Idiopathic intracranial hypertension (IIH) is a neurological disease, characterized by increased intracranial pressure and papilledema, and often associated with headache, transient loss of vision and pulsatile tinnitus. IIH typically occurs in women of childbearing age. Over 90.0% of patients are with obesity or over weighted. Loss of sensory visual function is the major morbidity associated with IIH and some patients even develop into blindness. Most patients will have varied degrees of visual impairment, or even a few become blind. Frisén grading system, visual field examination and spectral-domain optical coherence tomography can be used to evaluate and monitor the IIH papilledema functionally and morphologically. In recent years, IIH treatment trials in other countries confirmed that, weight loss and low-salt diet combined with acetazolamide treatment has a clear improvement for IIH patients with mild visual impairment. In-depth understanding of the etiology, clinical manifestations, diagnostic criteria and the main treatment has important clinical significance for IIH patients
Infectious and infection-related optic neuritis is an important type of optic neuritis. Infectious optic neuritis is caused by direct spread of pathogenic organism to optic nerve from local infection or blood transmission. Infection-related optic neuritis is caused by pathogens-induced immune allergic reaction. They present with atypical clinical features of optic neuritis, including progressive vision loss, persistent eye pain or headache, ineffectiveness or even worse of glucocorticoid therapy. Fundus manifestations include optic disc swelling with peripapillary hemorrhage or neuro-retinitis, and the feature of concurrent uveitis. When these patients first visit ophthalmic clinics, they often lack signs of systemic infection, thus it is easy to misdiagnose them as other types of optic neuropathy and mistakenly treat them. In particular, high-dose glucocorticoid therapy can lead to very serious consequences. Therefore, how to correctly diagnose infectious and infection-related optic neuritis in the early stages are very important for ophthalmologists and need to be seriously kept in our mind.
Commonly clinical visual electrophysiological detection methods include flash ERG, pattern ERG, VEP and multifocal ERG, etc. These inspection methods play an important role in disease identification and visual function judgment. However, lacking of understand the basic principles of electrophysiological testing, misunderstandings of the clinician influence the choice of examination methods or interpretation of examination results. The understanding of the basic principles of electrophysiological testing and analysis of the causes of these misunderstandings have important clinical significance in the correct application of visual electrophysiological testing technology.
Optic nerve glioma (ONG) is a rare central nervous system tumor that occurs in children and adolescents. It’s main pathological type is low-grade pilocytic astrocytoma. It is divided into sporadic ONG and neurofibromatosis type 1 (NF-1) related ONG. Due to the close relationship between ONG and the optic nerve, there is its particularity in diagnosis and treatment. The diagnosis of ONG mainly relies on medical history, symptoms and signs, as well as imaging examinations such as MRI and CT. ONG should be differentiated from optic nerve sheath meningioma, optic neuritis, optic nerve metastasis and other diseases. In recent years, newly discovered molecular targeted therapy and anti-vascular endothelial growth factor drugs are a powerful supplement to ONG. When chemotherapy is not sensitive or resistant, radiotherapy can be considered, but it is only recommended for patients over 7 years of age. Surgery can be considered when the patient’s visual impairment is severe and the appearance of the eye is significantly affected. In addition, due to the susceptibility of NF-1 patients to tumors, the chemotherapy regimen should take into account the risk of secondary leukemia caused by the drug, and the timing of radiotherapy should be after the age of 10. We look forward to further ONG clinical research, which will bring more references for future clinical work.
Optic nerve sheath meningiomas (ONSM) account for about 1%-2% of all meningiomas. Most of them are benign and the disease progresses slowly. ONSM is a relatively rare intraorbital benign tumor in clinical practice. Due to its close relationship with the optic nerve, the diagnosis and treatment are special, so there are many controversies and misunderstandings in the diagnosis and treatment of ONSM. ONSM is often misdiagnosed as acute optic nerve papillitis, optic nerve atrophy, ischemic optic neuropathy, acute retrobolic optic neuritis, optic disc vasculitis or optic fatigue due to its similar clinical features to other optic nerve diseases such as optic neuritis. The treatment includes observation, surgical treatment and radiotherapy, and appropriate treatment should be selected for different patients according to the changes of their condition. Therefore, understanding of the controversies and misunderstandings in the diagnosis and treatment of ONSM is of great clinical significance for timely and accurate diagnosis, appropriate treatment and improvement of the prognosis of patients.
Leber's hereditary optic neuropathy (LHON) is a rare hereditary optic nerve disease. At present, the understanding of its etiology and pathogenesis is relatively clear. With the emergence of new drugs such as idebenone and the possibility of gene therapy for LHON, it has brought hope for patients to recover. However, because genetic testing technology has not been widely developed in China, clinical misdiagnosis of LHON as optic neuritis still occurs from time to time. How to make timely identification and correct diagnosis of LHON still poses certain challenges for Chinese ophthalmologists. In addition, in terms of treatment, the choice of treatment methods and treatment costs in the pre-onset (gene mutation carriers) and different periods after the onset of LHON are also huge challenges for patients and their families.
Autoimmune retinopathy (AIR) is an umbrella term for a group of rare autoimmune retinal degenerative disease presumably caused by cross-reactivity of serum autoantibodies directed against ratinal antigens, and include cancer-associated retinopathy, melanoma-associated retinopathy and non-paraneoplastic autoimmune retinopathy. Common feature of AIR include progressively painless vision loss with abnormal electrophysiology responses associated with positive anti-retinal antibodies. They present a clinical diagnosis challenge on account of the rare incidence, unobvious clinical symptoms and lack of specific and sensitive biological markers. Early diagnosis and treatment may play a critical role to avoid the irreversible immunological retinal damage.
Neuromyelitis optica spectrum disorders (NMOSDs) are a class of immune-mediated inflammatory demyelinating diseases of the central nervous system that mainly involve the optic nerve and spinal cord. As an important environmental factor, the gut microbiota may play an important role in the occurrence and development of NMOSDs. Previous studies have shown that the structure and number of intestinal flora in NMOSDs patients are different from those of normal healthy people. The altered intestinal flora may cross-react with central nervous system autoantigens, induce T cell differentiation, and affect short-chain fatty acids, etc. The metabolite secretion pathway triggers the occurrence of NMOSDs. The summary of the changes of gut microbiota in patients with NMOSDs and the possible underlying mechanisms by summarizing the literature, aim to provide more effective treatments for the prevention and treatment of NMOSDs in the future.
Tumor immunotherapy includes immune checkpoint inhibitor (ICI), tumor vaccines, and adoptive cell therapy. Immunotherapy, as the main systemic treatment for advanced malignant tumors, kills tumor cells by activating the immune system and prolongs the survival of patients. However, excessive immune responses can cause immune-related adverse events (irAE), causing damage to systemic tissues. ICI are the main tumor immunotherapy drugs that cause optic nerve irAE. The most common optic nerve irAE are optic neuritis, only a few patients appeared arteritic anterior ischemic optic neuropathy. Sudden painless loss of bilateral vision is the most common clinical manifestation. In severe cases, the vision decrease to no light perception. Early diagnosis and early adequate glucocorticoid treatment can improve the symptoms. Therefore, neuro-ophthalmologists and oncologists should know the clinical characteristics of optic nerve irAE, in order to diagnose and treat early and improve the prognosis.
ObjectiveTo observe the clinical characteristics of patients with visual impairment caused by fungal sphenoid sinusitis and analyze the influencing factors related to visual prognosis. Methods A retrospective clinical study. From January 2006 to December 2020, 44 patients (55 eyes) with visual impairment caused by fungal sphenoid sinusitis confirmed by imaging and pathological examination in the Department of Ophthalmology of Beijing Tongren Hospital were included in the study. Patients was first diagnosed in the Department of Ophthalmology due to monocular or binocular vision loss, or binocular diplopia, limited eye movement and ptosis. All patients underwent visual acuity examination and fundus color photography. CT examination of paranasal sinus or orbit was performed in 37 cases; magnetic resonance imaging (MRI) of paranasal sinus, brain or orbit was performed in 34 cases. All patients underwent endoscopic sinus opening combined with intrasinus lesion clearance; 14 cases were treated with antifungal drugs after operation. The average follow-up time was 59.61±37.70 months. Comparison of clinical characteristics between invasive and non-invasive fungal sphenoid sinusitis were by χ2 test or Fisher exact test. The influencing factors with P＜0.2 in univariate analysis were selected for multivariate regression analysis. ResultsAmong the 44 patients, there were 19 males and 25 females; the ratio of male to female was 1:1.3; the average age of visual symptoms was 61.48 ± 12.17 years; 23 cases (52.3%, 23/44) suffered from immune dysfunction, including 21 cases of diabetes mellitus. The visual acuity decreased in 33 cases (44 eyes) (75.0%, 33/44). There were 15 cases of binocular diplopia with eye movement disorder (34.0%, 15/44), including 6 cases with visual impairment. The visual acuity of the affected eye was no light perception-0.8. There were 35 cases with headache (79.5%, 35/44). Nasal symptoms were found in 14 cases (31.8%, 14/44). There were 40 and 4 cases of Aspergillus and Mucor infection in sphenoid sinus, respectively. Among the 37 cases who underwent CT examination of paranasal sinus or orbit, there were soft tissue filling in the sinus cavity, including 19 cases of high-density calcification in the sinus cavity (51.4%, 19/37); bone defect of sinus wall were in 24 cases (64.9%, 24/37). There were 26 cases (70.3%, 26/37) of sinus wall osteosclerosis. MRI of paranasal sinus, brain or orbit was performed in 34 cases. T1WI of sphenoid sinus lesions showed low signal, high signal and equal signal in 14, 10 and 9 cases, respectively; T2WI showed high signal, low signal and equal signal in 13, 16 and 2 cases respectively. After enhancement, the lesions were strengthened in 11 cases, no obvious enhancement in 23 cases, and the surrounding mucosa was thickened and strengthened. The lesions involved the orbital apex and cavernous sinus in 18 and 16 cases, respectively; orbital apex and cavernous sinus were involved in 12 cases. Six months after operation, visual acuity was significantly improved in 27 eyes (65.9%, 27/41); visual acuity did not improve in 14 eyes (34.1%, 14/41). Multivariate regression analysis showed that the change of sinus wall osteosclerosis was associated with higher visual acuity improvement rate (odds ratio= 0.089, 95% confidence interval 0.015-0.529, P=0.008). ConclusionsFungal sphenoid sinusitis related visual impairment is relatively common in elderly female patients with low immune function; monocular vision loss with persistent headache is the most common clinical symptom; imaging findings of sphenoid sinus lesions are an important basis for diagnosis. Sphenoid sinus opening combined with sinus lesion clearance is an effective treatment. After operation, the visual acuity of most patients can be improved. The prognosis of visual acuity was relatively good in patients with hyperplasia and sclerosis of sphenoid sinus wall bone.