Objective To observe the retinal manifestations and classification of mitochondrial encephalomyopathy，and explore the relationship between retinopathy and systemic manifestations.Method The clinical data of 88 inpatients with mitochondrial encephalomyopathy were retrospectively analyzed，in whom 12 patients（24 eyes）with retinal manifestations who diagnosed by ophthalmology consultation and complete medical records were collected. There were nine males and three females aged from 14 to 33 years with the mean age of（20.1±7.0）years. The disease duration ranged from 2.5 to 20 years，with the mean of（9.5±6.8）years. All the patients had the eye symptoms of the different degree，such as limbs weakness，hearing decline and central nervous system symptoms. Ophthalmologic examination including best corrected visual acuity，slit lampa microscope，indirect ophthalmoscopy，noncontact Tonometer，ptosis，ocular movement，pupillary reflex and color fundus photography. Among the patients，three，one，two and five patients had undergone fundus fluorescein angiography（FFA），optical coherence tomography（OCT），lectroretinogram（ERG）and visual field examination respectively. Diabetic retinopathy were divided into “salt and pepper”， retinitis pigmentosa（RP），retinal pigment epithelium（RPE），choroidal capillary atrophy and simplex optic atrophy according to the inspection results.Results All the patients′ both eyes were involved，the disease degree of bilateral eyes was accordant. The ptosis and（or）eye movement limitation were found in nine patients（75.0%），and decreased visual acuity was in six patients（50.0%）.“Salt and pepper” was found in six patients（12 eyes），presenting retinal granular pigmentation and depigmentation；the visual acuity was 0.4-1.2；no central nervous system symptoms were found in patients，such as hearing decline，twitch，ataxia and hypophrenia. RP was found in one patient（two eyes），presenting retinal cells sample pigmentation，retinal vessel shrink，optic atrophy；the vision were light perception in both eyes；hypophrenia，hearing decline，bilateral lower limbs pain and onset twitch were also found in them. RPE and choroidal capillary atrophy were found in three patients（six eyes），the choroidal great vessels and flake pigment accumulation surrounding the retina were observed；the visual acuity was hand movement0.7；limbs weakness was found in two patients；hearing decline was found in three patients；barylalia and hypophrenia were found in two patients；somnolence was found in one patient. Simplex optic atrophy was found in two patients（four eyes）；the vision was 0.1-0.7；central nervous system symptoms were found in patients，such as limbs weakness，twitch，hypophrenia and headache.Conclusion Retinopathy types is concerned with visual prognosis and central nervous system symptoms.
Objective To observe and analysis the features of images of fundus fluorescein angriography (FFA) in low-perfused retinopathy caused by cephalo-cervical peripheral vascular stenosis or occlusion. Methods The results of FFA of 27 patients diagnosed with carotid artery stenosis or occlusion by digital subtraction angiography (DSA) and examination of Doppler and vascular-pulsation were retrospectively analyzed. Result All of the patients had a delayed arm-retinal circulation duration from 20.0 to 81.08 seconds with the mean of 32.1 seconds; a delayed retinal arteriovenous filling duration from 6 to 64.0 seconds with the mean of 24.2 seconds. Delayed arm-retinal circulation duration and retinal a rteriovenous filling duration in 10 cases (37.0%); microangioma, vascular wall staining, nonperfused capillary area in 11 (40.7%); and anterior ischemic syndrome in 6 (22.2%) were found. In the 6 patients with anterior ischemic syndrome, 4 cases had narrow retinal artery, segmental changes of blood stream, vascular atresia, and abnormal arterio-venous anastomosis, and 2 cases had bold vascular loops. Conclusions The main manifestations of FFA in patients with low-perfused retinopathy are malperfusion and retinal ischemia, whose degrees relate to the extend of carotid artery stenosis or atresia, and the process of the disease.Serious retinal ischemia may combined with anterior ischemic syndrome. (Chin J Ocul Fundus Dis，2004，20：84-86)
Objective To study relationship between hemoglobin-AGE (Hb-AGE) levels and diabetic retinopathy (DR) in diabetics. Methods Hb-AGE content of 125 type 2 diabetic patients with or without DR was measured by competitive ELISA technique and compared with that of 50 normal controls. Results Hb-AGE level in type 2 diabetic patients was 65% higher than that in normal individuals (Plt;0.01), and Hb-AGE level in the patients with DR was significantly higher than patients with DR was significantly higher than that in those without DR (Plt;0.05). It was found that fasting plasma glucose (FPG) level was not directly correlated with Bb-AGE levels and development of DR ,but HbAc,plasma lipid and blood pressure were related to the both (Plt;0.05 or Plt;0.01). Multivariate analysis showed that there was closer relationship between seriousness degree of DR and Hb-AGE (partial correlation coefficient was 0.604,Plt;0.001). Conclusion Diabetic control is related to alterations in vivo Hb-AGE,which may contribute to occurrence and developement of DR in type 2 diabetes mellitus. (Chin J Ocul Fundus Dis,2000,16:147-149)