RESUMO
BACKGROUND AND OBJECTIVE: Anticipation of age at onset in the younger generations is a widely known characteristic of many diseases with genetic inheritance. This study was performed to assess whether there is anticipation of age at onset in younger generations of familial multiple sclerosis (MS) in a Spanish population and to compare clinical characteristics of familial and sporadic MS. METHODS: We studied a cohort of 1110 patients diagnosed with MS and followed-up in our MS Unit. Patients were considered as familial MS if they had in their family at least one relative of first or second degree diagnosed with MS. Otherwise, patients were considered to have sporadic MS. We compared the age at onset between relatives from different generations, and we also compared the age at onset of familial and sporadic MS. RESULTS: A lower age at onset in the younger generations was found (median 22 years vs. 30 years, P < 0.001) and a significant lower age at onset of the disease in familial MS comparing to sporadic MS (median 25 years vs. 29 years, P = 0.042). CONCLUSIONS: There is an anticipation of the age at onset of MS in the younger generations of patients with familial MS. There is also a lower age at onset in familial versus sporadic MS.
Assuntos
Antecipação Genética , Esclerose Múltipla Crônica Progressiva/epidemiologia , Esclerose Múltipla Crônica Progressiva/psicologia , Esclerose Múltipla Recidivante-Remitente/epidemiologia , Esclerose Múltipla Recidivante-Remitente/psicologia , Adulto , Idade de Início , Estudos de Coortes , Bases de Dados Factuais , Família , Feminino , Seguimentos , Humanos , Estimativa de Kaplan-Meier , Masculino , Prevalência , Sistema de Registros , Estudos Retrospectivos , Espanha/epidemiologia , Adulto JovemRESUMO
BACKGROUND: Scarce data exist about multiple sclerosis (MS) prevalence in South America. The objective of the study is to determine the prevalence of MS in a high populated area from Argentina (Greater Buenos Aires Metropolitan area) using the capture-recapture methodology. METHODS: Greater Buenos Aires is the generic denomination that refers to the megalopolis comprised by the autonomous city of Buenos Aires and the surrounding conurbation of the province of Buenos Aires. The study was carried out taking July 1996 as the prevalence month. We used capture-recapture method to estimate the prevalence of MS cross matching registries from four MS Centers. RESULTS: A total of 803 registries were obtained from the four lists. Log-linear model for capture-recapture method was used to analyze the data. The population of the area based on the 1990 census was 12,594,974; the number of MS cases estimated amongst sources interactions were between 1833 and 2359; the prevalence estimated ranged from 14 to 19.8 cases per 100,000 inhabitants. CONCLUSIONS: This is the first study to provide epidemiological data on the prevalence of MS in a large population in Argentina (Greater Buenos Aires Metropolitan area). Further epidemiological studies will clarify the true prevalence of MS in South America.
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Esclerose Múltipla/epidemiologia , Argentina/epidemiologia , Humanos , Modelos Estatísticos , Prevalência , Sistema de RegistrosRESUMO
BACKGROUND: Glucocorticoid treatment improves the speed of functional recovery of acute multiple sclerosis (MS) relapses but has not been shown to provide any long-term functional benefit. There is currently no convincing evidence that the clinical benefit is influenced by the route of administration or the dosage of glucocorticoid, or the particular glucocorticoid prescribed. Recent studies support similarities in the bioequivalence and in the clinical effect of high dose oral corticosteroids for MS relapses. OBJECTIVE: This survey aimed to determine the relapse treatment preferences of clinicians in Canadian MS clinics. METHODS: Members of the Canadian Network of MS Clinics are linked by an email server. A one page survey was distributed to the group to determine and report use of corticosteroids to manage MS relapses amongst Canadian MS specialists. RESULTS: Fifty-one clinicians from 17 MS clinics were surveyed. 32 (63%) surveys were returned representing 16 clinics. Five doses are most commonly prescribed, usually without a taper. Three or four doses and the use of a corticosteroid taper, however, are not uncommon. Gastric cytoprotection and sedatives are often prescribed for use as needed. CONCLUSION: This survey illustrates that when Canadian clinicians with expertise in managing MS treat MS relapses they choose high dose corticosteroids, either oral or i.v. The results therefore represent Canadian practice as these clinicians provide direct patient care and influence care by community neurologists. Until evidence clearly identifies a superior practice all options should be available to clinicians and their patients.
Assuntos
Corticosteroides/administração & dosagem , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Padrões de Prática Médica/estatística & dados numéricos , Administração Oral , Canadá , Humanos , Injeções IntravenosasRESUMO
Gut microbiome interaction goes beyond commensal function as vitamin production or support nutrients digestion. It also interplays with the host immune system and may be related to the development of immune-mediated diseases. Multiple sclerosis patients have dysbiosis compared to healthy individuals. But how this relates to disease development and severity is still uncertain. Dietary change including probiotic mixtures or ketogenic regimen has proven to change microbiome in multiple sclerosis (MS) subjects to one similar to healthy controls. However, proof of clinical benefits is lacking. We dissert on current knowledge about immune system and gut bacteria interactions. We discuss faecal microbial transplantation as a potential intervention to ameliorate gut dysbiosis in MS as well as the caveats of a clinical trial design.
Assuntos
Disbiose/terapia , Transplante de Microbiota Fecal , Microbioma Gastrointestinal/imunologia , Esclerose Múltipla/terapia , Disbiose/imunologia , Disbiose/microbiologia , Interações entre Hospedeiro e Microrganismos/imunologia , Humanos , Intestinos/imunologia , Intestinos/microbiologia , Esclerose Múltipla/imunologia , Esclerose Múltipla/microbiologia , Probióticos/uso terapêuticoRESUMO
BACKGROUND AND PURPOSE: Recently published North American Imaging in Multiple Sclerosis guidelines call for derivation of a specific radiologic definition of MS WM lesions and mimics. The purpose of this study was to use SWI and magnetization-prepared FLAIR images for sensitive differentiation of MS from benign WM lesions using the morphologic characteristics of WM lesions. MATERIALS AND METHODS: Seventeen patients with relapsing-remitting MS and 18 healthy control subjects were enrolled retrospectively. For each subject, FLAIR and multiecho gradient-echo images were acquired using 7T MR imaging. Optimized postprocessing was used to generate single-slice SWI of cerebral veins. SWI/FLAIR images were registered, and 3 trained readers performed lesion assessment. Morphology, location of lesions, and the time required for assessment were recorded. Analyses were performed on 3 different pools: 1) lesions of >3 mm, 2) nonconfluent lesions of >3 mm, and 3) nonconfluent lesions of >3 mm with no or a single central vein. RESULTS: The SWI/FLAIR acquisition and processing protocol enabled effective assessment of central veins and hypointense rims in WM lesions. Assessment of nonconfluent lesions with ≥1 central vein enabled the most specific and sensitive differentiation of patients with MS from controls. A threshold of 67% perivenous WM lesions separated patients with MS from controls with a sensitivity of 94% and specificity of 100%. Lesion assessment took an average of 12 minutes 10 seconds and 4 minutes 33 seconds for patients with MS and control subjects, respectively. CONCLUSIONS: Nonconfluent lesions of >3 mm with ≥1 central vein were the most sensitive and specific differentiators between patients with MS and control subjects.
Assuntos
Leucoaraiose/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Esclerose Múltipla Recidivante-Remitente/diagnóstico por imagem , Neuroimagem/métodos , Adulto , Veias Cerebrais/patologia , Diagnóstico Diferencial , Feminino , Humanos , Interpretação de Imagem Assistida por Computador/métodos , Leucoaraiose/patologia , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Sensibilidade e Especificidade , Substância Branca/diagnóstico por imagem , Substância Branca/patologiaRESUMO
The clinical features of relapses and progression largely define multiple sclerosis phenotypes. A relapsing course is followed by chronic progression in some 80% of cases within 2 decades. The relationship between these phases and long-term outcome remains uncertain. We have analysed these clinical features within a well-studied natural history cohort with mean follow-up of 25 years. For the entire cohort, median times to reach Disability Status Scale (DSS) 6, 8 and 10 were 12.7, 20.6 and 43.9 years, respectively. Among 824 attack-onset patients, the great majority entered a progressive phase with a mean time to progression of 10.4 years. The effects of relapses often cloud the clinical onset of progression. However, there are circumstances where onset of progression is early, relatively discrete and identifiable at DSS of 2 or less. Three subgroups allow for clarity of outcome comparison and they are (i) cases of primary progressive (PP) disease, (ii) attack-onset disease where only a single attack has occurred before onset of progression (SAP) and (iii) secondary progressive (SP) disease where recovery from relapses allows recognition of the earliest clinical stages when progression begins. Here we compare survival curves in these three groups. Among cohorts of SAP (n = 140), PP (n = 219) and SP (n = 146) where progression was stratified by DSS at its onset, there was no difference in time to DSS 6, 8 and 10. These findings demonstrate that the progressive course is independent of relapses either preceding the onset of relapse-free progression or subsequent to it. Among SAP patients, the degree of recovery from the single defining exacerbation had no significant effect on outcome. The site of the original attack was not usually where progression began. The relatively stereotyped nature of the progressive phase seen in all progressive phenotypes suggests regional and/or functional differential susceptibility to a process that appears degenerative in nature. The highly prevalent distal corticospinal tract dysfunction in progressive disease and the pathologically demonstrated selective axonal loss seen in this tract raises the possibility of a dying back central axonopathy, at least in part independent of plaque location or burden. Despite considerable individual variation, the progressive course of disability seen in groups of PP, SAP and SP-DSS2 is similarly stereotyped in quality and pace and may entail mechanisms common to all forms of progressive multiple sclerosis. The possibility that this is the primary process in some cases must be considered.
Assuntos
Esclerose Múltipla Crônica Progressiva/fisiopatologia , Adolescente , Adulto , Idade de Início , Avaliação da Deficiência , Progressão da Doença , Métodos Epidemiológicos , Feminino , Humanos , Masculino , Esclerose Múltipla Crônica Progressiva/diagnóstico , Esclerose Múltipla Crônica Progressiva/patologia , Prognóstico , Recidiva , Fatores de TempoRESUMO
Only 12 AIDS cases with hemichorea were reported in the literature. We report the first case of hemichorea associated with AIDS and cerebral toxoplasmosis in our country. A 26-year-old man had 3 episodes of focal seizures on the left side with subsequent loss of consciousness. A few weeks later, he noticed progressive left-sided weakness. Examination revealed a left hemiparesis. MRI of the head showed a round mass in the right frontal lobe and a smaller lesion in the left temporo-occipital area. Laboratory showed positive serum ELISA and Western Blot analysis for HIV antibodies. Serum tests for Toxoplasma showed elevated titers. He was treated with pyrimethamine and sulfadiazine. His weakness improved and he had no further seizures. Two weeks later, choreic movements appeared in the left foot, finally involving the entire left hemibody. A second MRI showed a new small lesion in the right cerebral peduncle. The patient completed 6 weeks of treatment, with further reduction in the size of the lesions. Nevertheless, the left hemichorea persisted. We believe that the hemichorea our patient had was caused by the contralateral peduncular lesion. Lesions involving the subthalamic nucleus or its connections may cause contralateral hemiballismus or hemichorea. In spite of the favorable response to antitoxoplasmic therapy, the hemichorea persisted. The present report illustrates an uncommon neurological complication in AIDS. We believe that a combination of a focal cerebral lesion and the HIV infection caused the movement disorder presented by the patient.
Assuntos
Síndrome da Imunodeficiência Adquirida/complicações , Coreia/complicações , Toxoplasmose Cerebral/complicações , Adulto , Humanos , Imageamento por Ressonância Magnética , Masculino , Toxoplasmose Cerebral/patologiaRESUMO
We report 10 HTLV-I virus seropositive subjects, eight of them with HTLV-I associated myelopathy (HAM), two of them also infected with HIV as well as two asymptomatic HTLV-I+ relatives of two unrelated patients. HTLV-I is endemic in several tropical areas, where it causes different neurological diseases. Only few patients have been reported in our country since 1994. We studied 8 patients, who fulfilled the clinical criteria for chronic spastic paraplegia, and 2 other non-symptomatic HTLV-I seropositive relatives, with electromyography (EMG), motor and sensory conduction velocities (NCV), somatosensory, visual and brainstem auditory evoked potentials (SSEP, VEP and BAEP), Magnetic Resonance Images (MRI) and cerobrospinal fluid (CSF) analysis. The latter was carried out only in seven symptomatic patients. In every case positive ELISA tests for HTLV-I/II were confirmed by Western Blot. The two asymptomatic persons were clinically and electromyographically assessed, one of them was also submitted to SSEPs studies. Three patients were males. Patient's ages ranged from 5 to 65 years old. All symptomatic patients showed muscular weakness, spasticity with pyramidal signs and sphincter disturbances. Five of them had paresthesias and 2 had burning pain on their feet. The EMGs and the NCVs were normal in 7 patients and in the 2 asymptomatic ones. SSEPs, obtained by stimulating the posterior tibial nerves, were impaired in 7 patients and in the asymptomatic person who received the procedure. The 7 symptomatic patients who underwent lumbar puncture had positive tests for HTLV-I in CSF, 3 out of these 7 patients had also high protein levels and 4 had increased number of lymphocytes. In 2 patients intrathecal IgG production could also be demonstrated. MRI were normal in 7 patients and in the 2 asymptomatics, the exception being a female who had bilateral hyperintense lesions in cerebral white matter in T2. In conclusion, tropical spastic paraparesis is apparently a rare disorder in Argentina. However, some cases have been reported recently. Most probably, its prevalence is currently underestimated. Its diagnosis should be considered in every patient with progressive spastic paraplegia.
Assuntos
Potenciais Evocados , Paraparesia Espástica Tropical/fisiopatologia , Adulto , Idoso , Argentina/epidemiologia , Eletromiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Paraparesia Espástica Tropical/líquido cefalorraquidiano , Paraparesia Espástica Tropical/epidemiologiaRESUMO
INTRODUCTION: Multiple sclerosis is one of the commonest idiopathic inflammatory diseases of the central nervous system in young adults. The study of the natural history of multiple sclerosis implies in the first place, establishing precise limits for the diagnosis of the disease, which is still of unknown origin. It is also necessary to analyze the wide range of varieties, which as different categories or subgroups and with different degrees of severity, make up the clinical spectrum of this entity. DEVELOPMENT AND CONCLUSIONS: Throughout this review benign and malignant multiple sclerosis, remitting and progressive forms, are studied from epidemiological and clinical points of view in the context of large populations of patients with multiple sclerosis on long-term follow up at a specialized centre, with the objective of finding, if possible, the patterns of the natural history of the disease.
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Esclerose Múltipla , Adulto , Encéfalo/fisiopatologia , Humanos , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/etiologia , Esclerose Múltipla/fisiopatologiaRESUMO
INTRODUCTION: With the aim of integrating clinical thinking and obtaining epidemiological and biostatistical results in long series of patients who present balance disorders, we have developed a neuro otological protocol and its corresponding database. This offers answers to the questions of how to assess the patient, how to conduct a clinical interview correctly, and which complementary studies should be requested and how. RESULTS: The information from the protocol is stored and analysed in our database, which was designed in the Epi Info software application, produced by CDC (NIH, USA), in collaboration with the WHO Global Program on AIDS. The application, which we call ENO LK, has already been used to store data concerning 1,100 patients whose average age is 54.5 years old and 62.8% of which were females (SD 18, range 4 93). 69.1% were diagnosed as suffering from vertigo, 12.7% displayed instability, 1.9% syncope and 16.3% had other causes (37% psychogenic and 28% disorders affecting the central integrator). Of the 760 patients with vertigo, in 55% it was positional (60% of these were idiopathic benign), 6.3% were sustained (peripheral causes accounted for 74% and a vascular aetiology was predominant in the central causes), 26.6% were recurring and 12.1% otolithic (in this series the vertigos all had a central aetiology). CONCLUSIONS: With this application the user has the possibility of obtaining epidemiological and diagnostic conclusions efficiently and effectively, as well as aiding to follow up all patients who present balance disorders.
Assuntos
Processamento Eletrônico de Dados/instrumentação , Equilíbrio Postural/fisiologia , Transtornos de Sensação/diagnóstico , Adolescente , Adulto , Encefalopatias/diagnóstico , Criança , Pré-Escolar , Diagnóstico Diferencial , Diplopia/diagnóstico , Otopatias/diagnóstico , Eletronistagmografia , Feminino , Humanos , Masculino , Transtornos Mentais/diagnóstico , Pessoa de Meia-Idade , Nistagmo Optocinético , Movimentos Sacádicos/fisiologia , Vertigem/diagnósticoRESUMO
PATIENTS AND METHODS: We analysed the records of the individuals who were attended because of dizziness or vertigo in the vestibular sector, with the aim of describing the epidemiological clinical profile of a group of patients with such symptoms. 1300 patients were systematically evaluated according to our neuro otological examination protocol. Diagnoses were ordered, according to the international classification reported by Drachman and later modified by Bahlo, in four categories: 1. Vertigo, 2. Instability, 3. Pre syncope and 4. Miscellaneous. Each of these classes was organised according to the topography of the lesion and these were in turn grouped by aetiologies (viral, vascular, tumoural, demyelinating, post traumatic, idiopathic, autoimmune, etc.). The data were stored and analysed in a computer database, Epi info 6.02 (OMS 1994), which was especially adapted by the researchers for the purpose. RESULTS: 63.1% were women. The average age was 55.5 years old (SD: 17.5, interval: 4 93). Vertigo was diagnosed in 68.9%, instability was found in 12.4%, 1.8% presented syncope and miscellaneous disorders occurred in 16.9% (of these, 64.1% had disorders of the central integrator and 16.4% were of a psychogenic origin). Of the 1300 patients, 896 presented vertigo; the positional type was seen in 54%, sustained in 6.5%, recurrent in 27.7% and 11.8% were found to have the otolithic type. CONCLUSIONS: The relevance of the epidemiological work based on clinical evaluation and the thorough neuro otological examination in our medium must be highlighted. These findings were similar to those reported in the international literature in more delimited series.
Assuntos
Tontura/epidemiologia , Tontura/etiologia , Vertigem/epidemiologia , Vertigem/etiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Interpretação Estatística de Dados , Tontura/diagnóstico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Vertigem/diagnósticoAssuntos
Encéfalo/diagnóstico por imagem , Fatores Imunológicos/efeitos adversos , Leucoencefalopatia Multifocal Progressiva/diagnóstico por imagem , Leucoencefalopatia Multifocal Progressiva/etiologia , Imageamento por Ressonância Magnética , Natalizumab/efeitos adversos , Adulto , Encéfalo/efeitos dos fármacos , Meios de Contraste , Diagnóstico Diferencial , Feminino , Gadolínio , Humanos , Fatores Imunológicos/uso terapêutico , Esclerose Múltipla Recidivante-Remitente/diagnóstico por imagem , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Natalizumab/uso terapêuticoRESUMO
OBJECTIVE: To examine the effects of interferon beta (IFNß)-1b on all-cause mortality over 21 years in the cohort of 372 patients who participated in the pivotal randomized clinical trial (RCT), retaining (in the analysis) the original randomized treatment-assignments. METHODS: For this randomized long-term cohort study, the primary outcome, defined before data collection, was the comparison of all-cause mortality between the IFNß-1b 250 µg and placebo groups from the time of randomization through the entire 21-year follow-up interval (intention-to-treat, log-rank test for Kaplan-Meier survival curves). All other survival outcomes were secondary. RESULTS: After a median of 21.1 years from RCT enrollment, 98.4%(366 of 372) of patients were identified, and, of these, 81 deaths were recorded (22.1% [81 of 366]). Patients originally randomly assigned to IFNß-1b 250 µg showed a significant reduction in all-cause mortality over the 21-year period compared with placebo (p = 0.0173), with a hazard ratio of 0.532 (95% confidence interval 0.314-0.902). The hazard rate of death at long-term follow-up by Kaplan-Meier estimates was reduced by 46.8% among IFNß-1b 250 µg-treated patients (46.0% among IFNß-1b 50 µg-treated patients) compared with placebo. Baseline variables did not influence the observed treatment effect. CONCLUSIONS: There was a significant survival advantage in this cohort of patients receiving early IFNß-1b treatment at either dose compared with placebo. Near-complete ascertainment, together with confirmatory findings from both active treatment groups, strengthens the evidence for an IFNß-1b benefit on all-cause mortality. CLASSIFICATION OF EVIDENCE: This study provides Class III evidence that early treatment with IFNß-1b is associated with prolonged survival in initially treatment-naive patients with relapsing-remitting multiple sclerosis.
Assuntos
Adjuvantes Imunológicos/uso terapêutico , Interferon beta/uso terapêutico , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Esclerose Múltipla Recidivante-Remitente/mortalidade , Adulto , Idade de Início , Causas de Morte , Feminino , Humanos , Interferon beta-1b , Estimativa de Kaplan-Meier , Masculino , Análise de SobrevidaRESUMO
Anti-myelin basic protein (MBP) antibodies in pediatric-onset MS and controls were characterized. Serum samples were obtained from 94 children with MS and 106 controls. Paired CSF and serum were obtained from 25 children with MS at time of their initial episode of acute demyelinating syndrome (ADS). Complementary assays were applied across samples to evaluate the presence, and the physical binding properties, of anti-MBP antibodies. While the prevalence and titers of serum anti-MBP antibodies against both immature and mature forms of MBP were similar in children with MS and in controls, binding characteristics and formal Surface Plasmon Resonance (SPR) studies indicated surprisingly high binding affinities of all pediatric anti-MBP antibodies. Serum levels of anti-MBP antibodies correlated significantly with their CSF levels, and their presence in children with MS was associated with significantly increased risk of an acute disseminated encephalomyelitis-like initial clinical presentation. While antibodies to both immature and mature forms of MBP can be present as part of the normal pediatric humoral repertoire, these anti-myelin antibodies are of surprisingly high affinity, can access the CNS during inflammation, and have the capacity to modulate disease expression. Our findings identify an immune mechanism that could contribute to the observed heterogeneity in spectrum of clinical presentations in early-onset MS.
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Autoanticorpos/fisiologia , Esclerose Múltipla/diagnóstico , Esclerose Múltipla/imunologia , Bainha de Mielina/imunologia , Proteínas do Tecido Nervoso/imunologia , Fatores de Transcrição/imunologia , Doença Aguda , Adolescente , Autoanticorpos/sangue , Autoanticorpos/líquido cefalorraquidiano , Biomarcadores/sangue , Biomarcadores/líquido cefalorraquidiano , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Esclerose Múltipla/sangue , Esclerose Múltipla/líquido cefalorraquidiano , Proteína Básica da Mielina , Proteínas do Tecido Nervoso/sangue , Proteínas do Tecido Nervoso/líquido cefalorraquidiano , Fatores de Risco , Síndrome , Fatores de Transcrição/sangue , Fatores de Transcrição/líquido cefalorraquidiano , Adulto JovemRESUMO
Approximately 85% of multiple sclerosis (MS) cases begin as clinically isolated syndromes (CIS). Results from the Controlled High-Risk Subjects Avonex((R)) Multiple Sclerosis Prevention Study (CHAMPS) demonstrated that, in patients with CIS, treatment with intramuscular (IM) interferon beta-1a (IFNbeta-1a) 30 mug once weekly delayed conversion to clinically definite MS (CDMS) in the total population and in subgroups based on presenting syndromes and baseline magnetic resonance imaging (MRI) characteristics. Changes to clinical and MRI risk classification of presenting symptoms in recent studies prompted reanalysis of CHAMPS data. Presenting syndromes were assessed using a derived algorithm that stratifies patients into mono- or multifocal categories based on functional system scores. The ability of IM IFNbeta-1a to delay progression to CDMS in subgroups based on clinical presentation and MRI characteristics was assessed. Reanalysis of CHAMPS patients showed that 30% could be classified by clinical criteria as having multifocal disease at baseline. IM IFNbeta-1a initiated at a first demyelinating attack delayed CDMS in monofocal patients (P = 0.0013), patients with or without gadolinium-enhancing lesions (P = 0.0007, P = 0.0405) and patients with at least nine T2 lesions at baseline (P = 0.0044). These data confirm that IM IFNbeta-1a delays conversion to CDMS in patients with CIS.
Assuntos
Adjuvantes Imunológicos/administração & dosagem , Interferon beta/administração & dosagem , Esclerose Múltipla , Adulto , Algoritmos , Encéfalo/patologia , Progressão da Doença , Feminino , Gadolínio , Humanos , Injeções Intramusculares , Interferon beta-1a , Imageamento por Ressonância Magnética , Masculino , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla/epidemiologia , Esclerose Múltipla/prevenção & controle , Análise Multivariada , Medição de Risco/métodos , Fatores de RiscoRESUMO
There are few studies reporting multiple sclerosis prevalence rates in the Buenos Aires region, Argentina (latitude 34 degrees S) (between 12-18.5/100 000 inhabitants), and no studies have been performed in the larger region between parallels 36 degrees and 55 degrees S. The aim of this study is to determine the prevalence rates and clinical features of multiple sclerosis in residents of the Argentine Patagonia. Four cities from the region were selected for this study, giving a sample population of 417 666 inhabitants (approximately 24% of the total Patagonia population). 1(st) March 2002 was determined as prevalence day. Patients were ascertained using multiple case-finding methods. The point prevalence rate was 17.2/100 000 (17.2 age-adjusted to the world population). Prevalence rates were higher for women than for men, 22.1 versus 12.2/100 000 inhabitants (21.4 versus 12.7 sex-adjusted to the world population). The study population was mainly of European descent and mestizoes. Clinical features were similar to those reported in other countries. This study shows that Argentine Patagonia is a medium-risk area with no south-north gradient between parallels 55 degrees and 36 degrees S. The Patagonia population shows recent internal migration that makes it difficult to determine whether the exposure to potential risk factors has been long enough to modify the disease incidence.
Assuntos
Esclerose Múltipla Crônica Progressiva/epidemiologia , Esclerose Múltipla Recidivante-Remitente/epidemiologia , Adolescente , Adulto , Argentina/epidemiologia , Feminino , Humanos , Incidência , Indígenas Sul-Americanos/estatística & dados numéricos , Masculino , Pessoa de Meia-Idade , Dinâmica Populacional , Prevalência , Fatores de Risco , Distribuição por Sexo , População Branca/estatística & dados numéricosRESUMO
BACKGROUND: Epidemiological and ecological studies suggest links between vitamin D deficiency and increased multiple sclerosis (MS) prevalence. OBJECTIVE: To evaluate the safety and tolerability of oral calcitriol therapy in an open label pilot study. METHODS: 15 ambulatory patients with relapsing-remitting MS and at least one clinical relapse within the previous 12 months received oral calcitriol (target dose: 2.5 microg/d) for 48 weeks. Dietary calcium was restricted to 800 mg/d. Patients were monitored using frequent clinical and laboratory examinations, the expanded disability status scale (EDSS), and brain magnetic resonance imaging (MRI). RESULTS: Two patients withdrew because of symptomatic hypercalcaemia (serum calcium >3.35 mmol/l in each case) resulting from persistent dietary indiscretion. Two diet compliant patients required temporary dose adjustments for mild asymptomatic hypercalcaemia. Diet compliant patients experienced mild adverse effects. The on-study exacerbation rate (27%) was less than baseline. Four patients experienced five clinical relapses but only one patient worsened by >1 EDSS point. Brain MRI revealed enhancing lesions in five patients at baseline (33%) and in four (29%) at both 24 and 48 weeks. CONCLUSIONS: Oral calcitriol is safe and well tolerated for up to one year by diet compliant relapsing-remitting MS patients. Further study of vitamin D related mechanisms is warranted in MS.
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Calcitriol/uso terapêutico , Agonistas dos Canais de Cálcio/uso terapêutico , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla/patologia , Administração Oral , Adulto , Calcitriol/administração & dosagem , Calcitriol/efeitos adversos , Agonistas dos Canais de Cálcio/administração & dosagem , Agonistas dos Canais de Cálcio/efeitos adversos , Feminino , Humanos , Masculino , Periodicidade , Recidiva , Índice de Gravidade de DoençaRESUMO
The clinical course of multiple sclerosis can be classified as relapsing from onset (relapsing-remitting), or progressive from onset (primary progressive - PPMS). These clinical phenotypes have been based on historical and clinical observations. It has been reported that PPMS patients tend to have quantitatively less MRI activity and disease burden. We evaluated the sensitivity and diagnostic value of conventional brain MRI scan in 143 PPMS patients. Brain MRIs were blindly evaluated to determine if they satisfied Paty and/or Fazekas diagnostic criteria. Patients were divided into those with typical, atypical or normal scans. They satisfied brain MRI criteria in 92% cases. Findings included: 131 typical, four atypical, and eight normal scans. All 12 non-typical scans' subjects had spinal onset; spinal MRI scans were positive in four of seven cases. Sex, age of onset, site and number of symptoms involved at onset among those groups were not significantly different but accumulation of disability had a tendency to be slower in these few individuals with normal or atypical head MRI's. Although there may be quantitative differences in lesion activity/burden, MRI scanning in PPMS unexpectedly has diagnostic sensitivity very similar to that seen in RRMS. A normal brain MRI is unusual in PPMS patients.
Assuntos
Encéfalo/patologia , Imageamento por Ressonância Magnética/normas , Esclerose Múltipla Crônica Progressiva/diagnóstico , Adulto , Estudos de Coortes , Pessoas com Deficiência , Estudos de Avaliação como Assunto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla Crônica Progressiva/fisiopatologia , Esclerose Múltipla Recidivante-Remitente/diagnóstico , Esclerose Múltipla Recidivante-Remitente/fisiopatologia , Sensibilidade e Especificidade , Método Simples-Cego , Medula Espinal/patologiaRESUMO
We report a natural history study of 216 patients with primary progressive (PP)- multiple sclerosis defined by at least 1 year of exacerbation-free progression at onset. This represents 19.8% of a largely population-based patient cohort having a mean longitudinal follow-up of 23 years. This subgroup of PP-multiple sclerosis patients had a mean age of onset of 38.5 years, with females predominating by a ratio of 1.3:1.0. The rate of deterioration from disease onset was substantially more rapid than for relapsing-remitting multiple sclerosis, with a median time to disability status score (DSS) 6 and DSS 8 of 8 and 18 years, respectively. Forty-nine percent of patients were followed through to death. Examination of the early disease course revealed two groups with adverse prognostic profiles. Firstly, a shorter time to reach DSS 3 from onset of PP-multiple sclerosis significantly adversely influenced time to DSS 8. Second, involvement of three or more neurological systems at onset resulted in a median time to DSS 10 of 13.5 years in contrast to PP-multiple sclerosis patients with one system involved at onset where median time to death from multiple sclerosis was 33.2 years. However, age, gender and type of neurological system involved at onset appeared to have little influence on prognosis. Life expectancy, cause of mortality and familial history profile were similar in PP-multiple sclerosis and non-PP-multiple sclerosis (all other multiple sclerosis patients from the total population). From clinical onset, rate of progression was faster in the PP-multiple sclerosis group than in the secondary progressive (SP)-multiple sclerosis group. When the rates of progression from onset of the progressive phase to DSS 6, 8 and 10 were compared, SP-multiple sclerosis had a more rapid progressive phase. A substantial minority (28%) of the PP-multiple sclerosis cohort had a distinct relapse even decades after onset of progressive deterioration. These studies establish natural history outcomes for the subgroup of multiple sclerosis patients with primary progressive disease.