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.
Optic neuritis (ON) is one of the symptom of a central nervous system demyelinating, systemic or infectious disease. The pathogenetic mechanism of ON is still not completely clear, and its core is inflammation and immune that occurred in the optic nerve axons, and apoptosis of RGC was induced. Few patients experience recurrent episodes after treatment, presenting a remission - recurrence course of polyphasic disease, named recurrent ON (RON). Two forms of RON have been assigned: recurrent isolated optic neuritis, which is a chronic corticosteroid-dependent optic neuropathy with intermittent acute relapses, and recurrent isolated optic neuritis, which is a non-progressive relapsing ON without steroid dependence. Recurrence of ON causes cumulative damage to the optic nerve lesions and impaired visual signal transmission, thus causing irreversible damage to vision. Therefore, it is very important to have a deep understanding of the pathogenesis of ON and the clinical characteristics of RON, so as to better conduct clinical treatment.
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.
Objective To detect the color damage in patients with idiopathic optical neuritis (ION) after the treatment.Methods A total of 26 ION patients (44 eyes) with ION whose visual acuity were above 1.0 were collected. All the patients had undergone the treatment of incretion and had the visual acuity more than 1.0 after the treatment.The results of MRI and blood examination were normal. Another 24 healthy persons were selected as the normal control. Total error scores (TES) and each error score of red, green and blue were measured via Farnsworth Munsell100 hue tester. The TES origin scores and their square roots were used for a statistical analysis. The results of the two groups were compared.Results There weresignificant differences in TES and its square roots between ION group and the normal control group (t=3.079，3.133；P=0.0033，0.0026).The differences in the level of error scores of each color between the tow groups were not significant (t=1.91,1.15,1.62; P=0.061,0.26,0.11);but the differences in the square roots of red color between the two groups were statistically(t=2.21,P=0.031).Conclusion After the treatment,the visual acuity of ION patients increases,but the color damage still exist; red color damage happens first.
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.
Purpose To investigate mitochondrial DNA (mt-DNA) mutations in optic neuritis of unknow reason (ONUR) and to assess the pathogenic and differential diagnostic values of screening for mt-DNA mutations in ONUR. Method Thirty patients with ONUR were screened for mt-DNA mutations by using SSCP,mutation-specific primer PCR and sequencing. Results mt-DNA mutations were found in 12 out of the thirty patients.All of the mutations were at 11778 position,but no one at 3460 and 15257. Conclusions Quite a number of patients (12/30,40%) with ONUR were caused actually by mt-DNA mutation．Screening for mt-DNA mutation in these patients has a pathogenic and differential diagnostic significance. (Chin J Ocul Fundus Dis,2000,16:78-79)
Objective To observe the results of function MRI and perimetry in patients with visual pathway diseases.Methods Three patients (6 eyes) with pituitary adenoma and craniopharyngioma diagnosed via pathological examination and three healthy volunteers aged from 24 to 30 were collected. The best corrected visual acuity was nonlight perception1.0 in the 6 sick eyes and 1.0 in the healthy eyes; all the involved individuals had no other ocular diseases except myopia and without any contraindications of MRI. Common tests including the best visual acuity, fundus test by direct or indirect ophthalmoscope, center static visual field tested by Octopus 101 perimeter, program 32, tendency oriented perimetry were performed. The visual stimulation subtended a field of view of about 12 degrees，consisted of high contrast and drifting checkerboards. MRI parameters: GE signa VH/i 30T scanner. Functional data: GRE-EPI sequence，20 slices lying perpendicular to the calcarine sulcus. Anatomical data was obtained using 3DSPGR sequence to acquire high resolution. The cortical surface was unfolded and then cut and inflated. Functional data was presented to the inflated surface and subsequently analyzed by AFNI software.Results In six eyes, three had temporal defects, two had upper temporal visual field defects, and the other one did not finish the visual field test. The retinotopic representations of health adults were obtained by using the phaseencoded visual stimulation. The Eccentricity coordinate maps showed that foveal representations lay in the occipital poles and the representations appeared further anterior as eccentricity increased. The polar angle coordinate maps showed that early retinotopically organized areas had a representation of visual field. The visual cortex beneath the calcarine sulcus matched with the upper visual field of the opposite side and which upon the calcarine sulcus matched with the under visual field of the opposite side. Less or no visual cortex response was revealed in the patients′ function MRI or the response in injury side was vanished. The visual cortex response related with the visual field defects could not be induced in function MRI.Conclusion There is a good correlation between function MRI data and the results of perimetric evaluation. The function MRI can show the visual cortex response correlated with the visual field defects of the patients with visual pathway diseases.
Objective To observe the clinical characteristics of demyelinating optic neuritis (DON) in Chinese children under the age of 16. Methods A retrospective review of the medical charts of 42 pediatric patients with DON was conducted in this study. Twenty-two patients (52.4%) were male, and 20 patients (47.6%) were female. The patients aged from 3 to 15 years, with the mean age of (9.5±2.3) years. There were 35 bilateral patients and 7 unilateral patients. Twenty-seven patients (64.3%) had prodromal symptoms before onset. All patients underwent visual function and imaging tests, such as best corrected visual acuity (BCVA), fundus photography, visual evoked potential (VEP), visual field, MRI. The patients were tested for serum levels of antibodies for aquaporin 4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) with a cell-based assay. All patients were received corticosteroid therapy. The mean follow-up was (1.17±0.42) years. The children who had coordination ability and with BCVA≥0.3 were received examination of Humphery automatic perimeter. Data were collected on the age, gender, clinical features, neuroimaging, serological specific antibodies, treatment and vision prognosis. Results 23.8% of the children were bilateral optic neuritis in onset stages. 64.2% were recurrent optic neuritis and 83.3% exhibited bilateral diseases eventually. BCVA had decreased to ≤0.1 in 87.0%% eyes and disc swelling was observed in 77.9% eyes during the onset stages. All eyes had visual field defects and abnormal VEP exam results, with delayed latency of P100 and P2, and varying degrees of amplitude reduction. Serum AQP4 antibody and MOG antibody were tested by cell-based assay, 2/42 children (4.7%) were positive for AQP4 antibody and 5/24 children (20.8%) were positive for MOG antibody. All of anti-AQP4+ and anti- MOG+ cases relapsed. All children underwent orbital magnetic resonance imaging (MRI), 40 cases (95.2%) showed demyelination features of optic nerve, and 5 cases (11.9%) showed long segments lesion (more than 1/2 length of the optic nerve). There were 2 anti-AQP4+ cases and 3 anti- MOG+ cases from the 5 cases with long segments lesion. MRI also showed brain demyelinating lesions in 4 children (3 of them were anti- MOG+) or spinal cord demyelinating lesions in 3 children (2 of them were anti- MOG+). After treatment with glucocorticoid, visual acuity improved in all eyes, of which 84.4% with BCVA≥0.5. Forty-eight eyes of 26 children accept dynamic visual field during the course of treatment, showed the vision abnormalities associated with optic nerve damage. Conclusions Children under the age of 16 with DON can experience severe visual impairment, higher recurrence tendencies, and higher rate of disc involvement, but good response to glucocorticoid therapy. AQP4 or MOG antibodies positive might be concurrent with brain and (or) spinal cord demyelinating lesions and indicated a poorer prognosis.
Objective To compare the differences of optic disc morphology and optic nerve head parameters between Vogt-Koyanagi-Harada (VKH) syndrome and optic neuritis (ON) with optic disc edema. Methods This is a retrospective study including 21 first-onset VKH patients (35 eyes) as VKH group and 22 first-onset ON patients with optic disc edema (27 eyes) as ON group. The differences of age (t=−1.11) and gender (χ2=0.20) between two groups were not significant (P＞0.05). Sixty-two eyes of 43 age and gender-matched healthy subjects were enrolled in this study as control group. All subjects underwent three dimensional optical coherence tomography (3D-OCT) examinations. The difference of optic disc morphology between two groups was observed. The parameters included average thickness of entire circumpapillary retinal nerve fiber layer (CP-RNFL), thickness of nasal, superior, temporal and inferior quadrant of CP-RNFL, disc area, disc cup area, rim area, cup/disc (C/D) area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio. The disc retinal pigment epithelium (RPE) angle was observed too. Results Among 35 eyes in VKH group, 31 eyes (88.57%) had retinal detachment next to the disc, 3 eyes (8.57%) had serrated inner limiting membranes of the disc. Twenty eyes (64.52%) had highly reflective points, lines, or membrane-like structures in the retinal detachment areas. No such signs appeared in ON patients. Compared with ON group, the optic cup area, C/D area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio were bigger, the thickness of mean CP-RNFL and the superior, inferior quadrants of CP-RNFL were thinner, disc RPE angles was smaller in VKH group (P＜0.05). Compared with control group, the disc area, optic cup area, rim area were bigger, C/D vertical diameter ratio was smaller, the mean CP-RNFL and 4 quadrants CP-RNFL were thicker, disc RPE angles was smaller in VKH group (P＜0.05); the disc area, optic cup area, C/D area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio were smaller, the mean CP-RNFL and 4 quadrants CP-RNFL were thicker, disc RPE angles was bigger in VKH group (P＜0.05). Conclusions VKH patients have smaller disc RPE angles and more chance to develop retinal detachment next to disc than ON patients. The C/D area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio are bigger, the mean CP-RNFL and the superior, inferior quadrants of CP-RNFL are thinner in VKH eyes.
Objective To investigate the etiological distribution of the patients with optic neuritis in China and compare the results with those in western countries. Methods Ophthalmological and neurological detailed clinical and laboratorial examinations were performed on 204 patients with primarily diagnosed optic neuritis (ON). We determined the etiologies using international accepted diagnostic criteria. Results Among 113 patrents with ON, 83(73.5%) were considered as with idiopathic demyelinating optic neuritis ( IDON). Sinusitis was common in these patients but was considered to be the probable cause of ON only in 4. Tuberculo-meningitis caused ON was found in 2 cases and syphilitic ON in 1. The causes of 23 cases (20.4%) were unknown. Conclusions Idiopathic demyelinating ON is the most common pathogeny of ON. Despite of some minor differences of causes and prognosis, the etiology of presumed ON in our population is similar to that reported in western countries. (Chin J Ocul Fundus Dis,2006,22:367-369)