BACKGROUND Atypical optic neuritis,consisting of neuromyelitis optica spectrum disorders(NMOSD)or myelin oligodendrocyte glycoprotein antibody disease(MOGAD),has a very similar presentation but different prognostic im...BACKGROUND Atypical optic neuritis,consisting of neuromyelitis optica spectrum disorders(NMOSD)or myelin oligodendrocyte glycoprotein antibody disease(MOGAD),has a very similar presentation but different prognostic implications and longterm management strategies.Vascular and metabolic factors are being thought to play a role in such autoimmune neuro-inflammatory disorders,apart from the obvious immune mediated damage.With the advent of optical coherence tomography angiography(OCTA),it is easy to pick up on these subclinical macular microvascular and structural changes.AIM To study the macular microvascular and structural changes on OCTA in atypical optic neuritis.METHODS This observational cross-sectional study involved 8 NMOSD and 17 MOGAD patients,diagnosed serologically,as well as 10 healthy controls.Macular vascular density(MVD)and ganglion cell+inner plexiform layer thickness(GCIPL)were studied using OCTA.RESULTS There was a significant reduction in MVD in NMOSD and MOGAD affected as well as unaffected eyes when compared with healthy controls.NMOSD and MOGAD affected eyes had significant GCIPL thinning compared with healthy controls.NMOSD unaffected eyes did not show significant GCIPL thinning compared to healthy controls in contrast to MOGAD unaffected eyes.On comparing NMOSD with MOGAD,there was no significant difference in terms of MVD or GCIPL in the affected or unaffected eyes.CONCLUSION Although significant microvascular and structural changes are present on OCTA between atypical optic neuritis and normal patients,they could not help in differentiating between NMOSD and MOGAD cases.展开更多
Neuromyelitis optica(NMO)refers to an antibody mediated,inflammatory disorder of the central nervous system(CNS)characterized by recurrent or monophasic attacks of optic neuritis and myelitis.Most patients with NMO po...Neuromyelitis optica(NMO)refers to an antibody mediated,inflammatory disorder of the central nervous system(CNS)characterized by recurrent or monophasic attacks of optic neuritis and myelitis.Most patients with NMO possess a specific serum immunoglobin,NMO-IgG,which can serve as a biomarker for NMO.The autoantibodies target aquaporin-4(AQP4),the main water channel protein found in the CNS including the brain,spinal cord,and optic nerve.The remaining 10-25%of patients are seronegative for NMO-IgG despite meeting the diagnostic criteria for NMO.Recent studies have shown that a subset of these patients is seropositive for antibodies against myelin oligodendrocyte glycoprotein(MOG).This paper will provide an overview of the current English scientific literature published regarding the history,epidemiology,AQP4 biomarker,MOG biomarker,diagnosis,clinical features,related diseases in NMO spectrum disorder(NMOSD),and treatments of NMO.展开更多
AIM：To compare the thickness of the peripapillary retinal nerve fiber layer（RNFL）and ganglion cell-inner plexiform layer（GCIPL）among patients with various forms of optic neuritis（ON）and to identify whether any ...AIM：To compare the thickness of the peripapillary retinal nerve fiber layer（RNFL）and ganglion cell-inner plexiform layer（GCIPL）among patients with various forms of optic neuritis（ON）and to identify whether any particular parameters or their thinning pattern can be used to distinguish the type of ON.METHODS：This prospective study was conducted at the Department of Ophthalmology,Faculty of Medicine,Siriraj Hospital,Thailand,between January,2015 and December,2016.We enlisted patients over 18 years of age with history of ON and categorized patients into 4 groups：1）aquaporin 4 antibodies（AQP4-IgG）positive;2）multiple sclerosis（MS）;3）myelin oligodendrocyte glycoprotein antibodies（MOG-IgG）positive;4）idiopathic-ON patients.Healthy controls were also included during the same study period.All patients underwent complete ophthalmological examination and spectral domain optical coherence tomography（OCT）imaging to analyze RNFL and GCIPL thickness after at least 3mo since the last episode of acute ON.The generalized estimating equation（GEE）models were used to compare the data amongst ON groups. RESULTS： Among 87 previous ON eyes from 57 patients（43 AQP4-IgG＋ON,17 MS-ON,8 MOG-IgG＋ON,and 19idiopathic-ON）,mean logMAR visual acuity of AQP4-IgG＋ON,MS-ON,MOG-IgG＋ON,and idiopathic-ON groups was 0.76±0.88,0.12±0.25,0.39±0.31,and 0.75±1.08,respectively.Average,superior,and inferior RNFL were significantly reduced in AQP4-IgG＋ON,MOG-IgG＋ON and idiopathic-ON eyes,relative to those of MS-ON.Differences were not statistically significant for RNFL or GCIPL between the AQP4-IgG＋ON and MOG-IgG＋ON groups,whereas visual acuity in MOG-IgG＋ON was slightly,but not significantly,better（0.39 vs 0.76）.Although RNFL thickness in MOG-IgG＋ON was significantly reduced as compared to MS-ON,mean visual acuity and GCIPL were not different.CONCLUSION：Thinning of superior and inferior quadrants of RNFL are more commonly seen in MOG-IgG＋ON and AQP4-IgG＋ON.Long term visual acuity in MOG-IgG＋ON is often better than AQP4-IgG＋ON,whereas the structural change from OCT is comparable.展开更多
目的探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性的中枢神经系统脱髓鞘病(central nervous system demyelinating disease)临床、影像学等特征。方法回顾性分析2017年12月至2019年8月无锡市儿童医院诊断的7例MOG抗体阳性中枢神经系统...目的探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性的中枢神经系统脱髓鞘病(central nervous system demyelinating disease)临床、影像学等特征。方法回顾性分析2017年12月至2019年8月无锡市儿童医院诊断的7例MOG抗体阳性中枢神经系统脱髓鞘病患儿的病例资料、随访情况等资料。结果7例患儿中,男2例,女5例,最终诊断2例为急性播散性脑脊髓炎(ADEM),2例为视神经脊髓炎谱系疾病(NMOSD),1例为视神经炎(ON),1例为急性横贯性脊髓炎(ATM),1例尚不能归类。脑脊液检查6例白细胞升高,2例蛋白升高。采用细胞转染色免疫荧光法(CBA)检测MOG抗体,7例血清均阳性,4例脑脊液阳性。影像学检查多部位受累,存在颅内病灶6例,脊髓2例。7例患儿静脉糖皮质激素和丙种球蛋白治疗效果好,随访7个月-2年不等,有2例复发。结论MOG抗体与多种儿童中枢神经系统脱髓鞘病有关,与感染和疫苗接种可能有关,对激素和丙种球蛋白治疗敏感,有易复发的特点。展开更多
文摘BACKGROUND Atypical optic neuritis,consisting of neuromyelitis optica spectrum disorders(NMOSD)or myelin oligodendrocyte glycoprotein antibody disease(MOGAD),has a very similar presentation but different prognostic implications and longterm management strategies.Vascular and metabolic factors are being thought to play a role in such autoimmune neuro-inflammatory disorders,apart from the obvious immune mediated damage.With the advent of optical coherence tomography angiography(OCTA),it is easy to pick up on these subclinical macular microvascular and structural changes.AIM To study the macular microvascular and structural changes on OCTA in atypical optic neuritis.METHODS This observational cross-sectional study involved 8 NMOSD and 17 MOGAD patients,diagnosed serologically,as well as 10 healthy controls.Macular vascular density(MVD)and ganglion cell+inner plexiform layer thickness(GCIPL)were studied using OCTA.RESULTS There was a significant reduction in MVD in NMOSD and MOGAD affected as well as unaffected eyes when compared with healthy controls.NMOSD and MOGAD affected eyes had significant GCIPL thinning compared with healthy controls.NMOSD unaffected eyes did not show significant GCIPL thinning compared to healthy controls in contrast to MOGAD unaffected eyes.On comparing NMOSD with MOGAD,there was no significant difference in terms of MVD or GCIPL in the affected or unaffected eyes.CONCLUSION Although significant microvascular and structural changes are present on OCTA between atypical optic neuritis and normal patients,they could not help in differentiating between NMOSD and MOGAD cases.
文摘Neuromyelitis optica(NMO)refers to an antibody mediated,inflammatory disorder of the central nervous system(CNS)characterized by recurrent or monophasic attacks of optic neuritis and myelitis.Most patients with NMO possess a specific serum immunoglobin,NMO-IgG,which can serve as a biomarker for NMO.The autoantibodies target aquaporin-4(AQP4),the main water channel protein found in the CNS including the brain,spinal cord,and optic nerve.The remaining 10-25%of patients are seronegative for NMO-IgG despite meeting the diagnostic criteria for NMO.Recent studies have shown that a subset of these patients is seropositive for antibodies against myelin oligodendrocyte glycoprotein(MOG).This paper will provide an overview of the current English scientific literature published regarding the history,epidemiology,AQP4 biomarker,MOG biomarker,diagnosis,clinical features,related diseases in NMO spectrum disorder(NMOSD),and treatments of NMO.
文摘AIM：To compare the thickness of the peripapillary retinal nerve fiber layer（RNFL）and ganglion cell-inner plexiform layer（GCIPL）among patients with various forms of optic neuritis（ON）and to identify whether any particular parameters or their thinning pattern can be used to distinguish the type of ON.METHODS：This prospective study was conducted at the Department of Ophthalmology,Faculty of Medicine,Siriraj Hospital,Thailand,between January,2015 and December,2016.We enlisted patients over 18 years of age with history of ON and categorized patients into 4 groups：1）aquaporin 4 antibodies（AQP4-IgG）positive;2）multiple sclerosis（MS）;3）myelin oligodendrocyte glycoprotein antibodies（MOG-IgG）positive;4）idiopathic-ON patients.Healthy controls were also included during the same study period.All patients underwent complete ophthalmological examination and spectral domain optical coherence tomography（OCT）imaging to analyze RNFL and GCIPL thickness after at least 3mo since the last episode of acute ON.The generalized estimating equation（GEE）models were used to compare the data amongst ON groups. RESULTS： Among 87 previous ON eyes from 57 patients（43 AQP4-IgG＋ON,17 MS-ON,8 MOG-IgG＋ON,and 19idiopathic-ON）,mean logMAR visual acuity of AQP4-IgG＋ON,MS-ON,MOG-IgG＋ON,and idiopathic-ON groups was 0.76±0.88,0.12±0.25,0.39±0.31,and 0.75±1.08,respectively.Average,superior,and inferior RNFL were significantly reduced in AQP4-IgG＋ON,MOG-IgG＋ON and idiopathic-ON eyes,relative to those of MS-ON.Differences were not statistically significant for RNFL or GCIPL between the AQP4-IgG＋ON and MOG-IgG＋ON groups,whereas visual acuity in MOG-IgG＋ON was slightly,but not significantly,better（0.39 vs 0.76）.Although RNFL thickness in MOG-IgG＋ON was significantly reduced as compared to MS-ON,mean visual acuity and GCIPL were not different.CONCLUSION：Thinning of superior and inferior quadrants of RNFL are more commonly seen in MOG-IgG＋ON and AQP4-IgG＋ON.Long term visual acuity in MOG-IgG＋ON is often better than AQP4-IgG＋ON,whereas the structural change from OCT is comparable.
文摘目的探讨儿童髓鞘少突胶质细胞糖蛋白(MOG)抗体阳性的中枢神经系统脱髓鞘病(central nervous system demyelinating disease)临床、影像学等特征。方法回顾性分析2017年12月至2019年8月无锡市儿童医院诊断的7例MOG抗体阳性中枢神经系统脱髓鞘病患儿的病例资料、随访情况等资料。结果7例患儿中,男2例,女5例,最终诊断2例为急性播散性脑脊髓炎(ADEM),2例为视神经脊髓炎谱系疾病(NMOSD),1例为视神经炎(ON),1例为急性横贯性脊髓炎(ATM),1例尚不能归类。脑脊液检查6例白细胞升高,2例蛋白升高。采用细胞转染色免疫荧光法(CBA)检测MOG抗体,7例血清均阳性,4例脑脊液阳性。影像学检查多部位受累,存在颅内病灶6例,脊髓2例。7例患儿静脉糖皮质激素和丙种球蛋白治疗效果好,随访7个月-2年不等,有2例复发。结论MOG抗体与多种儿童中枢神经系统脱髓鞘病有关,与感染和疫苗接种可能有关,对激素和丙种球蛋白治疗敏感,有易复发的特点。
