Ras homolog family (Rho)/ Rho-associated coiled-coil kinase (ROCK) signaling pathway widely exists in human and mammal cells, which is closely related to inhibition of repair after optic nerve damage. The expression level of Rho/ROCK signaling pathway-related proteins is up-regulated in glaucoma, and related with the death of retinal ganglionic cell (RGC) and the axon activity. ROCK inhibitors can protect the surviving RGC and promote axon extension with a dose-dependent manner. ROCK inhibitors also can inhibit glial scar formation, lower intraocular pressure and inhibit inflammatory response to some degrees. Rho/ROCK signaling pathway correlates with the optic nerve disease progression, and ROCK inhibitors hope to become a new therapeutic drug.
Objective To observe the clinical manifestations and the effect factors of thyroid-associated ophthalmopath (TAO) with optic neuropathy. Methods The clinical data of 62 cases (120 eyes) of TAO with optic neuropathy diagnosed in 1994-2001 were retrospectively analyzed. Results The incidence of TAO with optic neuropathy was 18.3% in all the simultaneous TAO inpatients, and was more frequently found in the male than in the female ones. The incidence of TAO with optic neuropathy was correlated with orbitono-increase, ocular myopathy, hypertension, hyperlipemia, diabetes, and cardiopathy (P＜0.0000), but not with exophthalmos, intraocular pressure, and the disease course of TAO (Pgt;0.05). In 62 patients with the methylprednisolone pulse therapy, the visual acuity improved in 33 (29.0%), improved while the treatment but decreased after stop treating in 29 (24.2%), and no changes in 58 (46.8%). In 63 eyes undergone orbital decompression with methylprednisolone pulse therapy, 59 (93.7%) had better visual acuity. Conclusion The incidence of TAO with optic neuropathy was correlated with orbitono-increase, ocular myopathy, hypertension, hyperlipemia, diabetes, and cardiopathy. Met hylprednisolone pulse therapy and orbital decompression are the effective measures for TAO with optic neuropathy. (Chin J Ocul Fundus Dis，2004，20：142-144)
Objective To analyze the new primary mutation in Chinese people with Leberprime;s hereditary optic neuropathy (LHON). Methods Genomic DNA was collected from 260 suspected LHON patients and 100 normal healthy persons. The mitochondria DNA mutation at nucleotide position (NP) 15257 and the hot spot (14452-14601 bp) of ND6 gene which include the mutations at NP (14482, 14498, 14568, 14596, 14495, and 14459) were screened by using polymerase chain reaction (PCR), heteroduplex-single strand conformation polymorphism (HA-SSCP) and restriction fragment length polymorphism (RFLP) analysis and sequencing. Primary mutation spectrum of Chinese race was analyzed. Results Eight kinds of polymorphism of mitochondria DNA were found in 260 suspected LHON patients and 100 normal healthy persons, including NP 14488C, 14518G, and 14617G which hadnrsquo;t been reported (http://www.mitomap.org/). No mutation at NP 15257, 14482, 14498, 14568, 14596, 14495, and 14459 was found. Conclusion The NP 15257A may not be the primary mutation in Chinese. Because of the race difference, 14452-14601 bp in ND6 gene may not be the hot spot in Chinese patients with LHON, and other hot spots may exist. (Chin J Ocul Fundus Dis, 2006, 22: 82-85)
Objective To observe the characteristics of changes of 24hour ambulatory blood pressure and heart rate of 50 patients with anterior ischemic optic neuropathy (AION). Methods Fifty patients with AION and the persons without in the control group, which had the same number, gender and age as the patients with AION, underwent 24-hour ambulatory blood pressure and heart rate measurement. Results Both groups had no difference in mean blood pressure and heart rate during the daytime (t=1.25,0.93; P＞0.05), higher than those in the nighttime (t=3.63,3.16; P＜0.05). Mean blood pressure and heart rate of AION group at night were lower than those of the control group (t=3.82,1.77; P＜0.01,0.05), especially diastolic pressure of AION group was lower than that of the control group from 2 am to 7 am (P＜0.01), as well as the heart rate from 2 am to 5 am (P＜0.05 or P＜0.01). The curves of blood pressure of AION group showed more gradual and fluctuant rising, while those of the control group showed sharper and less fluctuant rising. Conclusion According to the curves of blood pressure rising, the patients with AION may have some defects in auto-regulatory mechanism of blood pressure. The low spots of blood pressure and heart rate in early morning, which might be a critical point leading to AION. (Chin J Ocul Fundus Dis, 2002, 18: 259-261)
Objective To investigate the major types and clinical manifestations of mitochondrial DNA (mtDNA)mutations in Chinese patients with Leber′s hereditary optic neuropathy(LHON). Methods A total of 119 patients with bilateral optic neuropathy from 117 pedigrees, including 37 with determinate diagnosis of LHON(group A) and 82 with suspected LHON(group B),were tested for mtDNA mutations by using single-strand conformational polymorphism, mutation-specific primer polymerase chain reaction and sequencing. Pertinent clinical data and history of the patients with the 11778 mutation were collected. Results Nucleotide positions(np)11778 mutation and np 14484 mutation was found in 33 (89.2%) and 3 (8.1%) patients respectively in group A, while np 11778 mutation was obtained in 26（31.7%）in group B. No 3460 mutation was found in group A or B. The clinical manifestations of 59 patients with np 11778 mutation were as follows: acute or chronic visual loss，no ophthalmalgia, the age of onset of 10-25, and either a central or paracentral scotoma in perimetry. The visual recovery rate was 8.6%～11.6%. Conclusion Chinese patients with LHON have a very high incidence of np 11778 mutation and the clinical manifestations of the patients with np 11778 mutation are similar to those of Caucasian patients. (Chin J Ocul Fundus Dis，2004，20：78-80)
The diameters of the optic nerves in 54 patients with high intracranial pressure(ICP)were checked and measured with B type ultrasonic tomography and the relationship between thewidth of optic nerve and the optic papiliedema was studied. The results showed that the average diameter (6. 324-0.53)mm of the optic nerves in patients with high ICP was wider than that(3.61 ~:0.29 )mm in health subjects (P(0.01). The rate of increasing width of optic nerve (87.00%)was higher than that (67.00~)of papilledema (P(0.05). In addition,in 19 patients with ICP between 1.97 and 2.50 kPa, the rate of increasing width of optic nerve (79. 00~)was higher than that (42.00%)of papilledema (P (0. 05). These results indicated that measuring the diameter of optic nerve might be more practical than observing tile presence of papilledema in diagnosing high ICP,especially in early stage. （Chin J Ocul Fundus Dis,1996,12: 86-87）
ObjectiveTo observe the clinical features and visual prognostic factors of ethambutol-induced optic neuropathy (EON).MethodsA cohort study. Twenty-four inpatients (46 eyes) identified as EON in Neuro-Ophthalmology Department of Chinese PLA General Hospital from January 2014 to December 2017 were enrolled, including 14 males (26 eyes) and 10 females (20 eyes) with a ratio of 1.4/1 male/female. The average age was 42.79±15.12 years and the average weight was 62.46±12.31 kg. The average time duration between oral administration of ethambutol and occurrence of EON was 9.94±16.49 months. The average time of ethambutol duration was 7.06±11.68 months, with an average accumulative dose of 156.7±1 779.0 g and the average daily dose of 15.07±8.95 mg/(kg·d). All patients were tested with visual acuity, fundus photos, colour vision, OCT, visual field, VEP, orbital MRI and the gene of OPA1 and mitochondrial deoxyribonucleic acid (mtDNA). All the patients accepted drug withdrawal immediately after diagnosis, and were given the treatment of systemic nerve nutrition and improvement of microcirculation for 2 weeks. The time of follow-up was more than 12 months. According to whether the visual acuity (VA) in any of eyes was over than 0.1 at the last follow-up, all the patients were divided into two groups: the bad VA group (VA less than or equal to 0.1) and the better VA group (VA over than 0.1) group. The χ2 test and Fisher's exact probabilistic method test were used to compare the counting data between groups, and the Wlincox rank sum test was used to compare the measurement data. Multiple factors of VA outcome between the patients with bad or better va were analyzed by logistic regression.ResultsThirty eyes (65.2%) had VA less than or equal to 0.1 and 5 eyes (10.9%) had VA over than 0.5 at EON onset. The VA of the rest 11 eyes (23.9%) was higher than 0.1 and lower than 0.5. At the last follow-up, 20 eyes (43.5%) had VA less than or equal to 0.1 and 9 eyes (19.6%) had VA over than 0.5, the VA of the rest 17 eyes (36.9%) was higher than 0.1 and lower than 0.5. Fundus examination revealed 7 eyes (15.3%) with optic disc edema. OCT revealed significant loss of the retinal nerve fiber layer (RNFL) in the affected eyes, mainly in the temporal RNFL of the optic disc. All patients had dyschromasia, mainly in distinguishing the color of red and green. The types of visual field defect was as following: central dark spot (52.2%), diffuse visual acuity decreased (30.4%), temporal hemianopsia (17.4%). Orbital MRI revealed that 12/24 (50.0%) patients had T2 lesions with T1 enhancement in 6/24 patients (25.0%). Genetic test showed that 4 patients (16.7%) had gene mutation. Among them, there were 2 patients with OPA1 mutation, 1 with mtDNA 14340 point mutation and 1 with the mtDNA 11778 point mutation. Thirteen patients showed better VA outcomes (over than 0.1) while 11 showed bad VA outcomes after discontinuation of ethambutol. Between the better VA group and the bad VA group, there were statistically significant differences in the daily dose of ethambutol and gene mutation (P=0.031, 0.023). The daily dose was related to visual prognosis of EON while only the daily dose of more than 18 mg/(kg·d) may lead to bad VA outcomes according to the logistic analysis (95% CI 0.007-0.736, OR=0.069, P=0.027).ConclusionsEON may have OPA1 and mtDNA mutation with more bilateral eyes involved and less optic edema, which about 43.5% of the patients showed irreversible visual impact. The daily dose of ethambutol is related to the vision recovery.
Objective To study prospectively the morphological relations among hemorrhage of optic disc, defect of retinal nerve fiber layer, and peripapillary atrophy in normal-tension glaucoma (NTG). Methods Qualitative and quantitative methods were applied to evaluate the relations among hemorrhage of optic disc, defect of retinal nerve fiber layer, and peripapillary atrophy by using stereo ocular fundus photography and computer analyzer systems. Results In 37 patients (42 eyes) in NTG hemorrhage group, 50 hemorrhagic spots at optic disc was found, including coloboma of retinal nerve fiber layer in 35 eyes (83.3%)(35/42). In 35 patients (40 eyes) in non-hemorrahge group, partial coloboma of retinal nerve fiber layer was found in 21 eyes (52.5%)(21/40). The difference of incidence of coloboma of retinal nerve fiber layer between the two groups was not significant (chi;2=1.403, P=0.236). The incidence of atrophic arc in hemorrhage group differed much from which in non-hemorrhage group (chi;2=7.008, P=0.008). The area of atrophic arc at beta;section in hemorrhage group ［(2.05plusmn;0.88)mm2］ was significantly different from which in non-hemorrhage group［(1.42plusmn;0.53)mm2］(t=-2.618, P=0.012). In the follow-up period, the difference of incidence of disc-rim loss between hemorrhage and non-hemorrhage group was significant, (chi;2=5.802, P=0.016); while the difference of the incidence of visual field defect between the two groups was not. Conclusion In eyes with NTG, hemorrhage of optic disc, coloboma of retinal nerve fiber layer and atrophic arc are closely related. More disc-rim loss and changes of atrophic-arc area in hemorrhage group in the follow-up period suggests that hemorrhage of optic disc is the risk factor of development of NTG. (Chin J Ocul Fundus Dis, 2006, 22: 232-235)
Objective To observe the characteristics of indocyanine green angiography and fundus fluorescein angiography (ICGA amp; FFA) in anterior ischemic optic neuropathy ( AION ) , and to investigate the etiology of AION and the value of ICGA and FFA in the diagnosis and study of AION. Methods Simultaneous ICGA and FFA were performed on 32 eyes of 31 AION patients and 38 eyes of 38 non-AION patients. Results The eyes that all or a part of the optic disclies in the watershed zon es of the choroidal blood supply were significantly more in the AION group tha n those in the non-AION group (Plt; 0.005 ) . All the watershed zones through the optic disc were vertical in shape. The fluorescence filling of the inferior and superior choroidal blood vessels near the optic disc were all slower than that of the temporal and nasal choroidal blood vessels. The types of watershed zones in the AION group were highly correspondent to the ischemic areas of the optic disc . In early ICGA , no fluorescence appeared at the optic disc, and in later stage, the ischemic area revealed no fluorescence, and the other area showed mostly asymmetric fluorescence. Conclusion The onset of AION is correlative to the choroidal blood supply around the optic disc. ICGA is more valuable in the diagnosis and study of AION than FFA . (Chin J Ocul Fundus Dis, 2001,17:111-114)
Optical coherence tomography (OCT) can depict mild papilledema and slight changes in the internal segment and external segment; measure thickness of the retinal nerve fiber layer, retinal thickness and macular volume; detect missing axons and damage to the macular ganglion cell complex. Thus, OCT has important application values and widespread prospects in diagnosis and differential diagnosis of glaucoma and nonglaucoma optic neuropathy, optic nerve diseases and macular diseases, outer and inner retinopathy as well as evaluation of curative effects, followup observation, prognosis and mechanisms in neuroophthalmological diseases. Neuro-ophthalmologists should pay more attention to the exploration and application of OCT in the field of neuro-ophthalmology.