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[视网膜电图诊断与鉴别诊断:6年检查结果]

[ERG diagnosis and differential diagnosis: results of examination over 6 years].

作者信息

Stemeyer G, Stähli P

机构信息

Universitäts-Augenklinik Zürich.

出版信息

Klin Monbl Augenheilkd. 1996 May;208(5):306-10.

PMID:8766035
Abstract

PURPOSE

This study reviews the patient material first from the point of view of referral diagnosis. Secondly, we focus on difficulties in selective differential diagnoses.

PATIENTS AND METHOD

1501 patients underwent electroretinographic (ERG) testing from 1989 to 1994, amounting to 1815 ERG recordings, including follow-up examinations. The technique applied is full-field, single flash ERG with selective stimulation of the rod- and of the cone-systems. In 3.8% (57 cases) the ERG was performed under general anesthesia in outpatients.

RESULTS

Tapetoretinal degenerations, toxic retinal side effects, inflammatory disease and ocular trauma represented, in this order, the major groups of referral diagnoses aside from unclear visual loss. The documentation or the exclusion of tapetoretinal degeneration represented the largest share (57%) of the application of the diagnostic procedure. 171 cases of isolated retinitis pigmentosa (RP) and 33 cases of syndromic RP were identified. Frequent and rare diagnostic entities and their differential diagnoses within this group are discussed. Inevitably, a number of diagnostic decisions remain problematic, in particular at the first examination. These diagnostic difficulties are addressed also and include the differentiation between RP sine pigmento and congenital amaurosis Leber in infants, RP with macular involvement vs. cone-rod degeneration, unilateral RP vs. postinflammatory conditions, and progressive cone dystrophy vs. achromatopsia, cone-rod degeneration or Stargardt's disease.

CONCLUSIONS

Frequent and meaningful indications for ERG recording and difficult diagnostic decisions arise from this review of a relatively large group of patients. A number of diagnoses can hardly, if not at all be established without ERG testing. These include retinal cause of visual loss in infants, congenital amaurosis Leber, RP sine pigmento, early stages of RP, carrier status in XL RP and in choroideremia, progressive cone dystrophy, toxic retinopathy without fundus changes, retinal involvement in uveitis with opaque media, and incomplete CSNB.

摘要

目的

本研究首先从转诊诊断的角度回顾患者资料。其次,我们关注选择性鉴别诊断中的困难。

患者与方法

1989年至1994年期间,1501例患者接受了视网膜电图(ERG)检测,共进行了1815次ERG记录,包括随访检查。所应用的技术是全视野单次闪光ERG,对杆体和锥体系统进行选择性刺激。3.8%(57例)的ERG检查是在门诊患者的全身麻醉下进行的。

结果

除视力丧失原因不明外,视网膜色素变性、中毒性视网膜副作用、炎症性疾病和眼外伤依次是主要的转诊诊断类别。视网膜色素变性的记录或排除在诊断程序应用中占最大比例(57%)。确定了171例孤立性视网膜色素变性(RP)和33例综合征性RP。讨论了该组中常见和罕见的诊断实体及其鉴别诊断。不可避免地,一些诊断决策仍然存在问题,尤其是在初次检查时。还讨论了这些诊断困难,包括婴儿期无色素性RP与先天性莱伯黑蒙性盲的鉴别、黄斑受累的RP与锥体-杆体营养不良的鉴别、单侧RP与炎症后状况的鉴别、进行性锥体营养不良与色盲、锥体-杆体营养不良或斯塔加特病的鉴别。

结论

对相对大量患者的回顾表明,ERG记录有常见且有意义的指征,同时存在困难的诊断决策。如果不进行ERG检测,一些诊断几乎无法确立,甚至根本无法确立。这些包括婴儿视力丧失的视网膜原因、先天性莱伯黑蒙性盲、无色素性RP、RP早期、X连锁RP和脉络膜视网膜萎缩症的携带者状态、进行性锥体营养不良、无眼底改变的中毒性视网膜病变、伴有混浊介质的葡萄膜炎中的视网膜受累以及不完全性先天性静止性夜盲。

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