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BACKGROUND: Natalizumab and fingolimod are used as high-efficacy treatments in relapsing-remitting multiple sclerosis. Several observational studies comparing these two drugs have shown variable results, using different methods to control treatment indication bias and manage censoring. The objective of this empirical study was to elucidate the impact of methods of causal inference on the results of comparative effectiveness studies. METHODS: Data from three observational multiple sclerosis registries (MSBase, the Danish MS Registry and French OFSEP registry) were combined. Four clinical outcomes were studied. Propensity scores were used to match or weigh the compared groups, allowing for estimating average treatment effect for treated or average treatment effect for the entire population. Analyses were conducted both in intention-to-treat and per-protocol frameworks. The impact of the positivity assumption was also assessed. RESULTS: Overall, 5,148 relapsing-remitting multiple sclerosis patients were included. In this well-powered sample, the 95% confidence intervals of the estimates overlapped widely. Propensity scores weighting and propensity scores matching procedures led to consistent results. Some differences were observed between average treatment effect for the entire population and average treatment effect for treated estimates. Intention-to-treat analyses were more conservative than per-protocol analyses. The most pronounced irregularities in outcomes and propensity scores were introduced by violation of the positivity assumption. CONCLUSIONS: This applied study elucidates the influence of methodological decisions on the results of comparative effectiveness studies of treatments for multiple sclerosis. According to our results, there are no material differences between conclusions obtained with propensity scores matching or propensity scores weighting given that a study is sufficiently powered, models are correctly specified and positivity assumption is fulfilled.
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Esclerose Múltipla Recidivante-Remitente , Esclerose Múltipla , Cloridrato de Fingolimode/uso terapêutico , Humanos , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Natalizumab/uso terapêutico , Resultado do TratamentoRESUMO
Haemovigilance and blood safety became closely linked initially to the quality system, then very quickly to risk management in health-care institutions. The complexity of the transfusion process and the uniqueness of the transfusion act have been the source of a wide range of in-depth analyzes, via methods most often derived from the industry, that aimed at preventing the occurrence of unwanted situations with potential adverse effects on transfused patients. Though these methods have widely been successful, the experience gained over more than two decades indicates that many reported incidents are still avoidable. The time devoted to training, file analyzes (even in the absence of any reported incident), and simulating patient care remains essential. It is fundamental to bear in mind that there is no such thing as risk zero, no one is immune, and this does not happen to others. In this context, developing a different perspective, a new reflection, a vision integrating the complexity of fieldwork and the idea of risk may be an approach worthy of investigation. Indeed, an analysis centered on both of the ways a team copes with difficult situations and the variety of the interactions between professionals would allow medical practitioner to better position themselves through a better assessment and understanding of their role. Such a deeper reflection, through the mobilization of all the stakeholders of the many successful steps of the transfusion process, would increase blood safety and the overall resilience of the system.
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Segurança do Sangue , Transfusão de Sangue , Pessoal de Saúde , Humanos , Gestão de RiscosRESUMO
Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Approximately 75% of patients have antibodies against aquaporin-4, a water channel expressed on astrocytes. Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their first attack. Unlike multiple sclerosis, a progressive clinical course is very unusual and the accrual of disability is related to relapses. Aggressive treatment of attacks and highly efficient maintenance therapies to prevent attacks are therefore crucial to prevent residual disability. In this article, we review how high dose steroids and most importantly apheresis and modern therapies implicating B cell depletion, inhibition of complement and IL-6 reception are effective to change its natural history. We will emphasize the results of three recent double blind randomized controlled studies using monoclonal antibodies allowing strong hope to modify natural history of NMOSD.
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Neuromielite Óptica , Anticorpos Monoclonais , Aquaporina 4 , Progressão da Doença , Humanos , RecidivaRESUMO
INTRODUCTION: While monophasic and relapsing forms of myelin oligodendrocyte glycoprotein antibody associated disorders (MOGAD) are increasingly diagnosed world-wide, consensus on management is yet to be developed. OBJECTIVE: To survey the current global clinical practice of clinicians treating MOGAD. METHOD: Neurologists worldwide with expertise in treating MOGAD participated in an online survey (February-April 2019). RESULTS: Fifty-two responses were received (response rate 60.5%) from 86 invited experts, comprising adult (78.8%, 41/52) and paediatric (21.2%, 11/52) neurologists in 22 countries. All treat acute attacks with high dose corticosteroids. If recovery is incomplete, 71.2% (37/52) proceed next to plasma exchange (PE). 45.5% (5/11) of paediatric neurologists use IV immunoglobulin (IVIg) in preference to PE. Following an acute attack, 55.8% (29/52) of respondents typically continue corticosteroids for ≥ 3 months; though less commonly when treating children. After an index event, 60% (31/51) usually start steroid-sparing maintenance therapy (MT); after ≥ 2 attacks 92.3% (48/52) would start MT. Repeat MOG antibody status is used by 52.9% (27/51) to help decide on MT initiation. Commonly used first line MTs in adults are azathioprine (30.8%, 16/52), mycophenolate mofetil (25.0%, 13/52) and rituximab (17.3%, 9/52). In children, IVIg is the preferred first line MT (54.5%; 6/11). Treatment response is monitored by MRI (53.8%; 28/52), optical coherence tomography (23.1%; 12/52) and MOG antibody titres (36.5%; 19/52). Regardless of monitoring results, 25.0% (13/52) would not stop MT. CONCLUSION: Current treatment of MOGAD is highly variable, indicating a need for consensus-based treatment guidelines, while awaiting definitive clinical trials.
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Autoanticorpos , Imunoglobulinas Intravenosas , Adulto , Criança , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Glicoproteína Mielina-Oligodendrócito , Plasmaferese , Inquéritos e QuestionáriosAssuntos
Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Neuromielite Óptica/diagnóstico , Diagnóstico Diferencial , Feminino , Gliose/diagnóstico por imagem , Gliose/patologia , Humanos , Imunossupressores/uso terapêutico , Imageamento por Ressonância Magnética , Pessoa de Meia-Idade , Neuroimagem/métodos , Neuromielite Óptica/tratamento farmacológico , Índias OcidentaisRESUMO
Isolated tumefactive demyelinating lesion (TDL) is a rare disease and a challenging entity especially for the differential diagnosis, biopsy indications, and therapeutic decisions. Long-term evolution is not well known. The objective of the study is to describe clinical and MRI characteristics and long-term follow-up of patients with isolated TDL. We performed a retrospective study including patients (1) with one TDL radiologically defined by a ≥20 mm FLAIR hyperintensity involving the white matter associated with T1 hypointensity that enhanced after gadolinium injection and (2) without any other MS lesion on the first MRI. Tumor, abscess, or other inflammatory diseases (ADEM, Baló's concentric sclerosis, systemic disease) were excluded. Sixteen patients (11 females/5 males) were included. The mean age of onset was 35.7 years (range 20-65). MRI disclosed supratentorial lesions with a mean size of 39.4 mm and usually mild edema/mass effect. Peripheral (mainly open-ring pattern) and central (mainly heterogeneous) enhancement were respectively seen in 9/16 and 11/16 patients. CSF study (n = 15) found oligoclonal bands (OCB) in seven. A cerebral biopsy was performed in 11 cases showing acute inflammatory demyelination. Thirteen patients were treated by pulse steroids with marked improvement in ten. At last clinical follow-up (mean 65.8 months, range 6-181), diagnosis was MS in 5 (31 %), isolated TDL in 10 (63 %) and one patient had a second TDL (6 %). Isolated tumefactive demyelinating lesions are a rare diagnostic entity. After a mean follow-up of 5 years, almost one-third became MS whereas most of the patients had no further event.
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Neoplasias Encefálicas/diagnóstico , Encéfalo/patologia , Doenças Desmielinizantes/diagnóstico , Adulto , Idoso , Neoplasias Encefálicas/complicações , Doenças Desmielinizantes/complicações , Avaliação da Deficiência , Feminino , Humanos , Estudos Longitudinais , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND AND PURPOSE: BIONAT is a French multicentric phase IV study of natalizumab (NTZ)-treated relapsing-remitting multiple sclerosis (MS) patients. The purpose of this study was to collect clinical, radiological and biological data on 1204 patients starting NTZ, and to evaluate the clinical/radiological response to NTZ after 2 years of treatment. METHODS: Patients starting NTZ at 18 French MS centres since June 2007 were included. Good response to NTZ was defined by the absence of clinical and radiological activity. Data analysed in this first report on the BIONAT study focus on patients who started NTZ at least 2 years ago (n = 793; BIONAT2Y ). RESULTS: NTZ was discontinued in 17.78% of BIONAT2Y. The proportion of patients without combined disease activity was 45.59% during the first two successive years of treatment. Systematic dosage of anti-NTZantibodies (Abs) detected only two supplementary patients with anti-NTZ Abs compared with strict application of recommendations. A significant decrease of IgG,M concentrations at 2 years of treatment was found. CONCLUSIONS: The efficacy of NTZ therapy on relapsing-remitting MS in a real life setting is confirmed in the BIONAT cohort. The next step will be the identification of biomarkers predicting response to NTZ therapy and adverse events.
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Anticorpos Monoclonais Humanizados/uso terapêutico , Esclerose Múltipla Recidivante-Remitente/tratamento farmacológico , Vigilância de Produtos Comercializados , Adulto , Estudos de Coortes , Feminino , Humanos , Masculino , Natalizumab , Estudos ProspectivosRESUMO
BACKGROUND: Neuromyelitis optica (NMO) is a severe autoimmune disease of the central nervous system characterized by spinal cord and optic nerve involvement. Brainstem manifestations have recently been described. OBJECTIVE: To evaluate the time of occurrence, the frequency and the characteristics of brainstem symptoms in a cohort of patients with NMO according to the ethnic background and the serologic status for anti-aquaporin-4 antibodies (AQP4-abs). METHODS: We performed a multicenter study of 258 patients with NMO according to the 2006 Wingerchuk criteria and we evaluated prospectively the frequency, the date of onset and the duration of various brainstem signs in this population. RESULTS: Brainstem signs were observed in 81 patients (31.4%). The most frequently observed signs were vomiting (33.1%), hiccups (22.3%), oculomotor dysfunction (19.8%), pruritus (12.4%), followed by hearing loss (2.5%), facial palsy (2.5%), vertigo or vestibular ataxia (1.7%), trigeminal neuralgia (2.5%) and other cranial nerve signs (3.3%). They were inaugural in 44 patients (54.3%). The prevalence was higher in the non-Caucasian population (36.6%) than in the Caucasian population (26%) (p<0.05) and was higher in AQP4-ab-seropositive patients (32.7%) than in seronegative patients (26%) (not significant). CONCLUSIONS: This study confirms the high frequency of brainstem symptoms in NMO with a majority of vomiting and hiccups. The prevalence of these manifestations was higher in the non Caucasian population.
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Tronco Encefálico/fisiopatologia , Soluço/fisiopatologia , Neuromielite Óptica/fisiopatologia , Vômito/fisiopatologia , Adulto , Aquaporina 4/imunologia , Autoanticorpos/sangue , Biomarcadores/sangue , Tronco Encefálico/diagnóstico por imagem , Tronco Encefálico/imunologia , Europa (Continente) , Feminino , Soluço/diagnóstico , Soluço/etnologia , Soluço/imunologia , Humanos , Japão , Imageamento por Ressonância Magnética , Masculino , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/etnologia , Neuromielite Óptica/imunologia , América do Norte , Prevalência , Fatores de Risco , Testes Sorológicos , Vômito/diagnóstico , Vômito/etnologia , Vômito/imunologiaRESUMO
Background. Multiple sclerosis (MS) patients of African ancestry have a more aggressive disease course than white patients and could be resistant to interferon-beta (INFB). Methods. We studied the impact of INFB in treatment-naive Afro-Caribbean (AC) with clinically definite MS using our European Database for Multiple Sclerosis (EDMUS) (2003-2010). Main outcome measures were annual relapse rate after 2 years of treatment, proportion of exacerbation-free subjects 48 weeks after initiating INFB, and time to first relapse. Results. 76 AC-MS (59F/17M) were identified. Annual relapse rate of 1.29 decreased to 0.83 (-35.6%) after 2 years of treatment. The proportion of relapse-free patients at 48 weeks was 46.2%. Median time to first relapse was 52 weeks. Conclusion. INFB is not strong enough to control AC-MS patients in many cases which is problematic in a population of worse MS prognosis.
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BACKGROUND: Despite similarities, neuromyelitis optica (NMO) can be distinguished from multiple sclerosis (MS) by clinical, radiological and serological findings. OBJECTIVE: This case-control study aimed to determine whether patients with NMO or with MS in an Afro-Caribbean population originating from French West Indies shared the same or different HLA class I and II pattern distribution. METHODS: The association with HLA class II (DRB1 and DQB1) alleles was tested in 42 NMO patients, 163 MS patients and 150 healthy controls. HLA-DRB1 and DQB1 typing was undertaken on genomic DNA extracted from peripheral blood leucocytes. RESULTS: By comparison with healthy controls, significantly increased frequency of HLA-DRB1 03 (26.2% vs. 13%, odds ratio 2.4, 95% confidence interval 1.31-4.28, p after correction, cp 0.045) was observed in patients with NMO. By contrast, in MS patients, HLA-DRB1 15 (24.8% vs. 13%, odds ratio 2.21, 95% CI 1.45-3.36, cp < 0.0015), but not DRB1 03 allele, was positively associated with the disease. Moreover, a modest protective effect of HLA-DRB1 11 in the MS group, independently of DRB1 15 association, was found (13.7% vs. 7% in controls, odds ratio 0.48, p 0.006), but did not survive Bonferroni correction. CONCLUSION: In conclusion, comparison of the HLA-DRB1 and DQB1 distribution in NMO and MS in this Afro-Caribbean population shows important differences in the HLA associations among NMO and MS.
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População Negra/genética , Antígenos HLA-DQ/genética , Antígenos HLA-DR/genética , Esclerose Múltipla/genética , Neuromielite Óptica/genética , Adulto , Estudos de Casos e Controles , Feminino , Frequência do Gene , Predisposição Genética para Doença , Guadalupe/epidemiologia , Cadeias beta de HLA-DQ , Cadeias HLA-DRB1 , Humanos , Masculino , Martinica/epidemiologia , Pessoa de Meia-Idade , Esclerose Múltipla/etnologia , Esclerose Múltipla/imunologia , Neuromielite Óptica/etnologia , Neuromielite Óptica/imunologia , Razão de Chances , Fenótipo , Medição de Risco , Fatores de Risco , Adulto JovemRESUMO
Few studies report a protective role of childhood solar exposure to multiple sclerosis. Our objective was to confirm the protective role of childhood solar exposure in multiple sclerosis in Cuba, Martinique and Sicily. This was a matched case- control study, and cases met Poser criteria for clinically, laboratory (definite, probable) multiple sclerosis. Controls were resident population, without neurological disorder, living close to cases (within 100 km), matched for sex, age (+/-5 years), residence before age 15. We recruited 551 subjects during a 1-year period (193 cases, Cuba n = 95, Sicily n = 50, Martinique n = 48; 358 controls). Some (89%) met definite clinical multiple sclerosis criteria (relapsing remitting form (with and without sequel) (74%), secondary progressive (21%), primary progressive (5%)). Odds ratios in a uni-variate analysis were: family history of multiple sclerosis (5.1) and autoimmune disorder (4.0); wearing shirt (3.5), hat (2.7), pants (2.4); sun exposure causing sunburn (1.8); sun exposure duration (1 h more/day; weekends 0.91, weekdays 0.86); bare-chested (0.6); water sports (0.2). Independent factors in the multivariate analysis were family history of multiple sclerosis (4.8 (1.50-15.10)), wearing pants under sunlight (1.9 (1.10-3.20)), sun exposure duration (1 h more/ day, weekdays 0.90 (0.85-0.98), weekends 0.93 (0.87-0.99)), water sports (0.23 (0.13-0.40)). We conclude that outdoor leisure activities in addition to sun exposure reports are associated with a reduced multiple sclerosis risk, with evidence of dose response.
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Esclerose Múltipla/epidemiologia , Esclerose Múltipla/prevenção & controle , Luz Solar , Adolescente , Adulto , Idoso , Estudos de Casos e Controles , Cuba/epidemiologia , Feminino , Humanos , Masculino , Martinica/epidemiologia , Pessoa de Meia-Idade , Sicília/epidemiologia , Raios Ultravioleta , Adulto JovemRESUMO
BACKGROUND: Relapsing neuromyelitis optica (RNMO) is an uncommon but devastating inflammatory disorder of the central nervous system. Long term history in a wide series of RNMO is required for better knowledge of the course of the disease and identification of patients at high risk of death. METHODS: Clinical features of patients with RNMO (88 women/eight men) obtained from the geographic Caribbean database (Cuba and French West Indies) were used to determine the progression of disability and to identify clinical predictors of death. RESULTS: Median age at onset of RNMO was 29.5 years (range 11-74). Median duration of disease was 9.5 years (1-40). Median relapse rate was 0.7 attack/patient/year (0.1-3). 66 patients experienced severe visual loss in at least one eye and 46 in both eyes. Median time from onset to unilateral and bilateral severe visual loss was 3 and 15 years, respectively. Median times to reach Kurtzke Disability Status Scale 3, 6 and 8 from onset of RNMO were 1, 8 and 22 years. There were 24 deaths (25%); within 5 years in 63% of cases. A higher attack frequency during the first year of disease (p = 0.009), blindness (p = 0.04) and sphincter signs at onset (p = 0.02) and lack of recovery of first attack (p = 0.003) were independently associated with a shorter time to death. CONCLUSION: RNMO is a very rapidly disabling disease affecting primarily young women. This study has identified clinical features that predict a poor outcome. These findings suggest that early and aggressive immunotherapy might be warranted in RNMO.
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Neuromielite Óptica/mortalidade , Neuromielite Óptica/patologia , Adolescente , Adulto , Fatores Etários , Idoso , Criança , Estudos de Coortes , Feminino , Nível de Saúde , Humanos , Masculino , Pessoa de Meia-Idade , Neuromielite Óptica/complicações , Recuperação de Função Fisiológica , Análise de Regressão , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Adulto JovemRESUMO
BACKGROUND: In Caucasian populations neuromyelitis optica (NMO-IgG) antibody has been detected in 27.1% / 78.2% of patients with relapsing-NMO (R-NMO). The prevalence reported for the disease in the Caribbean is 3.1/100,000 in the French West Indies (FWI) and 0.52 /100,000 in Cuba, but the NMO antibody status is unknown. OBJECTIVE: To assess the NMO-IgG antibody status of Cuban/FWI RNMO patients, comparing with European cases tested at the same laboratories. METHODS: Serum NMO-IgG antibodies were assayed in 48 R-NMO patients (Wingerchucks 1999 criteria): Cuba (24)/FWI (24), employing Lennon et als method. We compared the demographic, clinical, disability and laboratory data between NMO-IgG +/- patients. All the data were reviewed and collected blinded to the NMO-IgG status. RESULTS: Seropositivity of the NMO-IgG antibody demonstrated a lower rate in the Caribbean (33.3%), as compared with Caucasian patients from Spain/Italy (62.5%) and France (53.8%). Caribbean patients with NMO-IgG (+) displayed more attacks, more spinal attacks and a higher EDSS than NMO-IgG (-) cases, while brain and spinal cord MRI lesions were more frequent during remission, with more vertebral segments, more gray, white matter and holocord involvement. CONCLUSIONS: NMO IgG positive antibodies in NMO patients had a lower rate in the Caribbean area - where the population has a predominant African ancestry - than in Caucasian Europeans, suggesting the influence of a possible ethnic factor in the pathogenesis of the disease, but they confer a worse course with more attacks, more disability and MRI lesions.
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Autoanticorpos/sangue , População Negra , Imunoglobulina G/sangue , Neuromielite Óptica/etnologia , Neuromielite Óptica/imunologia , População Branca , Adulto , Encéfalo/patologia , Cuba/epidemiologia , Avaliação da Deficiência , Progressão da Doença , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Martinica/epidemiologia , Neuromielite Óptica/diagnóstico , Recidiva , Índice de Gravidade de Doença , Medula Espinal/patologia , Adulto JovemRESUMO
BACKGROUND: In 2005, the McDonald MRI criteria for dissemination in space were revised to improve diagnosis of multiple sclerosis (MS) in non-Caucasians. METHODS: We included patients with a first clinically isolated syndrome (CIS) to assess their performance in the Afro-Caribbean population. Baseline brain and spine MRI examinations were available within 3 months after onset of CIS. The development of a second clinical event was used as the main outcome indicating clinically definite MS. RESULTS: A total of 66 patients (52F/14M) were included between January 1998 and January 2008 (mean age: 34.7; median follow-up: 34 months). CIS was classified as spinal cord (30.3%), optic neuritis (28.8%), brainstem (24.2%), multiregional (10.6%), hemispheric (4.5%), or undetermined (1.5%). Overall conversion rate was 42.4% (median: 11 months). The McDonald criteria revised for dissemination in space were fulfilled in 33.3% (sensitivity: 0.39 (+/-0.18); specificity: 0.66 (+/-0.15), positive predictive value: 0.46 (+/-0.20), negative predictive value: 0.60 (+/-0.15). CONCLUSION: The Afro-Caribbean population is characterized by a strong proportion of CIS in the spinal cord and a lower burden of disease on the baseline brain MRI. This may explain the low sensitivity of the 2005 McDonald criteria for dissemination in space. Further prospective studies emphasizing MRI spinal cord features are needed to improve diagnostic criteria in a population of African descent.
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Encéfalo/patologia , Doenças Desmielinizantes/diagnóstico , Medula Espinal/patologia , Adolescente , Adulto , Idade de Início , População Negra/etnologia , Criança , Doenças Desmielinizantes/etnologia , Doenças Desmielinizantes/patologia , Diagnóstico Diferencial , Progressão da Doença , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Martinica/etnologia , Pessoa de Meia-Idade , Fibras Nervosas Mielinizadas/patologia , Estudos Prospectivos , Sensibilidade e EspecificidadeRESUMO
INTRODUCTION: Data on epidemiology of neuromyelitis optica (NMO) remained scarce in the last century, but the recent development of diagnostic criteria now enables inclusion of both monophasic and relapsing NMO in epidemiologic studies. Given the rarity of NMO, multicentric studies are needed to confirm a presumed higher frequency in women and in populations of black/Asian ancestry. The Caribbean basin is a suitable area for collecting a large NMO cohort and to assess the prevalence, incidence, and mortality of this disorder. PATIENTS AND METHODS: This population-based survey of the NMO spectrum in the French West Indies (FWI) and Cuba included 151 cases. RESULTS: Ninety-eight patients (female/male ratio: 9.8) had NMO. Age of onset in NMO patients was 30.9 years. Mean annual incidence of NMO in the French West Indies for the period July 2002 to June 2007 was 0.20/100,000 inhabitants (IC 95% 0.05-0.35). Incidence rates were steady in the FWI during the 1992 to 2007 period. Decreasing mortality in the FWI during the 1992 to 2007 period explained the increasing prevalence which was 4.20/100,000 inhabitants (IC 95% 3.7-5.7) in June 2007. The prevalence of NMO in Cuba on November302004 was 0.52/100,000 inhabitants. (IC 95% 0.39-0.67). Prevalence rates did not differ significantly by ethnic group in Cuba, however, black Cubans exhibited the highest prevalence. DISCUSSION: Epidemiologic studies on NMO in each population are needed to determine whether aggressive therapies can reduce the mortality of this devastating disorder. CONCLUSION: In the Caribbean basin, NMO involves almost exclusively young women; the epidemiologic data confirm its predilection for populations of African ancestry. In the FWI, recent and aggressive therapy has lowered mortality but with an increase in the prevalence of NMO.
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Neuromielite Óptica/epidemiologia , Adolescente , Adulto , Fatores Etários , Região do Caribe/epidemiologia , Cuba/epidemiologia , Etnicidade , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Martinica/epidemiologia , Pessoa de Meia-Idade , Neuromielite Óptica/mortalidade , Fatores Sexuais , Terminologia como Assunto , Adulto JovemRESUMO
BACKGROUND: Plasma exchange (PE) is increasingly undertaken in diseases involving humoral factors and is proven to be beneficial in acute demyelinating diseases. Spinal attacks in relapsing neuromyelitis optica (NMO) and in extensive transverse myelitis (ETM) - a truncated form of NMO with spinal involvement - are usually devastating. OBJECTIVE: We retrospectively studied the outcome of PE-treated versus steroid-only treated spinal attacks in relapsing NMO and ETM. METHODS: We included 96 severe spinal attacks in 43 Afro-Caribbean patients. PE was given as an add-on therapy in 29 attacks. Expanded disability status score (EDSS) was obtained before attack, during the acute and residual stage. We defined the DeltaEDSS as the rise from basal to residual EDSS. RESULTS: The DeltaEDSS was found to be lower in the PE-treated group (1.2 +/- 1.6 vs 2.6 +/- 2.3; P < 0.01). A low basal impairment is associated with a better outcome. Improvement was obtained in both NMO-IgG negative and positive NMO attacks. Minor adverse events manifested in seven PE sessions (24%). CONCLUSION: PE appears to be a safe add-on therapy that may be employed early in severe spinal attacks in the NMO spectrum disorders in order to maximize improvement rate. PE efficiency is independent of NMO-IgG positivity.