中华眼底病杂志
中華眼底病雜誌
중화안저병잡지
CHINESE JOURNAL OF OCULAR FUNDUS DISEASES
2010年
4期
331-334
,共4页
张潇%董方田%于伟泓%张枝桥%戴荣平%卞爱玲
張瀟%董方田%于偉泓%張枝橋%戴榮平%卞愛玲
장소%동방전%우위홍%장지교%대영평%변애령
视网膜疾病/病因学%线粒体脑肌病/并发症%线粒体脑肌病/诊断
視網膜疾病/病因學%線粒體腦肌病/併髮癥%線粒體腦肌病/診斷
시망막질병/병인학%선립체뇌기병/병발증%선립체뇌기병/진단
Retinal disease/etiology%Mitochondrial encephalomyopathies/complications%Mitochondrial encephalomyopathies/diagnosis
目的 观察线粒体脑肌病患者眼底病变的特点、分类及其与全身性表现的关系.方法 回顾性病例研究.对内科住院治疗的88例线粒体脑肌病患者的临床资料进行分析,其中确诊线粒体脑肌病并经眼科会诊发现眼底异常、病历记录资料完整的12例24只眼纳入研究.男性9例,女性3例;年龄14～33岁,平均年龄(20.1±7.0)岁.病程2.5～20.0年,平均病程(9.5±6.8)年.患者均有不同程度的眼外症状,包括肢体无力、听力下降及中枢神经系统症状等.眼科检查行国际标准视力表最佳矫正视力、裂隙灯显微镜、间接检眼镜检查.非接触眼压计眼压检查,上睑下垂情况、眼球运动、瞳孔反射等检查和眼底彩色照相.行荧光素眼底血管造影(FFA)检查3例、光相干断层扫描(OCT)检查1例、视网膜电图(ERG)检查2例,视野检查5例.根据检查结果将视网膜病变分为"椒盐状"眼底、视网膜色素变性(RP)样眼底、视网膜色素上皮(RPE)和脉络膜毛细血管萎缩、单纯视神经萎缩等4种类型.回顾分析时同时观察与全身病变的关系.结果 所有患者均为双眼发病,双侧病变程度基本一致.出现上睑下垂和(或)眼球运动障碍者9例,占75.0%;视力下降者6例,占50.0%."椒盐状"眼底者6例12只眼,占有眼底异常改变者50.0%.表现为视网膜颗粒状色素沉着和脱色素;视力0.4～1.2;均无听力下降、抽搐、共济失调、智力减退等中枢神经系统表现.RP样眼底者1例2只眼,占有眼底异常改变者8.3%.表现为视网膜骨细胞样色素沉着、视网膜血管变细、视神经萎缩;双眼视力光感,伴有智力低下、听力下降、双下肢疼痛及发作性抽动.RPE和脉络膜毛细血管萎缩者3例6只眼,占有眼底异常改变者25.0%.可见暴露的脉络膜大血管,周围视网膜可见片状色素堆积;视力跟前手动～0.7;肢体无力2例;听力下降3例;言语不清、智力下降2例;嗜睡1例.单纯视神经萎缩者2例4只眼,占有眼底异常改变者16.7%.视力0.1～0.7;均有肢体无力、抽搐、智力下降、头痛等中枢神经系统表现.结论 线粒体脑肌病患者眼底改变分为"椒盐状"眼底、RP样眼底、RPE和脉络膜毛细血管萎缩和单纯视神经萎缩等4种类型.眼底病变类型与视力预后和中枢神经系统表现有关.
目的 觀察線粒體腦肌病患者眼底病變的特點、分類及其與全身性錶現的關繫.方法 迴顧性病例研究.對內科住院治療的88例線粒體腦肌病患者的臨床資料進行分析,其中確診線粒體腦肌病併經眼科會診髮現眼底異常、病歷記錄資料完整的12例24隻眼納入研究.男性9例,女性3例;年齡14～33歲,平均年齡(20.1±7.0)歲.病程2.5～20.0年,平均病程(9.5±6.8)年.患者均有不同程度的眼外癥狀,包括肢體無力、聽力下降及中樞神經繫統癥狀等.眼科檢查行國際標準視力錶最佳矯正視力、裂隙燈顯微鏡、間接檢眼鏡檢查.非接觸眼壓計眼壓檢查,上瞼下垂情況、眼毬運動、瞳孔反射等檢查和眼底綵色照相.行熒光素眼底血管造影(FFA)檢查3例、光相榦斷層掃描(OCT)檢查1例、視網膜電圖(ERG)檢查2例,視野檢查5例.根據檢查結果將視網膜病變分為"椒鹽狀"眼底、視網膜色素變性(RP)樣眼底、視網膜色素上皮(RPE)和脈絡膜毛細血管萎縮、單純視神經萎縮等4種類型.迴顧分析時同時觀察與全身病變的關繫.結果 所有患者均為雙眼髮病,雙側病變程度基本一緻.齣現上瞼下垂和(或)眼毬運動障礙者9例,佔75.0%;視力下降者6例,佔50.0%."椒鹽狀"眼底者6例12隻眼,佔有眼底異常改變者50.0%.錶現為視網膜顆粒狀色素沉著和脫色素;視力0.4～1.2;均無聽力下降、抽搐、共濟失調、智力減退等中樞神經繫統錶現.RP樣眼底者1例2隻眼,佔有眼底異常改變者8.3%.錶現為視網膜骨細胞樣色素沉著、視網膜血管變細、視神經萎縮;雙眼視力光感,伴有智力低下、聽力下降、雙下肢疼痛及髮作性抽動.RPE和脈絡膜毛細血管萎縮者3例6隻眼,佔有眼底異常改變者25.0%.可見暴露的脈絡膜大血管,週圍視網膜可見片狀色素堆積;視力跟前手動～0.7;肢體無力2例;聽力下降3例;言語不清、智力下降2例;嗜睡1例.單純視神經萎縮者2例4隻眼,佔有眼底異常改變者16.7%.視力0.1～0.7;均有肢體無力、抽搐、智力下降、頭痛等中樞神經繫統錶現.結論 線粒體腦肌病患者眼底改變分為"椒鹽狀"眼底、RP樣眼底、RPE和脈絡膜毛細血管萎縮和單純視神經萎縮等4種類型.眼底病變類型與視力預後和中樞神經繫統錶現有關.
목적 관찰선립체뇌기병환자안저병변적특점、분류급기여전신성표현적관계.방법 회고성병례연구.대내과주원치료적88례선립체뇌기병환자적림상자료진행분석,기중학진선립체뇌기병병경안과회진발현안저이상、병력기록자료완정적12례24지안납입연구.남성9례,녀성3례;년령14～33세,평균년령(20.1±7.0)세.병정2.5～20.0년,평균병정(9.5±6.8)년.환자균유불동정도적안외증상,포괄지체무력、은력하강급중추신경계통증상등.안과검사행국제표준시력표최가교정시력、렬극등현미경、간접검안경검사.비접촉안압계안압검사,상검하수정황、안구운동、동공반사등검사화안저채색조상.행형광소안저혈관조영(FFA)검사3례、광상간단층소묘(OCT)검사1례、시망막전도(ERG)검사2례,시야검사5례.근거검사결과장시망막병변분위"초염상"안저、시망막색소변성(RP)양안저、시망막색소상피(RPE)화맥락막모세혈관위축、단순시신경위축등4충류형.회고분석시동시관찰여전신병변적관계.결과 소유환자균위쌍안발병,쌍측병변정도기본일치.출현상검하수화(혹)안구운동장애자9례,점75.0%;시력하강자6례,점50.0%."초염상"안저자6례12지안,점유안저이상개변자50.0%.표현위시망막과립상색소침착화탈색소;시력0.4～1.2;균무은력하강、추휵、공제실조、지력감퇴등중추신경계통표현.RP양안저자1례2지안,점유안저이상개변자8.3%.표현위시망막골세포양색소침착、시망막혈관변세、시신경위축;쌍안시력광감,반유지력저하、은력하강、쌍하지동통급발작성추동.RPE화맥락막모세혈관위축자3례6지안,점유안저이상개변자25.0%.가견폭로적맥락막대혈관,주위시망막가견편상색소퇴적;시력근전수동～0.7;지체무력2례;은력하강3례;언어불청、지력하강2례;기수1례.단순시신경위축자2례4지안,점유안저이상개변자16.7%.시력0.1～0.7;균유지체무력、추휵、지력하강、두통등중추신경계통표현.결론 선립체뇌기병환자안저개변분위"초염상"안저、RP양안저、RPE화맥락막모세혈관위축화단순시신경위축등4충류형.안저병변류형여시력예후화중추신경계통표현유관.
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 in-patients 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, non-contact 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 movement-0. 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.
