Clinico-epidemiologic characteristics of optic neuritis in a tertiary eye centre in Eastern India based on the status of serum aquaporin-4 antibody

Purpose: To elucidate the clinico?epidemiologic characteristics of optic neuritis based on the status of serum aquaporin?4 antibody (AQP4?Ab) in patients with optic neuritis (ON). Methods: Medical records of 106 patients with ON and a follow?up of 3 years were reviewed. For each patient, the following data were extracted: medical history, findings of the ocular examination, brain, orbital or spinal MRI, and serological tests for AQP4. The ON was classified as typical or atypical based on disc examination and improvement in vision after intravenous methylprednisolone (IVMP). The clinical findings (typical or atypical), disease course, and outcomes were analyzed according to the serostatus of the ON. Results: 10 patients ((9.4%) were seropositive for AQP4?Ab; all had atypical ON. 96 patients (91%) were seronegative for AQP4?Ab: 36 atypical ON and 60 typical ON. Profound visual impairment at presentation was seen in all patients. However, at the end of the study period, seropositive and seronegative atypical ON had poor visual outcomes as compared to seronegative typical ON (P = 0.002). Five seropositive and four seronegative patients with atypical ON developed transverse myelitis. Bilateral disease with relapse was more in seropositive patients (80%); however, seronegative with atypical ON also had bilateral presentation and relapse in 42% and 41%, respectively. Conclusion: AQP4?Ab seropositive patients mostly present with atypical features such as bilateral recurrent ON, poor visual outcome, and increased incidence of transverse myelitis. However, atypical clinical features can also be seen in seronegative ON with a poor visual outcome and a recalcitrant course.

Effect of adrenocortical hormone on detection of NMO-IgG in neuromyelitisoptica patients / 中国生化药物杂志

Objective To evaluate the effect of adrenocortical hormone on detection of NMO-IgG in neuromyelitis optica ( NMO ) patients.Methods 30 cases with NMO collected in the hospital according to the figures were randomly divided into adrenocorticotropic hormone group and control group, 15 cases in each group.adrenocorticotropic hormone group were given glucocorticoid therapy on the basis of conventional therapy.The control group were treated with conventional therapy, and The serum NMO-IgG levels were detected by indirect immunofluorescence taking stable expression of human origin aquaporin -4 (AQP4) in human embryonic kidney 293 (HEK293) cell line as substrate.Results 19 cases (63.3%) serum NMO-IgG positive patients in 30 cases with spinal cord inflammation, serum antibody titers from 1:115 to 1砄6355.The positive rate of serum NMO-IgG in adrenocorticotropic hormone group was 86.7%, which was higher than 40.0% in control group (χ2 =7.03,P<0.05).Conclusion The adrenocortical hormone could help the detection of NMO-IgG antibody and increase the efficiency of clinical diagnosis.

Neuromielitis Óptica: patología, diagnóstico y tratamiento en el siglo XXI / Optic Neuromyelitis: pathology, diagnosis and therapy in the XXI century

La neuromielitis óptica (NMO) o enfermedad de Devic, pertenece al grupo de las enfermedades desmielinizantes del sistema nervioso central, afecta de manera significativa los nervios ópticos y la médula espinal. Desde el siglo XIX cuando Eugene Devic dio a conocer una serie de casos en los que existía asociación de lesiones en los nervios ópticos y la medula espinal, la relación de la neuromielitis óptica (NMO) y la Esclerosis Múltiple (EM) ha sido controversial, considerándose una variante de esta última; datos recientes muestran que puede ser distinguida de la Esclerosis Múltiple (EM). Los síntomas de la NMO son por lo general más agudos y severos y la presencia de un autoanticuerpo específico en sangre llamado NMO-IgG tipo acuoporina-4 (AQP4) desempeña un rol muy importante en la patogenia de esta enfermedad,asimismo se encuentran varias características entre ellas de tipo clínico, de laboratorio, neuroimágenes y en la anatomía patológica que diferencian la esclerosis múltiple (EM) de esta enfermedad. La proporción entre mujeres y hombres es mayor de 4 a 1.La presentación clínica y el curso de la enfermedad puede ser con recaídas en 80-90% y en un 10-20% curso monofásico. La manifestación distintiva de la entidad NMO es la ocurrencia ya sea consecutiva o simultánea de NO (unilateral o bilateral) y la presencia de mielitis longitudinal extensa (Mle). La terapia con corticosteroides intravenosos normalmente es el tratamiento inicial para los ataques agudos de neuritis óptica o mielitis. La plasmaféresis es la terapia de rescate cuando no hay respuesta a los esteroides durante los ataques de neuromielitis óptica. Los agentes inmunosupresores se usan para la prevención de las recaídas.

Optic Neuromyelitis (NMO) or Devic’s disease, is a demyelinating disease of central nervous system, affects the optic nerves and spinal cord. Since the nineteenth century when Eugene Devic unveiled a series of cases in which there was an association of lesions in the optic nerves and spinal cord with relation of NMO and Multiple Sclerosis (MS) has been controversial, in some cases described like a variant of the latter but recent data show that can be distinguished from multiple sclerosis (MS). Symptoms of NMO are usually more acute and severe and then we can find a presence of a specific autoantibody called NMO-IgG, it is a blood type acuoporina-4 (AQP4) and plays an important role in the pathogenesis of this disease. Also in the new millennium are various types of clinical, laboratory, neuroimaging and pathological anatomy then differentiates multiple sclerosis (MS) of this disease. In the epidemiology the ratio of women to men is greater than 4 to 1.The clinical presentation and course of the disease can be relapsing by 80-90% and 10-20% monophasic course. The distinctive manifestation is the occurrence NMO entity either consecutively or simultaneously not (unilateral or bilateral) and the presence of extensive longitudinal myelitis. Intravenous corticosteroid therapy is usually the initial therapy for acute attacks of optic neuritis or myelitis. Plasmapheresis therapy is used when the steroids therapy no work during acute attacks of NMO. Immunosuppressive agents used to prevent relapses.

Detecting serum anti-aquaporin-4 antibody in neuromyelitis optica patients by mimic antigen based ELISA / 中华微生物学和免疫学杂志

Objective To explore mimic antigen based ELISA for detecting serum anti-acquaporin4(AQP4) antibody in neuromyelitis optica(NMO) patients. Methods Three polypeptides: AQP456-69,AQP4135-155, AQP4209-230 were designed to simulate antigen epitopes of AQP4 through biological information and structure biology analysis, the peptides was used as antigen in ELISA to detect serum anti-AQP4 antibody in 9 NMO patients and 7 other miscellaneous neurological disorders which hayed been detected by immumofluorescence method. Results The mean value of A of anti-AQP4 antibody postive patients which have been determined by immumofluorescence method were higher than the controls in ELISA with AQP4135-155,AQP4209-230 as antigen (P<0.05). When the patients serum were diluted at 4 and 8 times, the A values were higher than controls significantly ( P<0.05 ). Conclusion Outmembrane polypeptides AQP4135-155,AQP4209-230 may be the main antigen epitope or main part of antigen epitope, they could be used to detect serum anti-AQP4 antibody in NMO patients.

Seroprevalence of NMO-IgG antibody in Brazilian patients with neuromyelitis optica / Soroprevalência do anticorpo NMO-IgG em pacientes brasileiros com neuromielite óptica

OBJECTIVE: To determine the seroprevalence of neuromyelitis optica antibody (NMO)-IgG in Brazilian patients with clinical diagnosis of relapsing neuromyelitis optica, also known as Devic's disease. METHOD: We determined NMO-IgG titers in 28 patients (25 of them females) that fulfilled the 1999 NMO diagnostic criteria proposed by Wingerchuk et al. RESULTS: NMO-IgG was detected in 18 NMO patients (64.3 percent). CONCLUSION: Our results showed that seroprevalence of NMO-IgG in Brazilian NMO patients was similar to the observed in other studies.

OBJETIVO: Determinar a soroprevalência do anticorpo neuromielite óptica (NMO)-IgG em pacientes brasileiros com diagnóstico de neuromielite óptica recorrente, também conhecida como doença de Devic. MÉTODO: Nós pesquisamos a presença do anticorpo NMO-IgG em 28 pacientes (25 do sexo feminino) que preenchiam os critérios diagnósticos para NMO propostos por Wingerchuk et al. em 1999. RESULTADOS: Dezoito pacientes (64,3 por cento) apresentaram a pesquisa positiva do NMO-IgG. CONCLUSÃO: Nossos resultados demonstraram que a soroprevalência do anticorpo NMO-IgG em pacientes brasileiros com NMO é semelhante àquela encontrada em outros estudos.

Devic’s neuromyelitis optica: a critical review

Devic's neuromyelitis optica (NMO) is an idiopathic inflammatory demyelinating and necrotizing disease characterized by predominant involvement of the optic nerves and spinal cord. In Asian countries relapsing NMO has been known as opticospinal multiple sclerosis. It has long been debated if NMO is a variant of multiple sclerosis (MS) or a distinct disease. Recent studies have shown that NMO has more frequently a relapsing course, and results from attack to aquaporin-4 which is the dominant water channel in the central nervous system, located in foot processes of the astrocytes. Distinctive pathological features of NMO include perivascular deposition of IgG and complement in the perivascular space, granulocyte and eosinophil infiltrates and hyalinization of the vascular walls. These features distinguish NMO from other demyelinating diseases such as MS and acute demyelinating encephalomyelopathy. An IgG-antibody that binds to aquaporin-4, named NMO-IgG has high sensitivity and specificity. Magnetic resonance imaging (MRI) studies have revealed that more frequently there is a long spinal cord lesion that extends through three or more vertebral segments in length. Brain MRI lesions atypical for MS are found in the majority of cases. Treatment in the acute phase includes intravenous steroids and plasma exchange therapy. Immunosupressive agents are recommended for prophylaxis of relapses.

Neuromielite óptica ou doença de Devic (NMO) é uma doença inflamatória com desmielinização e necrose envolvendo preferencialmente os nervos ópticos e a medula espinal. Desde sua descrição inicial tem havido controvérsia se a NMO é uma variante da esclerose múltipla (EM) ou se é uma entidade independente. Na Ásia a doença é conhecida como esclerose múltipla óptico-espinal. Recentes avanços tem demonstrado que na maioria dos casos a NMO é recorrente e resulta de alterações inflamatórias por ataque à aquaporina-4, uma proteína localizada nos pés dos astrócitos na barreira hemato-encefálica. Patologicamente a NMO difere da EM pela presença de necrose e cavitação no centro da medula, deposição perivascular de IgG e complemento, infiltração de neutrófilos e eosinófilos, assim como por hiperplasia e hialinização dos vasos. O anticorpo contra a aquaporina-4 (IgG-NMO), detectado no soro dos pacientes, tem alta sensibilidade e especificidade. Imagem por ressonância magnética demonstra lesão medular que se estende três ou mais segmentos vertebrais. Na maioria dos casos há lesões cerebrais atípicas para EM. Corticosteróide venoso em altas doses e plasmaférese são usados no tratamento das fases agudas, enquanto os imunossupressores devem ser usados na profilaxia das recorrências.

A Case of Neuromyelitis Optica(Devic's Syndrome) with Acute Bilateral Central Retinal Artery Occlusion / 대한소아신경학회지

Neuromyelitis optica(NMO) or Devic's syndrome is an uncommon clinical syndrome associating with unilateral or bilateral optic neuritis and transverse myelitis. It is rarely found in children and usually reported in adults with serious neurologic manifestations. We report a case of an 8-year-old girl with neuromyelitis optica whose first clinical manifestation was acute visual loss of both eyes. Initially the patient had been diagnosed with central retinal artery occlusion and optic neuritis by ophthalmologic examination, a brain magnetic resonance imaging, and cerebrospinal fluid findings. She was treated with intravenous methylprednisolone pulse therapy and heparinization. Then the treatments were replaced with oral prednisolone and warfarin. At the fifteenth day after the start of oral prednisolone tapering, she visited our emergency room for voiding difficulty and paresthesia on both legs. A spinal magnetic resonance imaging revealed increased signal intensity in T2-weighted images from cervical to lumbar level, and neuromyelitis optica- IgG(NMO-IgG) was detected in the patient's serum. After we diagnosed her as having neuromyelitis optica, intravenous methylprednisolone and nine courses of daily plasmapheresis were tried. However, the patient still had visual loss, pain, and sensory loss below the sixth thoracic dermatome, and we tried maintenance therapy with intravenous rituximab. We report our case with reviews of the related literatures.

Multiple Sclerosis

Multiple Sclerosis (MS) is the most common demyelinating disease affecting the central nervous system of young adults living in western countries. The clinical suspicion of demyelination, as a pathological process, is high when a young adult develops one or more neurological episodes indicating a damage to white matter tracts within the central nervous system, especially when the optic nerve, brainstem, or spinal cord is involved. The patient with episodes disseminating in time, each of which can be attributed to demyelination, requires no investigation prior to establishing the diagnosis of clinically definite MS, if the age of clinical presentation falls between 20 and 50 years, if different anatomical sites within the central nervous system have necessarily been affected on separate occasions, and if the clinical manifestation is typical for MS. MS in Asian populations is characterized by selective and dominant involvement of the optic nerve and spinal cords, as well as some incidence of brainstem lesion and symptoms. About 35~40% of cases of MS in Korea are of this optico-spinal type. Optico-spinal MS (OSMS) generally has a higher female-to-male ratio than conventional MS. OSMS is also characterized by frequent relapses, severe disability, few brain lesions on MRI, and a very lower incidence of oligoclonal bands in CSF. Neuromyelitis Optica (NMO) (Devic syndrome), which causes severe optic neuritis (ON) and longitudinally extensive transverse myelits either with a monophase or relapse-remitting pattern, is rare in Korean. NMO- IgG was reported to be helpful for the diagnosis of early-stage NMO and differential diagnoses of MS.

Study on value of NMO-IgG in diagnosis of neuromyelitis optica / 中国免疫学杂志

Objective:To investigate the value of NMO-IgG in diagnosis of neuromyelitis optica(NMO).Methods:A total of 120 patients with NMO,including high-risk NMO(HR-NMO),multiple sclerosis(MS),and other neurological diseases were enrolled in the study.The titres of serum NMO-IgG in all patients’ were detected by indirect immunofluorence.Results:The sensitivity and specificity of NMO-IgG were 67%,92% respectively in diagnosis of NMO.There were significantly differences in serum NOM-IgG posieivity among patients with NMO and MS who experienced severe optical neuritis,transverse myelitis,spinal-cord lesion beyond 3 segments as well as the patients with normal MRI scan in brain brain normal in MRI scan,serum and accompanied with other immunological disorders(P