On September 27, 2022, the first international standard of Diagnosis and Classification of Optic Neuritis was published online in Lancet Neurology, the top journal of neurology. The publication of this standard fills the gap in the international diagnosis and classification of optic neuritis (ON), promotes the consistency of the global diagnosis classification of ON, and helps ophthalmologists and neurologists to diagnose ON more accurately in the future. In recent decades, although Chinese ophthalmologists have made great progress in their understanding of ON and diagnosis and treatment level, it is still necessary to continue to strengthen the standardized training of Chinese neuro-ophthalmologist specialists, cooperate to establish a national ON clinical epidemiology database, carry out Chinese multi-center clinical studies, and further verify and optimize the international ON diagnostic system in future clinical practice. The ON diagnostic standards and treatment standards are gradually improved for China.
Neuromyelitis optica-related optic neuritis (NMO-ON) is a kind of severe optic nerve disease, which always leads to replase, poor prognosis, and even blindness. Aquaporin 4 antibody (AQP4-IgG) is the main diagnostic biomarker for neuromyelitis optica with high specificity. Serum myelin oligodendrocyte glycoprotein antibody (MOG-IgG) is helpful for the diagnosis of AQP4-IgG negative patients. The study of biomarkers is helpful to deeply understand the pathogenesis of NMO-ON, help the diagnosis of the disease, and finally make precise treatment. Orbital MRI can help to differentiate MOG-IgG positive from AQP4-IgG positive neuromyelitis optica and optic neuritis, which is very important for the diagnosis of NMO-ON. At present, the standardized treatment of NMO-ON can be divided into two clinical stages: acute stage and remission stage. Corticosteroids and plasma exchange are the main treatments in acute stage, aiming at alleviating acute inflammatory reaction and improving prognosis. Immunosuppressive agents and biological agents are the main treatments in remission stage, aiming at preventing or reducing recurrence. With the development of the diagnosis and treatment of NMO-ON, we find that it is more and more important to strengthen the construction of neuro-ophthalmology team in China, establish clinical epidemiological database of NMO-ON, and carry out multi-centre, large-sample, prospective clinical control studies in China to provide evidence-based medicine for Chinese people. In addition, we need to strengthen efforts to establish and improve the diagnostic criteria for NMO-ON and the promotion of diagnostic and therapeutic criteria, and strive to improve the clinical diagnosis and treatment level of NMO-ON in China.
Critical flicker fusion frequency (CFF) is a dynamic visual function test that measures the minimum frequency at which a flicker source is perceived by the visual system as continuous light. The measurement method is convenient, the inspection time is short, and it can be effectively evaluated in the case of refractive interstitial opacity. Although CFF has many advantages, its application in the field of ophthalmology has not received sufficient attention. The pathway of CFF involves the pathway from the retina to the lateral geniculate body to the primary visual cortex, where the macrocellular pathway is sensitive to temporal resolution and responsible for transmitting rapidly changing information. Its measurements typically use red, green, or yellow light as a flashing light source to detect the functional integrity of the macular region. As a subjective test, the results of CFF can be affected by a variety of factors, such as drug use, fatigue, and luminous intensity. In order to improve the repeatability of the measurement, it is necessary to follow standardized measurement steps. CFF has important application value in the diagnosis of optic nerve diseases. It can assist in diagnosing the presence of optic neuropathy, evaluating the conduction function of the optic nerve, and monitoring the progression of the disease and the effect of treatment. As a convenient and efficient visual function evaluation tool, CFF has great potential in the diagnosis of optic nerve diseases and visual function monitoring. In view of its application prospects in the field of ophthalmology, this study calls for more attention and support from ophthalmologists, and carry out related basic and clinical research to further explore the application value of CFF in different disease conditions.
Neuromyelitis optica (NMO) is an autoimmune inflammatory diseases of the central nervous systems (CNS) mainly affecting the optic nerves and spinal cord. It has the characteristics of high recurrence rate and poor prognosis. NMO related optic neuritis is a common neuro-ophthalmic disease which often results in permanent visual loss or even blindness. Aquaporin 4 (AQP4) antibody is a specific and pathogenic autoantibody in NMO patients. Although AQP4 is expressed in multiple tissues, NMO pathology is remarkably limited to the CNS. Corticosteroids and other immunosuppressive drugs are the standard managements for NMO patients, in order to reduce the relapses and the severity of the acute attack. Multiple avenues of investigation in the laboratory have significantly advanced our understanding of NMO pathophysiology, which is helpful for our understanding of immunologic and nonimmunologic mechanisms. Many offer significant means for NMO therapy by selectively targeting pathways. In the future, moving these agents from the bench to the bedside offers the opportunity to identify safe and effective therapies that limit CNS injury and preserve visual function.
Neuromyelitis optica spectrum disorder (NMOSD) is a kind of demyelinating disease of central nervous system which mainly affect optic nerve and spinal cord. Because of its serious blindness and disability, how to effectively prevent relapse has become the focus of ophthalmologists. With the deep understanding of the pathogenesis and the progress of scientific and technological means, more and more monoclonal antibodies(mAb) continue to enter clinical trials. B cell surface antigen CD20 blocker, rituximab, has become a first-line drug for the treatment of NMOSD. CD19 blocker, inebilizumab, can reduce the recurrence and disability of NMOSD patients. The addition of interleukin 6 receptor blocker, satralizumab, and complement C5 inhibitor, eculizumab, reduce the recurrence. Some mAbs such as natalizumab and alemtuzumab may not be effective for the treatment of NMOSD. The expansion of mAb treatment indications and the launch of new drugs still require more clinical trials which are large-scale and international cooperation. At the same time, its potential adverse events and cost issues cannot be ignored.
Neuromyelitis optica (NMO) is a kind of demyelinating disorder that preferentially affects the optic nerves and spinal cord and results in permanent vision loss. NMO pathogenesis is thought to involve binding of anti-aquaporin-4 (AQP4) auto-antibodies to astrocytes, which causes complement-dependent cytotoxicity (CDC) and downstream inflammation leading to oligodendrocyte and neuronal injury. Vasculocentric deposition of activated complement is a prominent feature of NMO pathology. In recent years, a number of groups have found complements play an important role in the pathogenesis of NMO, and basic researches in NMO therapy due to its specificity and uniformity. Its inhibition would protect against proteins in the classical complement pathway so that cure the disease. This review will expound the the role of complement signaling pathway in the pathogenesis of NMO, and provide reference for a more in-depth understanding and clinical treatments of NMO.
Neuro-ophthalmology is an interdisciplinary discipline that spans multiple disciplines such as ophthalmology, neurology, and neurosurgery, and plays an important role in ophthalmology. Since the establishment of the Neuro-Ophthalmology Group in 2011, it has shown a good momentum of accelerated development of neuroophthalmology team building, promoting the process of neuroophthalmology standardization and strengthening interdisciplinary cooperation. However, there is still a gap between the level of neuro-ophthalmology in China and that in developed countries, which still cannot get rid of the stage of introduction and imitation. It is still a long way to form neuro-ophthalmology with unique characteristics in China. In the future, we should carry out clinical research while advancing basic research work, and establish a standard training system to strengthen the training of neuroophthalmologists. It is expected that in the next 10 years, China's neuro-ophthalmic diagnosis and research level will be significantly improved, and a number of landmark academic achievements will be achieved, and a number of internationally and domestically renowned neuro-ophthalmologist will be trained to further enhance the international academic status of neuro-ophthalmic research in China.
Plasma exchange (PE) is a therapeutic blood component replacement method. The blood of patients is first separated into plasma and blood cell components using a blood cell separator in vitro, the plasma containing harmful pathogenic substances is then discarded and replaced with the same volume of exchange solution. Finally the separated blood cells together with the exchange solution are returned back to the blood circulation of patients. By reducing the circulating antibodies, abnormal plasma proteins or cytokines and other pathogenic molecules, PE can block the disease process. PE has a good therapeutic effect on neuromyelitis optica-related optic neuritis (NMO-ON), which shows resistant to glucocorticoid therapy for the first onset. The American Society for Apheresis guideline evaluates PE for acute optic neuritis as a recommended grade 1B, type II indication. In the implementation of PE treatment for NMO-ON and other diseases, indications and contraindications should be strictly adhered to the guideline, treatment procedures and protocols should be optimized, common adverse events and its prevention and management should be known and alerted. It is important to conduct multi-center clinical cooperation and a high standard clinical randomized controlled study, to find out the optimal time window, the best protocol, and the associated factors for the efficacy and prognosis of PE in NMO-ON.
Idiopathic intracranial hypertension (IIH) is a neurological disorder characterized by an unexplained increase in intracranial pressure that primarily affects obese women of childbearing age, but individuals of any age, gender, or weight may also be affected. Its signature symptoms include disc edema, headache, visual disturbance, and throbbing tinnitus. Due to potentially serious complications, such as vision loss, accurate diagnosis and appropriate treatment management are critical to improving patients' quality of life. Ophthalmologists play a key role in the treatment process, as about half of patients first visit the eye department. Diagnosis of IIH depends not only on clinical presentation, but also on the exclusion of other diseases that may cause similar symptoms, and imaging and other tests to ensure an accurate diagnosis. In order to improve diagnostic accuracy and treatment efficiency, multidisciplinary collaborative diagnosis and treatment mode is advocated, especially in the face of patients with complex trauma or systemic diseases, which can effectively shorten the treatment time and ensure patient safety. Future research directions include establishing China's IIH epidemiological database, exploring clinical diagnosis and treatment methods and basic scientific research, aiming at forming diagnosis and treatment standards suitable for China's national conditions, improving medical level and improving patient prognosis. At the same time, a deeper understanding of the different forms of IIH will better serve the affected populations.
ObjectiveTo evaluate the effectiveness and safety of glucocorticoids in the treatment of non-arteritic anterior ischaemic optic neuropathy (NAION).MethodsGlucocorticoids published in the National Library of Medicine PubMed; Netherlands Medical Abstracts Database Embase; Cochrane Library, an evidence-based medical library; China Cnkipedia; China Biomedical Literature Service; Chongqing Vipul Chinese Science and Technology Journal Database, and Wanfang Science and Technology Journal Full Text Database were searched about computer. Randomized controlled trials (RCTs) and non-randomized controlled trials (NRCTs) for the treatments of NAION were subjected to meta-analysis. The search period was from the establishment of each database to March 2020. The literature was screened and data were extracted according to the inclusion and exclusion criteria. The methodological quality of the RCT and NRCT studies was evaluated using the Risk of Bias Assessment Tool and the MINORS evaluation scale, respectively. The data were analyzed using RevMan version 5.3 software which was provided by the Cochrane Collaboration Network.ResultsAn initial search of 395 papers was conducted, and 10 papers were finally included for this meta-analysis, including 3 RCT studies and 7 NRCT studies. A total of 1057 patients with NAION were included. The 3 RCT studies were analyzed descriptively as the outcome indicators were described in different ways. A meta-analysis of 7 NRCT studies showed that patients in the treatment group showed significantly better visual prognosis (relative risk=1.28, 95% confidence interval 1.09 to 1.51, P=0.003) and retinal nerve fibre layer thickness were obviously improved (mean difference=7.76, 95% confidence interval 1.58 to 13.94, P=0.01) than the control group. Four studies reported the occurrence of adverse reactions in the treatment versus control groups. None of the above studies provided a detailed analysis of the prognosis of patients with adverse reactions.ConclusionThe efficacy and safety of glucocorticoids in the treatment of NAION is unclear and needs to be validated in a larger sample of RCTs.