A case of limbic encephalitis with positive anti-leucine-rich glioma inactivated 1 protein antibody and anti-myelin oligodendrocyte glycoprotein antibody / 中华神经科杂志

A case of limbic encephalitis with positive anti-leucine-rich glioma inactivated 1 protein (LGI1) antibody and anti-myelin oligodendrocyte glycoprotein (MOG) antibody was reported. The patient was a middle-aged male with a history of retinal vein occlusion. The main symptoms were temporal lobe epilepsy, facial arm dystonia, autonomic nerve dysfunction. Magnetic resonance imaging showed long T 2 signal in the right hippocampus without enhancement and normal perfusion. Electroencephalogram showed paroxysmal slow wave and sharp slow wave in interictal period. Blood anti-MOG antibody, blood and cerebrospinal fluid anti-LGI1 antibody were double positive. The main diagnosis was limbic encephalitis. After treatment with hormone and gamma globulin, the symptoms were improved and double antibodies were turned negative. Anti-LGI1/MOG double positive cases are rare, and the clinical manifestations and imaging manifestations of double positive antibody cases are not completely consistent with those with each single antibody, with different characteristics. This report can help clinicians enhance awareness.

Advances in myelin oligodendrocyte glycoprotein antibody-associated disease / 国际儿科学杂志

Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is one of inflammatory demyelinating disorders of the central nervous system.Due to the diverse and atypical clinical manifestations, positive rate of current detection methods is not high, and early diagnosis is difficult.This review aims to elucidate the research progress of the epidemiology, pathogenesis, clinical characteristics, diagnostic criteria, biological markers, treatment and prognosis of MOGAD, in order to improve the understanding of MOGAD for clinicians, so as to identify, treat and reduce the recurrence of MOGAD as early as possible.

Analysis of influencing factors related to the prognosis of visual acuity on myelin oligodendrocyte glycoprotein antibody positive optic neuritis after methylprednisolone pulse / 中华眼底病杂志

Objective:To analyze the prognostic factors of vision of myelin oligodendrocyte glycoprotein (MOG) antibody positive associated optic neuritis (ON) after methylprednisolone pulse therapy.Methods:A clinical observational study. A total of 32 patients (47 eyes) with MOG antibody positive ON were observed and followed up in the ophthalmology department of Beijing Tongren Hospital Affiliated to Capital Medical University and Beijing Puren Hospital from March 2019 to January 2022. Clinical data including the best corrected visual acuity (BCVA) and orbital magnetic resonance imaging were recorded. The BCVA was examined by Snellen visual acuity chart, which was finally converted into the logarithm of the minimal angle of resolution (logMAR) for statistical analysis. There were 22 case (38 eyes) with complete image data. All patients were treated with intravenous methylprednisolone pulse (IVMP) for 3-5 days. According to the intervention time (from onset to glucocorticoid treatment), the patients were divided into three groups: <7 d group, 7-14 d and> 14 d group, with 16, 13, 11 eyes, respectively. The median follow-up time was 28 months. After 1 week, 1, 3 and 6 months treatment, the same equipment and methods were used for relevant examinations to observe the changes of visual acuity and the factors influencing the prognosis of visual acuity after IVMP treatment. Logistic regression and linear regression were used to analyze the prognostic correlation factors. Receiver operating characteristic (ROC) curve was used to determine the critical cut-off point of intervention timing.Results:Among the patients, 16 were male and 16 were female. The median onset age was 26 years. The onset duration time was 5-60 days. There were 18 cases (56.3%, 18/32) with abnormal serum immune indexes. The initial symptom was decreased vision with unilateral or bilateral ON. Seventeen (53.1%, 17/32) cases had unilateral ON and 15 (46.9%, 15/32) cases with bilateral ON. Thirty-six eyes (76.6%, 36/47) got optic disc edema, 37 eyes (78.7%, 37/47) accompanied by pain of ocular movement. The nadir logMAR BCVA was mean 1.69±0.13. Long T2WI signals with segmental thickening in the orbital segment of the optic nerve were obtained in 27 eyes (71.1%, 27/38) and in 24 eyes (63.2%, 24/38) with optic nerve and sheath enhancement. During the follow-up period, there were 10 cases of relapse (31.3%, 10/32). The logMAR BCVA of attacked eyes were 0.52±0.09, 0.22±0.06, 0.12±0.06, 0.10±0.06 at 1 week and 1, 3 and 6 months after IVMP treatment, respectively. The rate of BCVA improvement was the fastest at 1 week after treatment, and BCVA returned to stability at 3 months. Logistic regression analysis showed that the timing of intervention was significantly correlated with the prognosis of vision in primary onset patients (odds ratio=12.17, P=0.006), with a negative linear regression relationship ( r=-0.48, 95% confidence interval -0.71--0.17, P=0.008). Comparing the logMAR BCVA between the intervention time >14 group with the <7 group and the 7-14 group, there were statistically significant difference ( P=0.017, 0.037), respectively. The cut-off point of ROC curve to predict the optimal intervention time was 13.5 days. Other factors such as: gender, age, predisposing factor, pain of eye motion, edema of optic disc, bilateral ON, imaging changes, abnormal autoimmune indicators were not associated with the prognosis of visual acuity. Conclusion:The timing of hormone intervention in primary onset patients is an important factor affecting the prognosis of vision and the optimal intervention time window of IVMP is two weeks.

Clinical and imaging features of 13 children with myelin oligodendrocyte glycoprotein antibody encephalomyelitis / 中华实用儿科临床杂志

Objective To investigate the clinical and imaging features of myelin oligodendrocyte glycoprotein antibody(MOG)encephalomyelitis in children. Methods The clinical,laboratory finding,imaging and follow-up data of 13 children with MOG encephalomyelitis(MOG-EM)diagnosed by Children＇s Hospital of Nanjing Medical Univer-sity from December 2016 to December 2018 were retrospectively analyzed. Results Among the 13 children,4 cases were male and 9 cases were female,the median age was 8 years old and 1 month. Symptoms of the first episode included fever,drowsiness in 2 cases,visual acuity in 5 cases,convulsions in 3 cases,urinary retention in 2 cases,and ataxia in 2 cases. Abnormalities were found in 12 cases by the head magnetic resonance imaging(MRI),most of which showed ex-tensive or isolated subcortical white matter lesions,and a few deep gray matter nuclei and brainstem were involved;3 ca-ses of spinal MRI abnormalities,mainly characterized by long segmental transverse myelitis;6 cases optic nerve MRI ab-normalities were found in 6 cases,manifested as disease side optic nerve or optic chiasm abnormal signals;the titer of serum MOG antibody was 1: 10-1: 320 in 13 cases. All children responded well to glucocorticoids and gamma globu-lin,and all symptoms were alleviated after treatment. Two patients had recurrence during the follow-up period,which was characterized by optic neuritis. After azathioprine addition,there was no recurrence after 1 to 2 years of follow-up. Conclusions Children with MOG antibody encephalomyelitis present a decline in visual acuity commonly. The images are mainly acute disseminated encephalomyelitis-like changes,immunosuppressive therapy is effective,generally with a better prognosis.

Comparison of the low frequency fluctuation between AQP4 antibody positive and MOG antibody positive patients with optic neuritis / 中华实验眼科杂志

Objective@#To compare the changes of spontaneous brain activity in myelin oligodendrocyte glycoprotein antibody (MOG-Ab) positive and Aquaporin 4 antibody (AQP4-Ab) positive neuromyelitis optica spectrum disorder (NMOSD) by using resting-state functional magnetic resonance imaging (fMRI).@*Methods@#A case control study was designed.A total of 11 NMOSD patients with positive MOG-Ab and 21 NMOSD patients with positive AQP4-Ab were enrolled from October 2006 to May 2017 in PLA General Hospital.Thirty-four healthy controls closely matched in age, sex and education were recruited and underwent resting-state fMRI scans.The amplitude of low-frequency fluctuation (ALFF) was extracted to investigate the spontaneous brain activity.This study was approved by Ethics Committee of PLA General Hospital (S2019-111-01). All subjects enrolled signed informed consent.@*Results@#Two patients in the MOG-Ab positive group had seizure history, and no seizure history was observed in AQP4-Ab positive group and healthy control group.Compared with healthy control group, all patients in MOG-Ab positive group and AQP4-Ab positive group had significantly increased ALFF values of prefrontal gyrus.The ALFF values of bilateral anterior central gyrus and bilateral posterior central gyrus in AQP4-Ab positive group were 1.89±0.56 and 2.10±0.69, respectively, which were lower than 3.32±1.15 and 3.61±1.23 in MOG-Ab positive group, the differences were statistically significant (both at P<0.001, AlphaSim correction).@*Conclusions@#Resting-state fMRI could provide new evidence of possibly multi-focal disease mechanisms.Hyperactivity in prefrontal cortex, motor cortex and somatosensory cortex might reflect differences in pathological processes between MOG-Ab positive and AQP4-Ab positive NMOSD patients.

Clinical and imaging features of 13 children with myelin oligodendrocyte glycoprotein antibody encephalomyelitis / 中华实用儿科临床杂志

Objective@#To investigate the clinical and imaging features of myelin oligodendrocyte glycoprotein antibody(MOG) encephalomyelitis in children.@*Methods@#The clinical, laboratory finding, imaging and follow-up data of 13 children with MOG encephalomyelitis (MOG-EM) diagnosed by Children′s Hospital of Nanjing Medical University from December 2016 to December 2018 were retrospectively analyzed.@*Results@#Among the 13 children, 4 cases were male and 9 cases were female, the median age was 8 years old and 1 month.Symptoms of the first episode included fever, drowsiness in 2 cases, visual acuity in 5 cases, convulsions in 3 cases, urinary retention in 2 cases, and ataxia in 2 cases.Abnormalities were found in 12 cases by the head magnetic resonance imaging(MRI), most of which showed extensive or isolated subcortical white matter lesions, and a few deep gray matter nuclei and brainstem were involved; 3 cases of spinal MRI abnormalities, mainly characterized by long segmental transverse myelitis; 6 cases optic nerve MRI abnormalities were found in 6 cases, manifested as disease side optic nerve or optic chiasm abnormal signals; the titer of serum MOG antibody was 110-1320 in 13 cases.All children responded well to glucocorticoids and gamma globulin, and all symptoms were alleviated after treatment.Two patients had recurrence during the follow-up period, which was characterized by optic neuritis.After azathioprine addition, there was no recurrence after 1 to 2 years of follow-up.@*Conclusions@#Children with MOG antibody encephalomyelitis present a decline in visual acuity commonly.The images are mainly acute disseminated encephalomyelitis-like changes, immunosuppressive therapy is effective, generally with a better prognosis.

Anti-myelin oligodendrocyte glycoprotein antibody and central nervous system immune demyelinating diseases / 中华实用儿科临床杂志

Myelin oligodendrocyte glycoprotein (MOG) is a component of central nervous system myelin.Anti-MOG antibodies are important in both pediatric and adult demyelination and the clinical association of MOG antibody-induced demyelination has been refined to include neuromyelitis optica spectrum disease,optic neuritis,and acute disseminated encephalomyelitis.But there are still many questions about epidemiological,clinical,radiological,and prognostic profile in patients with MOG antibody-associated demyelination.No consensus has been reached on detecting this antibody.This review will summarize the advances in anti-Myelin oligodendrocyte glycoprotein antibody.