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1.
Mult Scler ; 30(3): 345-356, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38258822

RESUMO

BACKGROUND: Isolated first episodes of longitudinally extensive transverse myelitis (LETM) have typically been associated with neuromyelitis optica spectrum disorder (NMOSD) or myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). However, in some cases, serological testing and screening for other aetiologies are negative, a condition referred to as double seronegative longitudinally extensive transverse myelitis (dsLETM). OBJECTIVE: The objective of this study was to evaluate comparative outcomes of dsLETM, MOGAD-LETM and NMOSD-LETM. METHODS: Cohort study of LETM cases seen in the UK NMOSD Highly Specialised Service between January 2008 and March 2022. RESULTS: LETM = 87 cases were identified (median onset age = 46 years (15-85); median follow-up = 46 months (1-144); 47% NMOSD-LETM = 41 (aquaporin-4 antibodies (AQP4-IgG) positive = 36), 20% MOGAD-LETM = 17 and 33% dsLETM = 29). Despite similar Expanded Disability Status Scale (EDSS) at nadir, last EDSS was higher in AQP4-IgG and seronegative NMOSD-LETM (sNMOSD) (p = 0.006). Relapses were less common in dsLETM compared to AQP4-IgG NMOSD-LETM and sNMOSD-LETM (19% vs 60% vs 100%; p = 0.001). Poor prognosis could be predicted by AQP4-IgG (odds ratio (OR) = 38.86 (95% confidence interval (CI) = 1.36-1112.86); p = 0.03) and EDSS 3 months after onset (OR = 65.85 (95% CI = 3.65-1188.60); p = 0.005). CONCLUSION: dsLETM remains clinically challenging and difficult to classify with existing nosological terminology. Despite a similar EDSS at nadir, patients with dsLETM relapsed less and had a better long-term prognosis than NMOSD-LETM.


Assuntos
Mielite Transversa , Neuromielite Óptica , Humanos , Pessoa de Meia-Idade , Estudos de Coortes , Aquaporina 4 , Recidiva Local de Neoplasia/complicações , Prognóstico , Autoanticorpos , Imunoglobulina G , Glicoproteína Mielina-Oligodendrócito , Estudos Retrospectivos
2.
Brain ; 142(5): 1310-1323, 2019 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-30938427

RESUMO

Pathogenic antibodies targeting the aquaporin-4 water channel on astrocytes are associated with relapsing inflammatory neuromyelitis optica spectrum disorders. The clinical phenotype is characterized by recurrent episodes of optic neuritis, longitudinally extensive transverse myelitis, area postrema attacks and less common brainstem and cerebral events. Patients often develop major residual disability from these attacks, so early diagnosis and initiation of attackpreventing medications is important. Accurate prediction of relapse would assist physicians in counselling patients, planning treatment and designing clinical trials. We used a large multicentre dataset of 441 patients from the UK, USA, Japan and Martinique who collectively experienced 1976 attacks, and applied sophisticated mathematical modelling to predict likelihood of relapse and disability at different time points. We found that Japanese patients had a lower risk of subsequent attacks except for brainstem and cerebral events, with an overall relative relapse risk of 0.681 (P = 0.001) compared to Caucasians and African patients, who had a higher likelihood of cerebral attacks, with a relative relapse risk of 3.309 (P = 0.009) compared to Caucasians. Female patients had a higher chance of relapse than male patients (P = 0.009), and patients with younger age of onset were more likely to have optic neuritis relapses (P < 0.001). Immunosuppressant drugs reduced and multiple sclerosis disease-modifying agents increased the likelihood of relapse (P < 0.001). Patients with optic neuritis at onset were more likely to develop blindness (P < 0.001), and those with older age of onset were more likely to develop ambulatory disability. Only 25% of long-term disability was related to initial onset attack, indicating the importance of early attack prevention. With respect to selection of patients for clinical trial design, there would be no gain in power by selecting recent onset patients and only a small gain by selecting patients with recent high disease activity. We provide risk estimates of relapse and disability for patients diagnosed and treated with immunosuppressive treatments over the subsequent 2, 3, 5 and 10 years according to type of attack at onset or the first 2-year course, ethnicity, sex and onset age. This study supports significant effects of onset age, onset phenotype and ethnicity on neuromyelitis optica spectrum disorders outcomes. Our results suggest that powering clinical treatment trials based upon relapse activity in the preceding 2 years may offer little benefit in the way of attack risk yet severely hamper clinical trial success.


Assuntos
Aquaporina 4 , Imunoglobulina G , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/etnologia , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Estudos de Coortes , Bases de Dados Factuais/tendências , Etnicidade , Feminino , Seguimentos , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Neuromielite Óptica/tratamento farmacológico , Valor Preditivo dos Testes , Estudos Prospectivos , Estudos Retrospectivos , Adulto Jovem
3.
Brain ; 140(12): 3128-3138, 2017 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-29136091

RESUMO

See de Seze (doi:10.1093/brain/awx292) for a scientific commentary on this article. A condition associated with an autoantibody against MOG has been recently recognized as a new inflammatory disease of the central nervous system, but the disease course and disability outcomes are largely unknown. In this study we investigated clinical characteristics of MOG-antibody disease on a large cohort of patients from the UK. We obtained demographic and clinical data on 252 UK patients positive for serum immunoglobulin G1 MOG antibodies as tested by the Autoimmune Neurology Group in Oxford. Disability outcomes and disease course were analysed in more detail in a cohort followed in the Neuromyelitis Optica Oxford Service (n = 75), and this included an incident cohort who were diagnosed at disease onset (n = 44). MOG-antibody disease affects females (57%) slightly more often than males, shows no ethnic bias and typically presents with isolated optic neuritis (55%, bilateral in almost half), transverse myelitis (18%) or acute disseminated encephalomyelitis-like presentations (18%). In the total Oxford cohort after a median disease duration of 28 months, 47% of patients were left with permanent disability in at least one of the following: 16% patients had visual acuity ≤6/36 in at least one eye, mobility was limited in 7% (i.e. Expanded Disability Status Scale ≥ 4.0), 5% had Expanded Disability Status Scale ≥ 6.0, 28% had permanent bladder issues, 20% had bowel dysfunction, and 21% of males had erectile dysfunction. Transverse myelitis at onset was a significant predictor of long-term disability. In the incident cohort 36% relapsed after median disease duration of 16 months. The annualized relapse rate was 0.2. Immunosuppression longer than 3 months following the onset attack was associated with a lower risk of a second relapse. MOG-antibody disease has a moderate relapse risk, which might be mitigated by medium term immunosuppression at onset. Permanent disability occurs in about half of patients and more often involves sphincter and erectile functions than vision or mobility.


Assuntos
Autoanticorpos/sangue , Glicoproteína Mielina-Oligodendrócito/sangue , Neuromielite Óptica/sangue , Neuromielite Óptica/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Estudos de Coortes , Pessoas com Deficiência , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Neuromielite Óptica/diagnóstico , Prognóstico , Reino Unido/epidemiologia , Adulto Jovem
4.
J Neurol Neurosurg Psychiatry ; 88(2): 165-169, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27884934

RESUMO

IMPORTANCE: Chronic, intractable neuropathic pain is a common and debilitating consequence of neuromyelitis optica spectrum disorder (NMOSD) myelitis, with no satisfactory treatment; few studies have yet to explore its aetiology. OBJECTIVE: To establish if myelitis-associated chronic pain in NMOSD is related to the craniocaudal location of spinal cord lesions. METHOD: (1) Retrospective cohort of 76 aquaporin 4-antibody (AQP4-Ab)-positive patients from Oxford and Liverpool's national NMOSD clinics, assessing current pain and craniocaudal location of cord lesion contemporary to pain onset. (2) Focused prospective study of 26 AQP4-Ab-positive Oxford patients, a subset of the retrospective cohort, assessing current craniocaudal lesion location and current pain. RESULTS: Patients with isolated thoracic cord myelitis at the time of pain onset were significantly more disabled and suffered more pain. Cervical and thoracic lesions that persisted from pain onset to 'out of relapse' follow-up (current MRI) had highly significant (p<0.01) opposing effects on pain scores (std. ß=-0.46 and 0.48, respectively). Lesion length, total lesion burden and number of transverse myelitis relapses did not correlate with pain. CONCLUSIONS: Persistent, caudally located (ie, thoracic) cord lesions in AQP4-Ab-positive patients associate with high postmyelitis chronic pain scores, irrespective of number of myelitis relapses, lesion length and lesion burden. Although disability correlated with pain in isolation, it became an insignificant predictor of pain when analysed alongside craniocaudal location of lesions.


Assuntos
Aquaporina 4/imunologia , Dor Crônica/diagnóstico por imagem , Neuralgia/diagnóstico por imagem , Neuromielite Óptica/diagnóstico por imagem , Medula Espinal/diagnóstico por imagem , Adulto , Idoso , Autoanticorpos , Dor Crônica/etiologia , Dor Crônica/imunologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuralgia/etiologia , Neuralgia/imunologia , Neuromielite Óptica/complicações , Neuromielite Óptica/imunologia , Medição da Dor , Estudos Retrospectivos , Índice de Gravidade de Doença
6.
Mult Scler ; 23(2): 228-233, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27553618

RESUMO

BACKGROUND: The international panel for neuromyelitis optica (NMO) diagnosis has proposed diagnostic criteria for neuromyelitis optica spectrum disorders (NMOSD). OBJECTIVES: We assessed the impact of these criteria on diagnostic rates in a large cohort of patients. METHODS: We identified and applied the 2006 and 2015 criteria to all patients ( n = 176) seen in the NMO and non-multiple sclerosis central nervous system demyelination clinic (part of the UK NMO service) from January 2013 to May 2015. RESULTS: The 2006 criteria classified 63 of 176 (36%) patients as NMO. A total of 42 patients (67%) were aquaporin 4 (AQP4) immunoglobulin G (IgG) +ve and 21 (33%) AQP4 IgG -ve. The 2015 criteria classified 111 of 176 (63%) patients as NMOSD, of which 81 (73%) were AQP4 IgG +ve and 30 (27%) were AQP4 IgG -ve. There was an increase of 48 patients (76%) diagnosed as NMOSD using the new criteria. CONCLUSION: Application of the 2015 criteria led to a rise in diagnosis of NMOSD by 76%. The rise in the AQP4 IgG +ve group contributed 62% and the seronegative group contributed 14%.


Assuntos
Autoanticorpos/metabolismo , Neuromielite Óptica/diagnóstico , Aquaporina 4/metabolismo , Sistema Nervoso Central/imunologia , Feminino , Humanos , Imunoglobulina G/metabolismo , Masculino
8.
Pract Neurol ; 15(6): 463-5, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26245509

RESUMO

Neuromyelitis optica typically presents at a median age of 40-50 years. The myelitis is usually of acute onset, long (>3 vertebral segments) and causes severe sensorimotor and bladder and bowel disturbances. We describe a 73-year-old Caucasian woman with aquaporin-4 antibody-positive neuromyelitis optica whose index event was intermittent paroxysmal tonic spasms (and no other myelitis features) that recurred for 6 months and was associated with a short spinal cord lesion on MRI. This case reiterates recent observations that neuromyelitis optica can occur in older persons, and its myelitis can be 'short' and clinically mild.


Assuntos
Mielite/complicações , Espasmo/complicações , Idoso , Feminino , Humanos , Imageamento por Ressonância Magnética , Medula Espinal/patologia
9.
Artigo em Inglês | MEDLINE | ID: mdl-37977848

RESUMO

BACKGROUND AND OBJECTIVES: Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a recently identified autoimmune demyelinating disorder of the CNS affecting both adults and children. Diagnostic criteria for MOGAD have recently been published. We aimed to validate the 2023 MOGAD diagnostic criteria in a real-world cohort of patients with atypical CNS inflammation. METHODS: All patients referred to the National neuromyelitis optica spectrum disorder (NMOSD) specialized service at The Walton Center NHS Foundation Trust between 2012 and 2023 with an atypical demyelinating syndrome were evaluated. We systematically applied the 2023 MOGAD diagnostic criteria and previous 2018 International Diagnostic Recommendations for MOG encephalomyelitis to our retrospective cohort. RESULTS: 474 patients were screened and 66 were excluded for lack of clinical information. Preexisting diagnoses within our cohort included the following: MOGAD, n = 127; AQP4-IgG NMOSD, n = 125; seronegative NMOSD, n = 33; multiple sclerosis (MS), n = 10; and other diagnoses, n = 113. Of patients with preexisting MOGAD, 97% (123/127) fulfilled the 2023 MOGAD diagnostic criteria. Three patients with a low-positive MOG-IgG did not meet supportive features though 2/3 had insufficient investigations. Alternative diagnoses could not be excluded in 1 patient with MS-MOGAD overlap. No patients with a non-MOGAD diagnosis were found to fulfill the 2023 diagnostic criteria. The sensitivity and specificity of the 2023 MOGAD diagnostic criteria were 97% and 100% with no false positives, improving on 2018 International Diagnostic Recommendations for MOG encephalomyelitis. Low-positive MOG-IgG results were more often associated with a longer time from disease onset to sampling (p < 0.001). In addition, in patients with a MOG-IgG1 test within 6 months of clinical onset, approximately 25% can become low positive by 6 months. Of patients with preexisting MOGAD, 9% (12/127) had insufficient investigations and examinations to fully evaluate additional supportive features. However, in those who were completely evaluated, supportive features were fulfilled in 97% (111/115). DISCUSSION: The 2023 MOGAD diagnostic criteria were highly sensitive and specific and closely align with historically established cases of MOGAD. However, because additional supportive features are stipulated for patients with a low-positive MOG-IgG result, missed diagnoses may occur due to delayed testing or insufficient investigations.


Assuntos
Encefalomielite , Esclerose Múltipla , Neuromielite Óptica , Adulto , Criança , Humanos , Glicoproteína Mielina-Oligodendrócito , Estudos Retrospectivos , Autoanticorpos , Neuromielite Óptica/diagnóstico , Esclerose Múltipla/diagnóstico , Imunoglobulina G
10.
Mult Scler Relat Disord ; 83: 105452, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38277981

RESUMO

BACKGROUND: Relapse rates of 47 % have been reported in patients with neuromyelitis optica (NMOSD) using Azathioprine (AZA) and mycophenolate mofetil (MMF). Prediction of non-responders could help determine which patients are most likely to benefit from newer monoclonal antibody treatments from the outset. OBJECTIVES: To identify predictors of AZA and MMF treatment response in NMOSD. METHODS: Multicenter cohort study of NMOSD patients from Brazil and the United Kingdom, treated with AZA and MMF. An unsatisfactory response was defined as one severe or two non-severe attacks in a year. Cox regression was used to identify predictive factors of unsatisfactory response to AZA and MMF. RESULTS: 103 NMOSD patients, mean age 38 years, 83% female, and 65% of Black ethnic group were included. An unsatisfactory IS response was observed in 42% of patients over 2.5 years (IQR 1.0-8.8) years. A severe preceding attack was more common in non-responders (31.1% x 76.7%, p = <0.001). In multivariable analysis, severe attack (RR 3.13; 95 % CI 1.37-7.18, p = 0.007) or higher annualized relapse rate (RR 4.84; 95 % CI 2.01-11.65, p = < 0.001) predicted an unsatisfactory response. Interestingly, Black NMOSD patients had a lower risk of poor response (RR 0.39, 95 % CI 0.17-0.85, p = 0.019). CONCLUSION: Severe attack and a higher annualized relapse rate before AZA or MMF initiation were associated with an unsatisfactory IS response. In patients with these characteristics, treatment with higher-efficacy drugs should be considered from the outset.


Assuntos
Azatioprina , Neuromielite Óptica , Humanos , Feminino , Adulto , Masculino , Azatioprina/uso terapêutico , Ácido Micofenólico/uso terapêutico , Imunossupressores/uso terapêutico , Estudos de Coortes , Resultado do Tratamento , Recidiva
11.
Mult Scler Relat Disord ; 79: 105035, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37864992

RESUMO

BACKGROUND: CSF-specific oligoclonal bands (CSF-OCBs) can be used for dissemination in time (DIT) in the 2017 multiple sclerosis (MS) diagnostic criteria. A cut-off of ≥2 CSF-OCBs was recommended but studies have suggested ≥3 CSF-OCBs may be superior. OBJECTIVES: To assess utility of ≥2 and ≥3 CSF-OCBs as a cut-off for MS diagnosis. METHODS: Paired serum and CSF-OCBs sent to the Walton Centre, UK between July 2018 and June 2020 were included. CSF-OCBs were assessed using isoelectric focussing and reviewed by two blinded raters. Case records were reviewed. RESULTS: Of 1334 paired serum and CSF-OCB requests, 945 cases had sufficient clinical information. More than 1 CSF-OCB was detected in 268/945(28%) cases. Of these, 252 had ≥2 and 230 had ≥3 CSF-OCBs. The sensitivity and specificity for MS with ≥2 and ≥3 CSF-OCBs were 91.7%, 91.2%, 90.2% and 93.8% respectively. Only 3/22 patients with 2 CSF-OCBs had MS. In 25% of patients, CSF-OCBs reduced time to MS diagnosis (median 437.5 days (28-1332)). CONCLUSION: Although cut-offs of ≥2 or ≥3 CSF-OCBs performed similarly well, 2 CSF-OCBs were frequently seen with non-inflammatory pathology. Use of ≥3 CSF-OCBs for MS diagnosis should be considered. CSF analysis reduced time to MS diagnosis by approximately 14 months.


Assuntos
Esclerose Múltipla , Humanos , Esclerose Múltipla/diagnóstico , Bandas Oligoclonais , Focalização Isoelétrica , Sensibilidade e Especificidade , Imunoglobulina G
12.
JAMA Neurol ; 75(1): 65-71, 2018 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-29131884

RESUMO

Importance: Antibodies to myelin oligodendrocyte glycoprotein IgG (MOG-IgG) are increasingly detected in patients with non-multiple sclerosis-related demyelination, some of whom manifest a neuromyelitis optica (NMO) phenotype. Cortical involvement, encephalopathy, and seizures are rare in aquaporin 4 antibody (AQP4-IgG)-related NMO in the white European population. However, the authors encountered several patients with seizures associated with MOG-IgG disease. Objective: To compare incidence of seizures and encephalitis-like presentation, or both between AQP4-IgG-positive and MOG-IgG-positive patients. Design, Setting, and Participants: Retrospective case series of all patients who were seropositive for MOG-IgG (n = 34) and the last 100 patients with AQP4-IgG disease (NMO spectrum disorder) seen in the NMO service between January 2013 and December 2016, and analysis was completed January 4, 2017. All patients were seen in a tertiary neurological center, The Walton Centre NHS Foundation Trust in Liverpool, England. Main Outcomes and Measures: The difference in seizure frequency between the AQP4-IgG-positive and MOG-IgG-positive patient groups was determined. Results: Thirty-four patients with MOG-IgG disease (20 female) with a median age at analysis of 30.5 years (interquartile range [IQR], 15-69 years), and 100 AQP4-IgG-positive patients (86 female) with a median age at analysis of 54 years (IQR, 12-91 years) were studied. Most patients were of white race. Five of the 34 patients with MOG-IgG (14.7%) had seizures compared with 1 patient with AQP4-IgG (2-sided P < .008, Fisher test). On magnetic resonance imaging, all 5 MOG-IgG-positive patients had inflammatory cortical brain lesions associated with the seizures. In 3 of the 5 MOG-IgG-positive patients, seizures occurred as part of the index event. Four of the 5 presented with encephalopathy and seizures, and disease relapsed in all 5 patients. Four of these patients were receiving immunosuppressant medication at last follow-up, and 3 continued to take antiepileptic medication. In contrast, the only AQP4-IgG-positive patient with seizures had a diagnosis of complex partial epilepsy preceding the onset of NMO by several years and experienced no encephalitic illness; her magnetic resonance imaging results demonstrated no cortical, subcortical, or basal ganglia involvement. Conclusions and Relevance: Patients with MOG-IgG-associated disease were more likely to have seizures and encephalitis-like presentation than patients with AQP4-IgG-associated disease.


Assuntos
Aquaporina 4/imunologia , Encefalite/sangue , Imunoglobulina G/sangue , Glicoproteína Mielina-Oligodendrócito/imunologia , Convulsões/sangue , Adolescente , Adulto , Idoso , Europa (Continente) , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
13.
Disabil Rehabil ; 39(13): 1279-1286, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-27374494

RESUMO

AIM: Neuromyelitis optica (NMO) is a rare neuro-inflammatory condition characterized by acute relapses causing severe visual or physical disability. The impact on family members and their experiences have not been studied. The study aims were to explore the lived experience of partners of people with NMO and to investigate potential carer burden in this population. METHOD: A mixed-method design was used; 11 partners of people with NMO completed semi-structured interviews; 54 partners completed Zarit Burden Interview and Hospital Anxiety and Depression Scale. RESULTS: Three qualitative themes influenced partners' quality of life (QoL): role/relationship; it's all about them; and the impact of NMO. Life changed dramatically for participants after the first NMO attack, necessitating responsibility for physical, financial, social, and emotional support. As NMO symptoms improved and stabilized, freedom and QoL for spouses also improved, albeit with on-going worries regarding the impact of potential devastating future relapses. Quantitative findings showed mild/moderate carer burden (46%), mild/moderate anxiety (59%), and mild/moderate depression (24%). No partner indicated severe carer burden, anxiety, or depression. CONCLUSION: Participants regarded themselves as partners rather than carers whom require assessment and support for their emotional and health well-being. Health-care professionals need to acknowledge the important role partners play in the dynamics of the family unit, through greater discussion and inclusion. Implications for Rehabilitation NMO has a strong impact on couples, resulting in both physical caregiving needs and anxiety regarding the unpredictability of potential devastating relapses. Partners do not necessarily experience clinically significant "burden", anxiety or depression, and tools which screen for this may not capture the nature of their experiences. Health-care professionals need to acknowledge, consult, and respect the experience of partners during assessment and implementation of action plans. Partners should be individually assessed based upon the physical and emotional dependency created by NMO to improve their health and well-being.


Assuntos
Ansiedade/epidemiologia , Cuidadores/psicologia , Depressão/epidemiologia , Neuromielite Óptica/reabilitação , Cônjuges/psicologia , Adaptação Psicológica , Adulto , Idoso , Emoções , Feminino , Humanos , Entrevistas como Assunto , Masculino , Pessoa de Meia-Idade , Escalas de Graduação Psiquiátrica , Qualidade de Vida/psicologia , Recidiva , Índice de Gravidade de Doença , Reino Unido
14.
J Neurol ; 264(11): 2330-2344, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28289845

RESUMO

The evolution of neuromyelitis optica spectrum disorder (NMOSD) from a rare, incurable and misunderstood disease with almost universally poor outcomes to its present state in just over a decade is unprecedented in neurology and possibly in medicine. Our knowledge of NMOSD biology has led to the recognition of wider phenotypes, new disease mechanisms, and thus clinical trials of new and effective treatments. This article aims to update readers on the recent developments in NMOSD with particular emphasis on clinical advances, the 2015 diagnostic criteria, biomarkers, imaging, and therapeutic interventions.


Assuntos
Neurologistas/psicologia , Neuromielite Óptica , Aquaporina 4/imunologia , Citocinas/metabolismo , Proteína Glial Fibrilar Ácida/metabolismo , Humanos , Imunoglobulina G/sangue , Fatores Imunológicos/uso terapêutico , Glicoproteína Mielina-Oligodendrócito/imunologia , Neuroimagem , Neuromielite Óptica/sangue , Neuromielite Óptica/diagnóstico , Neuromielite Óptica/tratamento farmacológico
15.
J Neurol ; 264(10): 2088-2094, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28840314

RESUMO

Antibodies to myelin oligodendrocyte glycoprotein (MOG-IgG) have been described in patients with neuromyelitis optica spectrum disorders (NMOSD) without aquaporin-4 antibodies (AQP4-IgG). We aimed to identify the proportion of AQP4-IgG-negative NMOSD patients who are seropositive for MOG-IgG. In a cross sectional study, we reviewed all patients seen in the National NMO clinic over the last 4 years (after the availability of MOG-IgG testing), including clinical information, MRI, and antibody tests. 261 unique patients were identified. 132 cases satisfied the 2015 NMOSD diagnostic criteria. Of these, 96 (73%) were AQP4-IgG positive and 36 (27%) were AQP4-IgG negative. These 36 patients were tested for MOG-IgG and 15/36 (42%) tested positive. 20% (25/125) of the patients who did not satisfy NMOSD criteria had MOG-IgG. Approximately half of seronegative NMOSD is MOG-Ig seropositive and one in five of non-NMOSD/non-MS demyelination is MOG-IgG positive. Since MOG-associated demyelinating disease is likely different from AQP4-IgG disease in terms of underlying disease mechanisms, relapse risk and possibly treatment, testing for MOG-IgG in patients with AQP4-IgG-negative NMOSD and other non-MS demyelination may have significant implications to management and clinical trials.


Assuntos
Aquaporina 4/imunologia , Imunoglobulina G/sangue , Glicoproteína Mielina-Oligodendrócito/imunologia , Neuromielite Óptica/sangue , Neuromielite Óptica/epidemiologia , Neuromielite Óptica/imunologia , Adolescente , Adulto , Estudos Transversais , Doenças Desmielinizantes/sangue , Doenças Desmielinizantes/imunologia , Feminino , Humanos , Imunossupressores/uso terapêutico , Masculino , Pessoa de Meia-Idade , Neuromielite Óptica/tratamento farmacológico , Adulto Jovem
16.
J Neurol Sci ; 360: 4-9, 2016 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-26723962

RESUMO

OBJECTIVE: Our primary objective was to examine the neuropsychological and psychopathological profile of patients with neuromyelitis optica (NMO) and compare these to multiple sclerosis (MS) and healthy control (HC) groups. We also examined for relationships between cognitive and psychiatric variables and clinical factors including accumulated neurological disability and disease duration. METHODS: A neuropsychological test battery was administered along with a structured psychiatric interview and quantitative measures of mood symptoms. RESULTS: 42 NMO, 42 MS and 42 HC participants were assessed. Cognitive impairments were observed in 67% of NMO patients. The prevalence and profile of cognitive impairments and lifetime prevalence of depression was similar between NMO and MS groups. However, significantly higher rates of recurrent depression and suicidality were observed in NMO patients. Correlational analyses revealed higher levels of anxiety symptoms were associated with shorter disease duration in NMO, while higher depression symptom levels were associated with higher neurological disability and poorer cognition. CONCLUSIONS: Our results demonstrate substantial cognitive and psychiatric comorbidities in NMO patients. Similar rates of lifetime and current depression between NMO and MS appear to mask greater underlying psychiatric burden in NMO and further understandings of the course of neurobehavioural comorbidities is required to better comprehend the additional morbidity in NMO. Our data support a role for cognitive and psychiatric assessments in the comprehensive care of NMO patients.


Assuntos
Ansiedade/complicações , Transtornos Cognitivos/complicações , Depressão/complicações , Neuromielite Óptica/complicações , Adulto , Ansiedade/psicologia , Atenção/fisiologia , Transtornos Cognitivos/psicologia , Depressão/psicologia , Função Executiva/fisiologia , Feminino , Humanos , Masculino , Memória/fisiologia , Pessoa de Meia-Idade , Esclerose Múltipla/complicações , Esclerose Múltipla/psicologia , Neuromielite Óptica/psicologia , Testes Neuropsicológicos
17.
Mult Scler Relat Disord ; 4(6): 614-8, 2015 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-26590671

RESUMO

BACKGROUND: Transverse myelitis (TM) associated with Neuromyelitis Optica (NMO) can be severe and is well known to reduce mobility early in the disease. However the burden of bladder and bowel dysfunction is unknown and overlooked. We studied the frequency of bladder and bowel dysfunction and their impact on quality of life. METHODS: A cross-sectional study of 60 patients who had AQP4-IgG positive NMO associated TM was performed using the Bladder Control Scale, Lower Urinary Tract Quality of Life, Bowel Control Scale and Neurogenic Bowel Score, Short-Form-36 Health Survey and EDSS. The relationships between the variables were analysed with multiple linear regression. RESULTS: Fifty women and 10 men participated. 78% (47/60) patients reported bladder symptoms and a similar number reported bowel problems. 87% (52/60) patients reported either bladder or bowel dysfunction. 65% (39/60) developed residual symptoms after the first episode of myelitis and the remaining by the second episode. Both bladder and bowel dysfunction reduced quality of life and required modification of lifestyle in 83% (39/47) and 70% (33/47) respectively. CONCLUSION: Bladder and bowel dysfunction is very common in NMO associated myelitis developing early in the disease and significantly affects quality of life.


Assuntos
Intestino Neurogênico/psicologia , Neuromielite Óptica/fisiopatologia , Neuromielite Óptica/psicologia , Qualidade de Vida , Doenças da Bexiga Urinária/epidemiologia , Transtornos Urinários/psicologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Aquaporina 4/imunologia , Autoanticorpos/sangue , Estudos Transversais , Avaliação da Deficiência , Feminino , Humanos , Imunoglobulina G/sangue , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Intestino Neurogênico/epidemiologia , Intestino Neurogênico/fisiopatologia , Neuromielite Óptica/epidemiologia , Índice de Gravidade de Doença , Reino Unido/epidemiologia , Doenças da Bexiga Urinária/fisiopatologia , Doenças da Bexiga Urinária/psicologia , Transtornos Urinários/epidemiologia , Transtornos Urinários/fisiopatologia , Adulto Jovem
18.
J Am Geriatr Soc ; 62(8): 1460-6, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25041242

RESUMO

OBJECTIVES: To describe the nature of geriatric medical admissions to teaching hospitals in three countries in Africa (Nigeria, Sudan, Tanzania) and compare them with data from the United Kingdom. DESIGN: Cross-sectional cohort study. SETTING: Federal Medical Centre, Idi-Aba, Abeokuta, Nigeria; Soba University Hospital, Khartoum, Sudan; Kilimanjaro Christian Medical Centre, Moshi, Tanzania; and North Tyneside General Hospital, North Shields, United Kingdom. PARTICIPANTS: All people aged 60 and older urgently medically admitted from March 1 to August 31, 2012. MEASUREMENTS: Data were collected regarding age, sex, date of admission, length of stay, diagnoses, medication, date of discharge or death, and discharge destination. RESULTS: In Africa, noncommunicable diseases (NCDs) accounted for 81.0% (n=708) of admissions (n=874), and tuberculosis, malaria, and the human immunodeficiency virus and acquired immunodeficiency syndrome accounted for 4.6% (n=40). Cerebrovascular accident (n=224, 25.6%) was the most common reason for admission, followed by cardiac or circulatory dysfunction (n=150, 17.2%). Rates of hypertension were remarkably similar in the United Kingdom (45.8%) and Africa (40.2%). CONCLUSIONS: In the elderly population, the predicted increased burden of NCDs on health services in Africa appears to have occurred. Greater awareness and some reallocation of resources toward NCDs may be required if the burden of such diseases is to be reduced.


Assuntos
Hospitalização/estatística & dados numéricos , Idoso , Idoso de 80 Anos ou mais , Estudos Transversais , Feminino , Hospitais de Ensino/estatística & dados numéricos , Humanos , Masculino , Pessoa de Meia-Idade , Nigéria/epidemiologia , Admissão do Paciente/estatística & dados numéricos , Estudos Prospectivos , Fatores de Risco , Sudão/epidemiologia , Tanzânia/epidemiologia , Reino Unido/epidemiologia
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