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

Abstract

Neuromyelitis optica (NMO) is an inflammatory autoimmune disease that affects the central nervous system (CNS) associated to significant neurologic disability 1,2 . For many decades, NMO was considered a variant of multiple sclerosis (MS), characterized by recurrent bilateral optic neuritis and transverse myelitis occurring concomitantly or apart in time, and without evidence of disease outside the optic nerves and the spinal cord 3 . Nowadays, studies ABSTRACTAlthough neuromyelitis optica (NMO) is known to be a more severe disease than relapsing-remitting multiple sclerosis (RRMS), few studies comparing both conditions in a single center have been done. Methods: Comparison of our previously published cohort of 41 NMO patients with 177 RRMS patients followed in the same center, from 1994 to 2007. Results: Mean age of onset was 32.6 for NMO and 30.2 for RRMS (p=0.2062) with mean disease duration of 7.4 years for NMO and 10.3 years for RRMS. Patients with NMO had a higher annualized relapse rate (1.0 versus 0.8, p=0.0013) and progression index (0.9 versus 0.6, p<0.0001), with more patients reaching expanded disability status scale (EDSS) 6.0 (39 versus 17%, p=0.0036). The odds ratio for reaching EDSS 6.0 and being deceased due to NMO in comparison to RRMS were, respectively, 3.14 and 12.15. Conclusion: Patients with NMO have a more severe disease than patients with RRMS, including higher risk of dying of a demyelinating disease.Key words: neuromyelitis optica, multiple sclerosis, demyelinating diseases, risk factors. RESUMOEmbora a neuromielite óptica (NMO) seja reconhecida como mais grave que a esclerose múltipla remitente recorrente (EMRR), existem poucos estudos comparando as duas doenças em um único centro. Métodos: Comparação de nossa coorte publicada de 41 pacientes com NMO com 177 pacientes com EMRR seguidos no mesmo centro, de 1994 a 2007. Resultados: A média de idade inicial foi de 32,6 anos em NMO e 30,2 anos em EMRR (p=0,2062), com tempo médio de doença de 7,4 anos para NMO e 10,3 anos EMRR. Pacientes com NMO apresentaram maior taxa anualizada de surtos (1,0 versus 0,8, p=0,0013) e índice de progressão (0,9 versus 0,6, p<0,0001), com mais pacientes atingindo EDSS 6,0 (39 versus 17%, p=0,0036). Os riscos relativos de se alcançar 6,0 EDSS e falecer em decorrência de NMO em comparação com EMRR, foram, respectivamente, 3,14 e 12,15. Conclusão: Pacientes com NMO têm uma doença mais grave do que os pacientes com EMRR, incluindo maior risco de morrer de uma doença desmielinizante.Palavras-Chave: neuromielite óptica, esclerose múltipla, doenças desmielinizantes, fatores de risco.indicate that NMO is a distinct disease from MS 4 , with nearly 90% of patients presenting a relapsing remitting course and half of them exhibiting brain lesions on magnetic resonance imaging 2,5 . Although NMO is frequently cited to have a more severe clinical course than relapsing-remitting MS (RRMS), few direct head-to-head studies involving both diseases in a single center scenario have been done 6,7 . We recently published

Multiple Sclerosis Mortality by Race/Ethnicity, Age, Sex, and Time Period in the United States, 1999–2015

“…Differences in comorbidities and MS disease progression in this group have been speculated to explain these observations. In addition, disparities in mortality rates between racial and ethnic groups should raise questions about particular characteristics in these groups, including MS phenotypes and even alternate diagnosis that can lead to higher morbidity and mortality like neuromyelitis optica (NMO) spectrum disorders, for example [30]. Flanagan et al [31] recently reported a disproportionately higher prevalence of NMO/NMOSD affecting blacks compared to whites, introducing the possibility of misdiagnosing MS in this population.…”

The profile of patients followed at the Neuroimmunology Clinic at UNIFESP: 20 years analysis

“…Since a complete computerized system for all outpatient activity was only implemented on the second semester of 2013, the clinical activity information was evaluated from August 1 st 2013 to December 31 st 2013 (5 months). The intention of this report is to describe the outpatient clinic population, therefore we did not include appointments made at the emergency department nor clinical, prognostic and treatment response information, available in previous studies from our group 5,12,24,25,26,27 .…”

“…This is supported by studies that disclosed specific genetics risk factor for NMO in Brazil 13,14,15 . Additionally, in North America and European countries MS is known to be the main cause of disability and retirement in people under 50 years old 16 and, although this information is not clearly available for Brazil, national studies disclose that patients with NMO have a more severe disease than patients with RRMS, including higher risk of becoming wheelchair bound and dying of a demyelinating disease 12 , which reinforces the need of information on these disease, as it may have social and economic impact. It is important to state that both diseases are treatable and that neurologic disability can be avoided or postponed with specific medications, some of them available from the Sistema Único de Saúde (SUS), the Brazilian Public Health Care System 17 .…”

Why this is not multiple sclerosis: a case based approach

“…Patients with a clear evidence of exclusively vascular disease of the CNS (i.e., with more than 50 years old and classic vascular risk factors), patients that presented with predominant peripheral nervous system symptoms, psychological or psychiatric disease and metabolic or hormonal disorders were excluded from this study due to an obvious alternative diagnosis, thus not representing a diagnostic challenge. Patients with a final diagnosis of neuromyelitis optica were also excluded from this analysis, as they have a very distinct presentation to MS 15,16 . We reviewed each patient's clinical chart, MRI of the brain and spinal cord and cerebrospinal fluid (CSF) analysis to conduct a descriptive study of the signs and symptoms that led to an initial diagnosis of MS and further present each case's red flags that signaled the alternative diagnosis, based on previously published consensus statements 11,12,13 .…”