective for progression from EDSS 4 to EDSS 6 were: pyramidal or bladder-intestinal system-related first episode; 4‒6 episodes in the first 5 years; >2 years until start of first treatment; and smoking. Conclusions: Our findings reveal important characteristics of MS patients in our region. However, the associations between these parameters and MS pathophysiology remain to be elucidated.

RESUMO Introdução: A esclerose múltipla (EM), uma das doenças neurológicas crônicas mais comuns, afeta o sistema nervoso central em jovens adultos. Objetivo: Investigar fatores demográficos e clínicos que são efetivos no desenvolvimento de deficiência irreversível, desde o início da EM, e identificar fatores que afetam a transformação da fase de EM recorrente-remitente (EMRR) para a fase de EM secundária progressiva (EMSP). Métodos: Estudo retrospectivo de 741 pacientes que foram diagnosticados com EMRR e EMSP, de acordo com os critérios de McDonald, e inscritos no banco de dados turco MSBase, do Departamento de Neurologia da MS Polyclinic, da Universidade Técnica de Karadeniz, Turquia. Análise de Kaplan-Meier foi usada para avaliar o tempo para alcançar EDSS 4 e EDSS 6, desde o início da doença e o tempo entre EDSS 4 e EDSS 6. Resultados: Idade de início>40 anos, início do tipo polissintomático, primeiro ataque relacionado ao sistema piramidal ou bexiga-intestinal, ≥7 recaídas nos primeiros 5 anos e <2 anos entre os dois primeiros ataques foram considerados eficazes em pacientes com EM que atingiram EDSS 4 e EDSS 6. Parâmetros demográficos e clínicos que foram efetivos no progresso de EDSS 4 para EDSS 6: primeiro ataque relacionado ao sistema piramidal ou bexiga-intestinal, 4‒6 recaídas nos primeiros 5 anos, >2 anos até o início do primeiro tratamento e tabagismo. Conclusão: Estudo revelou características importantes dos pacientes com EM em nossa região. No entanto, as associações entre esses parâmetros e a fisiopatologia da EM ainda precisam ser elucidadas.

Aspectos clínicos y demográficos de los pacientes con esclerosis múltiple secundaria progresiva en Argentina / Clinical and demographic aspects of secondary progressive multiple sclerosis in Argentina

Resumen El objetivo del estudio fue evaluar los aspectos clínicos y demográficos de los pacientes con esclerosis múltiple (EM) secundaria progresiva (EMSP) en los pacientes incluidos en el Registro Argentino de EM (RelevarEM, número de registro de Clinical Trials 03375177). RelevarEM es un registro longitudinal, estrictamente observacional, de pacientes con EM y trastornos del espectro de neuromielitis óptica. Los aspectos clínicos y demográficos fueron descriptos en pacientes con EMSP respecto a aquellos con EM recaída en remisión (EMRR). Se incluyeron 1723 pacientes con EM (1605, 93.2% EMRR y 118, 6.8%, EMSP). En el grupo con EMSP la mediana de edad fue de 53 (intervalo inter-cuartil [IIQ] 47-62) años, 67% eran mujeres, mediana de tiempo de evolución de enfermedad 19.5 (IIQ 14-26) años, EDSS (expanded disability status scale), 6.5 y 48.3% estaban en tratamiento para su EM. Solo el 23.7% con EMSP estaban trabajando activamente y el 86% tenía certificado de discapacidad. Un 35.6% con EMSP presentaron nuevas lesiones en resonancia magnética y 5% tuvo recaídas clínicas en los 12 meses previos al análisis, mostrando una actividad de la enfermedad significativamente menor respecto a la forma EMRR (p < 0.01).

Abstract The objective of the study was to describe the clinical and demographic aspects of patients with secondary progressive multiple sclerosis (SPMS) included in the Argentine MS Registry (RelevarEM, Clinical Trials registry number 03375177). RelevarEM is a longitudinal, strictly observational registry of patients with MS and neuromyelitis optica spectrum disorders. Clinical and demographic aspects were described in patients with SPMS and compared with relapsing remitting MS patients (RRMS). A total of 1723 patients with MS were included (1605, 93.2% RRMS and 118, 6.8%, SPMS). In SPMS, the median age was 53 (inter quartile range [IQR] 47-62) years, 67% were women, median disease duration of 19.5 (IQR 14-26) years, median EDSS (expanded disability status scale) 6.5 and 48.3% were under treatment for their MS. Only 23.7% of patients with SPMS were actively working and 86% had a disability certificate; 35.6% of patients with SPMS presented new lesions in MRI and 5% had clinical relapses during the past 12 months of the registry entry showing a significantly lower disease activity compared with RRMS (p < 0.01).

Differentiation of relapsing-remitting and secondary progressive multiple sclerosis: a magnetic resonance spectroscopy study based on machine learning / Diferenciação de esclerose múltipla recorrente-remitente e progressiva secundária: um estudo de ressonância magnética com espectroscopia baseado em aprendizado de máquina

ABSTRACT Introduction: Magnetic resonance imaging (MRI) is the most important tool for diagnosis and follow-up in multiple sclerosis (MS). The discrimination of relapsing-remitting MS (RRMS) from secondary progressive MS (SPMS) is clinically difficult, and developing the proposal presented in this study would contribute to the process. Objective: This study aimed to ensure the automatic classification of healthy controls, RRMS, and SPMS by using MR spectroscopy and machine learning methods. Methods: MR spectroscopy (MRS) was performed on a total of 91 participants, distributed into healthy controls (n=30), RRMS (n=36), and SPMS (n=25). Firstly, MRS metabolites were identified using signal processing techniques. Secondly, feature extraction was performed based on MRS Spectra. N-acetylaspartate (NAA) was the most significant metabolite in differentiating MS types. Lastly, binary classifications (healthy controls-RRMS and RRMS-SPMS) were carried out according to features obtained by the Support Vector Machine algorithm. Results: RRMS cases were differentiated from healthy controls with 85% accuracy, 90.91% sensitivity, and 77.78% specificity. RRMS and SPMS were classified with 83.33% accuracy, 81.81% sensitivity, and 85.71% specificity. Conclusions: A combined analysis of MRS and computer-aided diagnosis may be useful as a complementary imaging technique to determine MS types.

RESUMO Introdução: A ressonância magnética é a ferramenta mais importante para o diagnóstico e acompanhamento na EM. A transição da EM recorrente-remitente (EMRR) para a EM progressiva secundária (EMPS) é clinicamente difícil e seria importante desenvolver a proposta apresentada neste estudo a fim de contribuir com o processo. Objetivo: o objetivo deste estudo foi garantir a classificação automática de grupo controle saudável, EMRR e EMPS usando a RM com espectroscopia e métodos de aprendizado de máquina. Métodos: Os exames de RM com espectroscopia foram realizados em um total de 91 amostras com grupo controle saudável (n=30), EMRR (n=36) e EMPS (n=25). Em primeiro lugar, os metabólitos da RM com espectroscopia foram identificados usando técnicas de processamento de sinal. Em segundo lugar, a extração de recursos foi realizada a partir do MRS Spectra. O NAA foi determinado como o metabólito mais significativo na diferenciação dos tipos de MS. Por fim, as classificações binárias (Healthy Control Group-RRMS e RRMS-SPMS) foram realizadas de acordo com as características obtidas por meio do algoritmo Support Vector Machine. Resultados: Os casos de EMRR e do grupo de controle saudável foram diferenciados entre si com 85% de acerto, 90,91% de sensibilidade e 77,78% de especificidade, respectivamente. A EMRR e a EMPS foram classificadas com 83,33% de acurácia, 81,81% de sensibilidade e 85,71% de especificidade, respectivamente. Conclusões: Uma análise combinada de RM com espectroscopia e abordagem de diagnóstico auxiliado por computador pode ser útil como uma técnica de imagem complementar na determinação dos tipos de EM.

Evaluación clínica y por imágenes de la esclerosis múltiple progresiva / Clinical and radiological outcomes measures in progressive multiple sclerosis

Durante los últimos años, el desarrollo de medidas de evaluación de la acumulación de la discapacidad y la actividad inflamatoria en las formas progresivas de esclerosis múltiple (EM) ha sido un punto central de investigación de diversos grupos. Se han desarrollado y aplicado distintos instrumentos con el fin de identificar en forma precisa y precoz la actividad y la progresión en este fenotipo de EM. Muchas de esas herramientas, con mayor o menor sensibilidad, han sido utilizadas en ensayos clínicos, aunque su uso en la práctica asistencial no es del todo familiar para los profesionales involucrados en el cuidado de pacientes con EM. El objetivo de esta revisión es describir las medidas de evaluación clínica y por imágenes implementadas durante los últimos años para la identificación de la actividad y evolución de esta enfermedad en sus formas progresivas.

During recent years, the development of measures to assess the accumulation of disability and inflammatory activity in the progressive forms of multiple sclerosis (MS) has been a central point of research in various groups. Several instruments have been developed and implemented in order to accurately and early identify the activity and progression in this MS phenotype. Many of these tools, with greater or lesser sensitivity, have been used in clinical trials, although their use in healthcare practice is not entirely familiar to professionals involved in the care of patients with MS. The objective of this review is to describe the clinical and imaging evaluation measures implemented during the last years to identify the activity and the evolution of the disease in its progressive forms.

Clinical Presentations and Phenotypic Spectrum of Multiple Sclerosis at a University Hospital in Saudi Arabia

BACKGROUND AND PURPOSE: The objective of this study was to determine the frequencies of different clinical presentations and the phenotypic spectrum of multiple sclerosis (MS). METHODS: This cross-sectional study was performed in the Neurology Department of King Fahd Hospital of University Alkhobar in the Kingdom of Saudi Arabia (KSA). Data of 190 MS patients who fulfilled the McDonald criteria were retrieved from medical records and analyzed. RESULTS: The age at disease onset was 26.27±8.2 years (mean±SD) and disease duration was 6.38±5.10 years. The male-to-female ratio was 1:1.6. Optic neuritis and myelitis were the most-frequent first clinical presentations. Sensory (73.1%), motor (61%), and visual (58.4%) symptoms were the most-frequent established clinical symptoms. Relapsing-remitting multiple sclerosis (RRMS) was present in 75% of the cases. Supratentorial T2-weighted white-matter lesions and deep-gray-matter or juxtacortical lesions were the most-frequent magnetic resonance imaging (MRI) lesions, comprising 28% and 23.7% of all MRI lesions observed in 93.6% and 79.4% of the cases, respectively. The scores on the Expanded Disability Status Scale were within the range of 1.0–5.5 in 82.1% of the patients. There were 145 (76.3%) patients taking interferon β therapy. CONCLUSIONS: MS presenting in the hospital setting is more common in KSA than reported previously, and the number of diagnosed cases in increasing. It is therefore an emerging and disabling neurological illness in KSA with clinical characteristics not dissimilar to those in other middle eastern countries. A decrease in the frequency of patients with secondary progressive multiple sclerosis (SPMS) indicates either that more new cases of RRMS are being diagnosed or that adequate treatments of RRMS are preventing the evolution to SPMS. Further larger and population-wide epidemiological and clinical studies with the long-term follow-up of MS patients are required to better assess the clinical spectrum of MS in KSA.

Self-Injurious Behavior Revealing Advanced Primary Progressive Multiple Sclerosis with a Massive Right Temporal Lesion

No abstract available.

Patients with multiple sclerosis do not necessarily consume more alcohol or tobacco than the general population / Pacientes com esclerose múltipla não necessariamente consomem mais álcool ou tabaco do que a população geral

Purpose Recent papers suggest that patients with multiple sclerosis (MS) are prone to alcohol misuse. This may be due to the combination of a lifelong and disabling disease with a psychiatric profile typical of MS. The objective of the present study was to assess these findings in a culturally different population of patients with MS.Method The present case-control transversal study assessed 168 patients with MS and 168 control subjects from Brazil.Results There were no evidence that patients with MS drank more alcohol or, smoked more than did controls. In fact, control subjects had a significantly higher alcohol consumption. The only trait associated to higher alcohol consumption was anxiety, both for patients and controls.Conclusion Unlike previous reports in the literature, patients with MS in our study did not drink or smoked more than a control population.

Propósito Artigos recentes sugerem que pacientes com esclerose múltipla (EM) tem tendência ao abuso de álcool. Isto poderia se dever à combinação de uma doença crônica e incapacitante e um perfil psiquiátrico típico da EM. O objetivo do presente estudo foi avaliar estes achados em uma população de pacientes com EM culturalmente diferente.Método O presente estudo caso-controle transversal avaliou 168 pacientes com EM e 168 controles, todos brasileiros.Resultados Não houve evidência que pacientes com EM usassem mais álcool ou tabaco do que os controles. Na verdade, os controles apresentavam um consumo significativamente maior de álcool. O único aspecto associado ao maior consumo de álcool foi a ansiedade, tanto para pacientes quanto para controles.Conclusão Ao contrário de outros dados da literatura, pacientes com EM neste nosso estudo não bebem ou fumam mais do que a população controle.

Contingent Negative Variation Is Associated with Cognitive Dysfunction and Secondary Progressive Disease Course in Multiple Sclerosis

BACKGROUND AND PURPOSE: The relationship between contingent negative variation (CNV), which is an event-related potential, and cognition in multiple sclerosis (MS) has not been examined previously. The primary objective of the present study was thus to determine the association between CNV and cognition in a sample of MS patients. METHODS: The subjects of this study comprised 66 MS patients [50 with relapsing-remitting MS (RRMS) and 16 with secondary progressive MS (SPMS)] and 40 matched healthy volunteers. A neuropsychological battery was administered to all of the subjects; CNV recordings were made from the Cz, Fz, and Pz electrodes, and the amplitude and area under the curve (AUC) were measured at each electrode. RESULTS: RRMS patients exhibited CNVs with lower amplitudes and smaller AUCs than the controls at Pz. SPMS patients exhibited CNVs with lower amplitudes and smaller AUCs than the controls, and CNVs with a smaller amplitude than the RRMS patients at both Cz and Pz. After correcting for multiple comparisons, a lower CNV amplitude at Pz was significantly associated with worse performance on measures of speed of information processing, verbal fluency, verbal learning, and verbal recall. CONCLUSIONS: CNV may serve as a marker for disease progression and cognitive dysfunction in MS. Further studies with larger samples and wider electrode coverage are required to fully assess the value of CNV in these areas.

Different cognitive profiles of Brazilian patients with relapsing-remitting and primary progressive multiple sclerosis / Diferentes perfis cognitivos de pacientes brasileiros com esclerose múltipla remitente-recorrente e progressiva primária

Cognitive impairment is a symptom of multiple sclerosis (MS). Different clinical forms of multiple sclerosis have different cognitive profiles, according to findings of previous studies which used extensive batteries of neuropsychological tests. OBJECTIVE: To investigate cognitive profiles of Brazilian patients with relapsing-remitting multiple sclerosis (RRMS) and primary progressive multiple sclerosis (PPMS) by using a brief battery of neuropsychological tests. METHOD: Sixty-six patients, within 18-65 of age and 3-18 years of education, were paired with healthy control subjects, regarding gender, age, and education level. RESULTS: On Symbol Digit Modalities Test and Hooper Visual Organization Test, cognition was affected in 50 percent in RRMS and 69 percent in PPMS. Fluency of "F" was impaired in 24 percent of RRMS and 81 percent of PPMS. Immediate recall was affected in 32 percent of RRMS and in 63 percent of PPMS; whereas late recall, in 46 percent of relapsing-remitting and in 69 percent of primary progressive. CONCLUSION: Cognitive profiles of relapsing-remitting and primary progressive patients are different.

O comprometimento cognitivo é um sintoma da esclerose múltipla (EM). Formas clínicas diferentes da EM apresentam diferentes perfis cognitivos, de acordo com resultados de estudos anteriores que usaram bateria extensa de testes neuropsicológicos. OBJETIVO: Investigar o perfil cognitivo de pacientes com esclerose múltipla remitente-recorrente (EMRR) e esclerose múltipla progressiva primária (EMPP) utilizando uma bateria neuropsicológica breve. MÉTODO: 66 pacientes com idade de 18 a 65 anos, escolaridade 3 a 18 anos, foram pareados a controles por sexo, idade e escolaridade; e avaliados. RESULTADO: No Symbol Digit Modalitie Test e Hooper Visual Organization Test 50 por cento com EMRR e 69 por cento com EMPP apresentaram desempenho comprometido. Na fluência da letra "F" o comprometimento foi de 24 por cento daqueles com EMRR e 81 por cento com EMPP. Na evocação imediata o comprometimento foi de 32 por cento na EMRR e 63 por cento na EMPP e, evocação tardia em 46 por cento na remitente-recorrente e 69 por cento com progressiva primária. CONCLUSÃO: Os perfis cognitivos dos pacientes com esclerose múltipla remitente-recorrente e progressiva primária são diferentes.

Seasonal distribution and evolving forms of multiple sclerosis patients diagnosed from April 2004 to November 2007 / Distribución estacional y formas evolutivas de esclerosis múltiple en pacientes diagnosticados entre Abril del 2004 y Noviembre del 2007

BACKGROUND: Multiple sclerosis (MS) was first reported in Cuba in 1965. The most frequent appearance is observed in the first six months of the year. OBJECTIVE: To determine the seasonal distribution and evolving forms of MS patients diagnosed with the disease between April 2004 and November 2007. METHOD: Twenty-one patients with suspected MS and 42 outbreaks were studied. Patients were classified according to Lublin and Revingold's criteria for clinical forms and according to McDonald. RESULTS: Most patients were classified in outbreak and remission and only two patients classified as primary-progressive multiple sclerosis. The higher number of outbreaks occurred in the first two quarters of the year. CONCLUSION: It is recommended to study further weather variables that may be related to the emergence of these outbreaks in our environment.

INTRODUCCIÓN: La esclerosis múltiple (EM) fue reportada en Cuba por primera vez en 1965. Se ha venido observando la aparición más frecuente en los primeros seis meses del año. OBJETIVO: Determinar la distribución estacional y formas evolutivas de EM en pacientes con diagnóstico de esta enfermedad entre abril 2004 y noviembre 2007. MÉTODO: Se estudiaron 21 pacientes con sospecha de EM y un total de 42 brotes. Los pacientes fueron clasificados según los criterios de Lublin y Revingold para las formas evolutivas y según los criterios de McDonald. RESULTADOS: Solo dos pacientes clasificaron en la forma evolutiva progresiva primaria; los primeros dos trimestres del año fueron los de mayor número de brotes. CONCLUSIÓN: Se recomienda profundizar en las variables climatológicas que pudieran tener vínculo con la aparición de estos brotes en nuestro medio.

Multiple sclerosis outcome and morbi-mortality of a Brazilian cohort patients / Caracteristicas clínico-evolutivas e morbi-mortalidade de uma coorte de pacientes brasileiros com esclerose múltipla

We studied the clinical and evolution characteristics of multiple sclerosis (MS) patients followed since the onset of HUCFF/UFRJ in 1978. The diagnosis of MS was based on Poser's et al. and MC Donald's et al. criteria. From 188 patients, 122 were included. Eighty-five were females. The mean age onset was 32.2 years-old (range 6.0 to 61.0±10.3), mainly Caucasians (82/67 percent). The relapsing-remitting course (MSRR) was more frequent (106/86.8 percent). Monosymptomatic onset was significantly more frequent in Caucasians than in Afro-Brazilians (p<0.05). Seventeen patients had benign form of MS and these patients presented association with MSRR when compared with severe form (p=0.01). The mortality rate was 2.12 percent (4 patients died). This study was similar to other Brazilian series with regard to sex and age, and lack of correlation between EDSS and number of relapses; it confirmed south-southeast African-descendants gradient distribution and association between first mono-symptomatic relapses and Caucasian; we found lower frequency of benign forms.

Estudamos as características clínico-evolutivas de pacientes com esclerose múltipla (EM) acompanhados no HUCFF-UFRJ desde 1978. Foram usados critérios de Poser et al. e MC Donald et al. para o diagnóstico de EM. De 188, 122 foram incluídos. Oitenta e cinco eram mulheres. A média de idade de início foi 32,2 anos (6,0-61,0±10,3), predominando caucasianos (n=82/67 por cento). A forma recorrente-remitente (EMRR) foi mais freqüente (n=106/86,8 por cento). Formas mono-sintomáticas no primeiro surto foram significativamente mais freqüentes em caucasianos do que em afro-brasileiros (p<0,05). Dezessete pacientes apresentavam a forma benigna (13,9 por cento) e 43 a grave (35,2 por cento). A forma benigna foi associada com a EMRR (p=0,01). A taxa de letalidade 2,12 por cento (4 óbitos). Nossos resultados são semelhantes aos de outras séries brasileiras no que se refere ao sexo e idade, e falta de correlação entre EDSS e número de surtos; confirmamos gradiente sul-sudeste de distribuição afro-descendente, associação significativa entre primeiro surto mono-sintomático e caucasianos e menor freqüência de formas benignas.

Benefit with methylprednisolone in continuous pulsetherapy in progressive primary form of multiple sclerosis: study of 11 cases in 11 years / Benefício do uso de pulsoterapia contínua com metilprednisolona na forma primária progressiva da esclerose múltipla: estudo de 11 casos em 11 anos

Primary progressive multiple sclerosis (PPMS) is defined clinically with a progressive course from onset. There is no approved treatment for the PPMS. Methylprednisolone IV (MP) hastens the recovery from MS relapses. We studied 11 patients that met the MacDonald's diagnostic criteria for PPMS. The dose of MP was 30 mg/kg in 250 mL of glucose solution in three consecutive days during the first week, two doses during the second and one dose in the third week. One weekly session for eight consecutive weeks was given. After, a once-a week/eight-week interval was maintained. The medium EDSS before treatment was 6.2, and after 11.2 years of treatment, the EDSS was 4.9. Although we studied a small sample of PPMS we may conclude that therapy with IVMP prevents clinical worsening of MS in the majority of patients with improvement in EDSS scores.

A forma progressiva da esclerose múltipla (FPEM) é definida como progressiva desde o início. Não há tratamento eficaz para esta forma. A metilprednisolona por via endovenosa (MPEV) é usada para os surtos de exacerbação da EM. Estudamos 11 pacientes que preenchiam os critérios de MacDonald para FPMS. A dose inicial de MPEV foi de 30 mg/kg em 250 mL de soro glicosado por três dias consecutivos na primeira semana, duas doses na segunda e uma dose na terceira semana. Seguiu-se uma sessão semanal por oito semanas. Após manteve-se uma dose semanal a cada oito semanas. A média do EDDS foi 9,6 antes e 4,9 após 11,2 anos de tratamento. Embora tenhamos estudado número reduzido de casos, podemos dizer que o uso de MPEV impede a progressão da FPEM na maioria dos pacientes estudados com melhora do EDDS.

Evaluation of ejection fraction changes during the course of treatment of mitoxantrone in patients with secondary progressive form of multiple sclerosis

Multiple sclerosis [MS] is one of the most common neurological disorders in young adults. Mitoxantrone, as an immunosuppressant and cytotoxic drug has shown a slight beneficial erect on the progressive form of the disease. However, it has been experienced that in cancer treatment this drug has caused cardiologic side effects such as congestive heart failure and decrease in ejection fraction. The aim of this study is to investigate the left ventricular ejection fraction changes induced by Mitoxantrone as administered in patients with secondary progressive form of multiple sclerosis. During the years of 2005-2007, the patients with secondary progressive MS who referred to the Neurological Clinic of Aria General Hospital in Mashhad were included in our cross-sectional study. These patients had no history of hematological, cardiac or hepatic disorders. Neither they had ever used immunosuppressant drugs. They were prescribed to intravenously take 12 mg/m2 of Mitoxantrone every 3 months for at least 2 years. Before each infusion of Mitoxantrone, the left ventricular ejection fraction [EF] was measured by echocardiography. After the treatment [8 infusions], the results of echocardiography were compared and analyzed through statistical methods using SPSS and Excel soft wares. Forty four patients with secondary progressive-MS were included in our study [24 females and 20 males]. Five of the subjects were younger than 30 year old, 27 patients were between 30 and 40, and 12 patients were above 40. The data was analyzed using the method of repeated measures. The results demonstrate that Mitoxantrone had no significant effect on the left ventricular ejection fraction. Also, the trend mean of EF during 8 months showed no significant drop. The mean of EF between sexes was not significant, either. As a whole, ten percent of the subjects treated with Mitoxanterone showed a decrease of EF which returned to its normal stage once the treatment was stopped. The benefit from using Mitoxanterone in multiple sclerosis was more than the complication caused by its usage. Multiple sclerosis, Mitoxantrone, Heart failure, echocardiography, Ejection fraction

Mitoxantrone reduced disability in Iranian patients with multiple sclerosis

Multiple sclerosis is a leading cause of disability in young adults. Mitoxantrone has recently been shown to be effective in ameliorating multiple sclerosis activity and reducing the decreasing relapse rate in patients with multiple sclerosis in Iran. This was a clinical trial on patients who received intravenous mitoxantrone, 12 mg/m[2] every 3 months. The study was performed at Isfahan Multiple Sclerosis Clinics, affiliated to Isfahan University of Medical Sciences. The clinical trial was conducted from October 2003 through April 2005. One hundred and forty-seven patients with worsening relapsing-remitting and secondary progressive multiple sclerosis received mitoxantrone, 12 mg/m[2] every 3 months. Clinical assessment was made every 3 months for one year. Of the 147 patients, 129 [93 females and 36 males] could successfully complete the course of our study. A significant therapeutic effect [P<0.0001] was detected for the attack rate which reduced to 0.09 [SD= 0.29] during treatment. Mitoxantrone was generally well tolerated and reduced progression of disability and clinical exacerbation in our patients. Physicians must be careful about the complications of mitoxantrone especially cardiotoxicity

Differential patterns of memory performance in relapsing, remitting and secondary progressive multiple sclerosis.

BACKGROUND: Memory dysfunction is common in multiple sclerosis (MS). A retrieval failure has been reported as the primary cause for the memory deficits, although some studies also described a faulty acquisition. AIMS: The aim of the study was to examine memory function in relapsing remitting (RR) and secondary progressive (SP) MS patients, analyze the patterns of performance and to investigate whether disease course influences this performance. DESIGN AND SETTINGS: Case-control prospective study conducted in a clinical setting. MATERIALS AND METHODS: Fifty-five RR, 23 SP MS patients and 80 normal subjects were evaluated with a comprehensive neuropsychological battery. Memory was assessed with tasks from the Signoret memory battery. Attention and executive function were also assessed. STATISTICAL ANALYSIS: Univariate analysis of variance, Mann-Whitney U-test, multivariate logistic regression and Chi-square test were used as appropriate. RESULTS: MS patients performed significantly worse than controls on almost all measures of memory (P < 0,001). MS subgroups differed in tasks of delayed recall (logical memory- P =0,019; wordlist delayed recall, P < 0,001), semantic cued recall (P < 0,001), recognition trials (P =0,006) rate of forgetting (P < 0,001) and confabulation and intrusion errors (P =0,004). CONCLUSIONS: Memory is consistently impaired in MS patients and disease course differentially affects the pattern of performance. SP patients show greater difficulties and a more pervasive pattern of dysfunction than RR patients. Delayed recall was the most affected memory measure and performance on this task discriminates between RR and SP MS patients. Relapsing remitting patients performed within the mildly impaired range while SP patients showed a moderate to severe impairment.

ADC measurements in various patterns of multiple sclerosis lesions.

OBJECTIVE: To determine the difference of mean apparent diffusion coefficients (ADC) among different patterns of focal multiple sclerosis (MS) lesions, to compare mean lesion ADC between 2 clinical subgroups and to correlate mean lesion ADC with disability. MATERIAL AND METHOD: Thirty seven patients (26 with relapsing-remitting multiple sclerosis (MS) and 11 with secondary-progressive MS) underwent both conventional and diffusion-weighted MR imaging of the brain. After creating ADC maps, region identification was done by using b = 0 images and T2-weighted images. ADC values were measured for MS lesions and (NAWM). RESULTS: A total of 288 lesions were identified on the images. The mean ADC for the lesions was significantly higher than that of NAWM Hypointense T1 lesions (n = 221) had a significantly higher mean ADC than isointense T1 lesions (n = 67) in both nonenhancing lesions (n = 250) and enhancing lesions (n = 38). The enhanced rim of ring-enhancing lesions (n = 18) had lower ADC than the central nonenhanced portions. Confluent lesions (n = 62) had a substantially higher mean ADC than discrete lesion (n = 226). Mean lesion ADC of secondary progressive MS was significantly higher than relapsing remitting MS. No correlation between mean lesion ADC and (EDSS) score was found CONCLUSION: Quantitative diffusion-weighted imaging is useful to elucidate the heterogeneous pathological substrate of MS in different patterns of MS lesions, to differentiate 2 major clinical subgroups.