Clinical characteristics of patients with early-and late-onset optic neuromyelitis optica spectrum disease

Objective:To analyze the different clinical features of patients with early-onset(EO-NMOSDs)and late-onset neuromyelitis optica spectrum diseases(LO-NMOSDs).Methods:A total of 51patients with neuromyelitis optica spectrum disease who were diagnosed in our hospital for the first time from January 2015 to December 2022 were included in the First Affiliated Hospital of Hainan Medical College and divided into 22 cases in the EO-NMOSDs group and 29 cases in the LO-NMOSDs group according to whether the age of onset was 50 years old.The basic data,Extended Disability Status Scale(EDSS)score,blood and cerebrospinal fluid test indicators of the two groups were statistically analyzed.Results:There were no significant differences in demographic characteristics,clinical features and serum AQP-4 antibody positivity rate between the two groups(all P>0.05),and there were significant differences in triglycerides(TG),low-density lipoprotein(LDL),apolipoprotein A(APOA),apolipoprotein B(APOB)and lipoprotein a(P=0.010,P=0.048,P=0.014,P=0.061,P=0.001,respectively),and cerebrospinal fluid LDH,There were significant differences between microprotein quantification and EDSS score(P=0.018,P=0.034,P=0.025,respectively),and the level of microprotein quantification in cerebrospinal fluid of LO-NMOSDs had a certain correlation with the degree of disability(r=0.52,P<0.03).Conclusion:LO-NMOSDs and EO-NMOSDs group patients have similar demographic characteristics,serum AQP-4 antibody positive rate and clinical features,but compared with EO-NMOSDs,patients in LO-NMOSDs group are prone to abnormal lipid metabolism,higher trace proteins in cerebrospinal fluid and more likely to be disabled,and among LO-NMOSDs,the higher the trace protein in the cerebrospinal fluid,the more severe the disability status of patients.

Retinal ganglion cell-inner plexiform and nerve fiber layers in neuromyelitis optica

AIM： To determine the thickness of the retinal ganglion cell-inner plexiform layer（GCIPL） and the retinal nerve fiber layer（RNFL） in patients with neuromyelitis optica（NMO）.METHODS： We conducted a cross-sectional study that included 30 NMO patients with a total of 60 eyes. Based on the presence or absence of optic neuritis（ON）, subjects were divided into either the NMO-ON group（30 eyes） or the NMO-ON contra group（10 eyes）. A detailed ophthalmologic examination was performed for each group; subsequently, the GCIPL and the RNFL were measured using highdefinition optical coherence tomography（OCT）. RESULTS： In the NMO-ON group, the mean GCIPL thickness was 69.28±21.12 μm, the minimum GCIPL thickness was 66.02±10.02 μm, and the RNFL thickness were 109.33±11.23, 110.47±3.10, 64.92±12.71 and 71.21±50.22 μm in the superior, inferior, temporal and nasal quadrants, respectively. In the NMO-ON contra group, the mean GCIPL thickness was 85.12±17.09 μm, the minimum GCIPL thickness was 25.39±25.1 μm, and the RNFL thicknesses were 148.33±23.22, 126.36±23.45, 82.21±22.30 and 83.36±31.28 μm in the superior, inferior, temporal and nasal quadrants, respectively. In the control group, the mean GCIPL thickness was 86.98±22.37 μm, the minimum GCIPL thickness was 85.28±10.75 μm, and the RNFL thicknesses were 150.22±22.73, 154.79±60.23, 82.33±7.01 and 85.62±13.81 μm in the superior, inferior, temporal and nasal quadrants, respectively. The GCIPL and RNFL were thinner in the NMO-ON contra group than in the control group（P〈0.05）; additionally, the RNFL was thinner in the inferior quadrant in the NMO-ON group than in the control group（P〈0.05）. Significant correlations were observed between the GCIPL and RNFL thickness measurements as well as between thickness measurements and the two visual field parameters of mean deviation（MD） and corrected pattern standard deviation（PSD） in the NMO-ON group（P〈0.05）. CONCLUSION： The thickness of the GCIPL and RNFL, as measured using OCT, may indicate optic nerve damage in patients with NMO.

Clinical characteristics of 72 cases with neuromyelitis optical associated optic neuritis

Objective:To analyze the clinical characteristics of neuromyelitis optical associated optic neuritis(NMO-ON)patients,and to provide reference and basis for the prevention and treatment accordingly.Methods:The medical records of 72 NMO patients with ON as the first clinical manifestation in China-Japan Friendship Hospital from January 2016 to December 2019 were retrospectively analyzed and summarized,including general information,morbidity characteristics,course of disease,comorbid diseases,immunological tests,treatment response and prognosis,etc.Results:Totally 72 NMO-ON patients had a median age of 33 years.The ratio of male to female is about 1:5.54;The median course was 67 months,mainly"relapseremission".Totally 61.11% patients were successively involved in both eyes,the median incidence of ON was 2 times,and the median time of the second onset of ON was 3 months.The 1-year and 3-year recurrence rates were 55.56% and 73.61%,respectively.Around 91.67% of the patients had the onset of ON alone,and 81.94% of the patients had monocular involvement.About 19.44% patients were associated with inducement,the most common was upper respiratory tract infection;15.28% patients were associated with systemic immune diseases,most commonly associated with Sjogren's syndrome and thyroid diseases and 75.64% patients had first visual acuity less than 0.1,aquaporin-4 immunoglobulin G(AQP4-IgG)status(P=0.032,OR=2.55)and onset age(P=0.037,OR=3.93)were independent risk factors for first visual acuity.Up to the last follow-up time,the rate of unilateral blindness was about 48.61%,and the median of unilateral blindness ON was 2 times.Other nervous system involvement occurred in 73.61% of patients,and spinal cord(61.11%)was the most common site of recurrence.Serum AQP4-IgG was positive in 80.00%(48/60)of patients.A total of 18 cases(25.00%)were associated with other systemic immune antibodies,most commonly associated with ANA antibody positivity.Conclusions:The first onset of NMO-ON patients is mostly ON alone,with unilateral involvement and high incidence in young and middle-aged women.Bilateral optic nerve involvement and repeated recurrence are common in the long course of disease.AQP4-IgG status and onset age are independent risk factors affecting the visual function of NMO patients for the first onset,and most patients have positive AQP4-IgG serum.Some patients are associated with systemic immune diseases represented by Sjogren's syndrome and thyroid disease,which are at high clinical risk and require early diagnosis and treatment intervention.