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Idiopathic multifocal choroiditis/punctate inner choroidopathy with acute photoreceptor loss or dysfunction out of proportion to clinically visible lesions.

机译:特发性多灶性脉络膜炎/点状内脉络膜疾病,急性光感受器丧失或功能障碍,与临床可见病变不成比例。

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摘要

PURPOSEududTo report acute/subacute vision loss and paracentral scotomata in patients with idiopathic multifocal choroiditis/punctate inner choroidopathy due to large zones of acute photoreceptor attenuation surrounding the chorioretinal lesions.ududMETHODSududMultimodal imaging case series.ududRESULTSududSix women and 2 men were included (mean age, 31.5 ± 5.8 years). Vision ranged from 20/20-1 to hand motion (mean, 20/364). Spectral domain optical coherence tomography demonstrated extensive attenuation of the external limiting membrane, ellipsoid and interdigitation zones, adjacent to the visible multifocal choroiditis/punctate inner choroidopathy lesions. The corresponding areas were hyperautofluorescent on fundus autofluorescence and were associated with corresponding visual field defects. Full-field electroretinogram (available in three cases) showed markedly decreased cone/rod response, and multifocal electroretinogram revealed reduced amplitudes and increased implicit times in two cases. Three patients received no treatment, the remaining were treated with oral corticosteroids (n = 4), oral acyclovir/valacyclovir (n = 2), intravitreal/posterior subtenon triamcinolone acetate (n = 3), and anti-vascular endothelial growth factor (n = 2). Visual recovery occurred in only three cases of whom two were treated. Varying morphological recovery was found in six cases, associated with decrease in hyperautofluorescence on fundus autofluorescence.ududCONCLUSIONududMultifocal choroiditis/punctate inner choroidopathy can present with transient or permanent central photoreceptor attenuation/loss. This presentation is likely a variant of multifocal choroiditis/punctate inner choroidopathy with chorioretinal atrophy. Associated changes are best evaluated using multimodal imaging.
机译:目的 ud ud要报告特发性多灶性脉络膜炎/点状内脉络膜病变患者的急性/亚急性视力减退和中央点旁壁窦,原因是脉络膜视网膜病变周围有大面积的急性感光细胞衰减。 ud udMETHODS ud ud多模式影像学病例系列。 ud udRESULTS ud ud包括六名女性和2名男性(平均年龄31.5±5.8岁)。视力范围从20 / 20-1到手部运动(平均20/364)。光谱域光学相干断层扫描显示,与可见的多灶性脉络膜炎/点状内部脉络膜病变相邻的外部限制膜,椭球和指间区广泛衰减。眼底自发荧光的相应区域是超自发荧光的,并与相应的视野缺损相关。全场视网膜电图(三种情况可用)显示锥体/棒反应显着降低,多焦点视网膜电图在两种情况下显示振幅降低和隐式时间增加。三名患者未接受任何治疗,其余患者接受口服皮质类固醇激素(n = 4),口服阿昔洛韦/伐昔洛韦(n = 2),玻璃体内/后腱膜醋酸曲安奈德(n = 3)和抗血管内皮生长因子(n = 2)。仅三例发生了视觉恢复,其中两例得到了治疗。在6例患者中发现了不同的形态学恢复,这与眼底自发荧光的过度自发荧光的降低有关。 ud ud结论 ud ud多灶性脉络膜炎/点状内脉络膜病变可表现为短暂或永久性中央感光器衰减/丢失。这种表现可能是多灶性脉络膜炎/点状内脉络膜病伴脉络膜视网膜萎缩的一种变体。使用多模式成像可以最好地评估相关的变化。

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