Neuromyelitis optica spectrum disorder (NMOSD) is a immune-mediated demyelinating disease of the central nervous system, characterized by high recurrence and disability rates. Preventing relapses is crucial in the treatment of this condition. Monoclonal antibodies have emerged as a novel and rapidly evolving clinical therapeutic strategy targeting NMOSD in recent years. An increasing number of studies and clinical trials have also confirmed the effectiveness and safety of monoclonal antibodies. Rituximab, a monoclonal antibody targeting the B-cell surface antigen CD20, has been widely used in the treatment of NMOSD. Currently, in China, the only approved monoclonal antibody for treating NMOSD is Inebilizumab, which targets the B-cell surface antigen CD19. Additionally, various monoclonal antibodies, such as interleukin-6 receptor inhibitors and complement C5 inhibitors, have been used in the treatment of NMOSD. With the deepening of the research on the pathogenesis of NMOSD, the molecular mechanism of disease-related immune network is further clarified, and multi-center clinical trials are widely carried out. More accurate monoclonal antibody treatment strategies for NMOSD will be applied to clinical practice, benefiting more patients.
ObjectiveTo observe the clinical characteristics and optical coherence tomography (OCT) features of pseudopapilledema (PPE) combined with peripapillary hyper-reflective ovoid mass-like structures (PHOMS) in children. MethodsA retrospective observational study. From October 2019 to May 2021, total 22 eyes from 12 children diagnosed as PPE combined with PHOMS in the Neuro-ophthalmology Department of The First Hospital of Xi’an (Affiliated of The First Hospital of Northwest University) were recruited. Among the children, 6 were male and 6 were female. The average age was (10.6±2.7) years. The average course from disease onset to diagnosis of PPE combined with PHOMS was (8.0±7.5) months. All patients underwent best corrected visual acuity (BCVA), relative afferent papillary defect (RAPD), Ishihara's test, fundus photography, OCT, fundus autofluorescence (FAF), ocular B-mode ultrasound, visual field and patternvisual evoked potential (P-VEP). The clinical and OCT characteristics of the patients were observed. ResultsThe anterior segments of the patients were normal. The intraocular pressures and Ishihara's test were all normal. All RAPD were negative. Total 22 eyes, BCVA was 1.0 in 21 eyes and one eye was 0.12. The fundus photography revealed blurred optic discs margin, showing mild to moderate edema-like elevation with more prominent in the nasal parts, presenting as a “C” shape halo. No obvious abnormal fluorescence was observed in FAF. The OCT scan of involvement eyes showed an elevated appearance in vary degrees, and the sharply marginated ovoid hyper-reflective mass-like structures which laterally herniated into the peripapillary region under retinal nerve fiber layer and above the Bruch membrane were detected with consecutive nasal enlargement scanning, corresponding to the nasal parts in the fundus photography. The higher degree of elevation, the larger the volume. Macular retina pigment epithelium layer and ganglion cell thickness were normal. Ocular B-mode ultrasound showed that the head of the optic nerve in the posterior wall of the eyeball (in front of the optic disc) was elevated in all affected eyes, and there was no strong signal echo in it. Visual field examination showed physical blind spot enlargement in 3 eyes and visual field defect in 2 eyes. P-VEP examination showed that the peak was slightly delayed in 3 eyes and the amplitude was slightly reduced in 3 eyes. ConclusionsEnlarged nasal optic disc OCT scan can improve the detection rate of PHOMS. PHOMS were detected bilaterally in the cases with binocular PPE while only in the effected eye in the cases of monocular PPE; the higher degree of PPE, the lager volume of PHOMS. PHOMS were could contribute to the diagnosis of PPE in children.
ObjectiveTo investigate the clinical characteristics of vascular neuro-ophthalmology in patients with central retinal artery occlusion (CRAO). MethodsA single-center, prospective clinical study. From January 2018 to December 2020, 49 eyes of 49 CRAO patients of The Neuro-ophthalmology Department of Xi'an First Hospital were included in the study. Data on patient demographic characteristics, vascular risk factors, disease characteristics, digital subtraction angiography (DSA) imaging characteristics of internal carotid arteries, treatment, treatment-related adverse events, and 1-month follow-up vascular events were collected. All patiens were examined by visual acuity, head CT and or magnetic resonance imaging. At the same time, 35 cases of internal carotid artery vascular DSA were examined; 14 cases of head and neck CT angiography were examined. The anatomical variation of the extracranial segment of the internal carotid artery was divided into tortuous, tortuous, and coiled; the aortic arch was divided into type Ⅰ, type Ⅱ, type Ⅲ, and bovine type. Intravenous thrombolysis, arterial thrombolysis, conservative treatment were performed. The follow-up time was 1 month after treatment. Functional vision was defined as vision ≥20/100. Vascular events were strokes, cardiovascular events, deaths and neovascular glaucoma during follow-up. ResultsAmong 49 eyes of 49 cases, 40 eyes were male (81.6%, 40/49), and 9 eyes were female (18.4%, 9/49); the average age was 60.7±12.9 years. There were 33, 17, and 16 cases with hypertension, type 2 diabetes, and cerebrovascular disease, respectively; 27 and 34 cases had a history of smoking and tooth loss, respectively. Taking antihypertensive, hypoglycemic, antiplatelet aggregation/anticoagulation, and hypolipidemic drugs were 15, 5, 8, and 5 patients, respectively. There were 11 cases of transient amaurosis before the onset, and 17 cases of CRAO after waking up. There were 33 cases (67.3%, 33/49) with infarction of the affected side of the brain tissue. DSA was performed in 35 cases, and the stenosis rate of the internal carotid artery on the affected side was 70%-99% and 100% were 3 (8.6%, 3/35) and 4 (11.4%, 4/35) cases, respectively. The ophthalmic artery on the affected side originated from the external carotid artery in 5 cases (14.3%, 5/35). There were 17 (54.8%, 17/31) and 2 (6.5%, 2/31) cases of tortuousity and kinking in the extracranial segment of the internal carotid artery. There were 15 (42.9%, 15/35), 6 (17.1%, 6/35), and 2 (5.7%, 2/35) cases of aortic arch type Ⅱ, type Ⅲ, and bovine type, respectively. Intravenous thrombolysis and arterial thrombolysis were performed in 13 and 29 cases, respectively. Complications occurred in 2 cases during treatment; 3 cases of symptoms fluctuated after treatment, and 10 cases of asymptomatic new infarcts occurred in imaging studies. Forty-eight cases were treated with antiplatelet aggregation/anticoagulation and hypolipidemic treatment. At discharge and 1 month after treatment, the recovery of functional vision was 7 and 17 cases, respectively. One month after treatment, 1 case died because myocardial infarction; 2 cases of neovascular glaucoma occurred. ConclusionThe proportion of CRAO patients with vascular risk factors and internal carotid artery abnormalities on the affected side is relatively high; the prognosis is relatively good after intravenous thrombolysis and/or arterial thrombolysis and secondary stroke prevention.