PDF(Pubmed)
Abstract：
UNASSIGNED: To describe the retinal phenotype of an unusual case of anti-TRPM1 autoantibody-positive unilateral melanoma-associated retinopathy (MAR) triggered by nivolumab therapy and compare with the phenotype of TRPM1-associated Congenital Stationary Night Blindness (TRPM1-CSNB).
UNASSIGNED: Unilateral MAR was diagnosed 3 months after starting nivolumab therapy for consolidation of a successfully treated melanoma. Retinal autoantibodies against TRPM1 were identified. ffERG, microperimetry and static chromatic perimetry confirmed unilateral ON-Bipolar Cell (ON-BPC) dysfunction and central rod sensitivity losses in the left eye; the contralateral eye was normal. There was borderline ganglion cell (GCL) and inner nuclear layer (INL) thinning, but a significantly thinner inner plexiform layer (IPL) in the affected compared to the unaffected eye. Longitudinal reflectivity profiles (LRPs) demonstrated an abnormal inner plexiform layer (IPL) lamination in the involved eye. Nearly identical changes were documented in two cases of TRMP1-cCSNB and in a case of anti-TRPM1 autoantibody-negative MAR. The functional changes partially recovered with discontinuation of the medication without added immunosuppression.
UNASSIGNED: Comparisons between the affected and unaffected eye in this unilateral MAR case revealed inner retinal abnormalities and abnormal lamination of the IPL associated with the classical retina-wide ON-BPC dysfunction, and localized central rod-mediated sensitivity losses. A nearly identical structural phenotype in two cases of cCSNB and a case of anti-TRPM1 autoantibody-negative MAR supports a specific structural-functional phenotype for these conditions with ON-BPC dysfunction.
UNASSIGNED: Unilateral MAR was diagnosed 3 months after starting nivolumab therapy for consolidation of a successfully treated melanoma. Retinal autoantibodies against TRPM1 were identified. ffERG, microperimetry and static chromatic perimetry confirmed unilateral ON-Bipolar Cell (ON-BPC) dysfunction and central rod sensitivity losses in the left eye; the contralateral eye was normal. There was borderline ganglion cell (GCL) and inner nuclear layer (INL) thinning, but a significantly thinner inner plexiform layer (IPL) in the affected compared to the unaffected eye. Longitudinal reflectivity profiles (LRPs) demonstrated an abnormal inner plexiform layer (IPL) lamination in the involved eye. Nearly identical changes were documented in two cases of TRMP1-cCSNB and in a case of anti-TRPM1 autoantibody-negative MAR. The functional changes partially recovered with discontinuation of the medication without added immunosuppression.
UNASSIGNED: Comparisons between the affected and unaffected eye in this unilateral MAR case revealed inner retinal abnormalities and abnormal lamination of the IPL associated with the classical retina-wide ON-BPC dysfunction, and localized central rod-mediated sensitivity losses. A nearly identical structural phenotype in two cases of cCSNB and a case of anti-TRPM1 autoantibody-negative MAR supports a specific structural-functional phenotype for these conditions with ON-BPC dysfunction.
摘要：
■描述由nivolumab治疗引发的抗TRPM1自身抗体阳性单侧黑色素瘤相关视网膜病变（MAR）的不寻常病例的视网膜表型，并与TRPM1相关的先天性固定夜盲症（TRPM1-CSNB）的表型进行比较。
■在开始nivolumab治疗合并成功治疗的黑色素瘤后3个月诊断为单侧MAR。鉴定了针对TRPM1的视网膜自身抗体。ffERG,显微视野和静态色度视野检查证实左眼单侧双极型细胞（ON-BPC）功能障碍和中央棒敏感性丧失；对侧眼睛正常。有交界神经节细胞（GCL）和内核层（INL）变薄，但与未受影响的眼睛相比，受影响的内部丛状层（IPL）明显更薄。纵向反射率分布（LRP）表明受累眼睛中存在异常的内部丛状层（IPL）层压。在两例TRMP1-cCSNB和一例抗TRPM1自身抗体阴性MAR中记录了几乎相同的变化。在不增加免疫抑制的情况下，停止药物治疗后，功能变化部分恢复。
在这个单侧MAR病例中，受影响和未受影响的眼睛之间的比较显示，内部视网膜异常和与经典视网膜宽的ON-BPC功能障碍相关的IPL异常分层，和局部中央杆介导的敏感性损失。在两个cCSNB病例和一个抗TRPM1自身抗体阴性MAR病例中，几乎相同的结构表型支持这些具有ON-BPC功能障碍的病症的特定结构功能表型。
■在开始nivolumab治疗合并成功治疗的黑色素瘤后3个月诊断为单侧MAR。鉴定了针对TRPM1的视网膜自身抗体。ffERG,显微视野和静态色度视野检查证实左眼单侧双极型细胞（ON-BPC）功能障碍和中央棒敏感性丧失；对侧眼睛正常。有交界神经节细胞（GCL）和内核层（INL）变薄，但与未受影响的眼睛相比，受影响的内部丛状层（IPL）明显更薄。纵向反射率分布（LRP）表明受累眼睛中存在异常的内部丛状层（IPL）层压。在两例TRMP1-cCSNB和一例抗TRPM1自身抗体阴性MAR中记录了几乎相同的变化。在不增加免疫抑制的情况下，停止药物治疗后，功能变化部分恢复。
在这个单侧MAR病例中，受影响和未受影响的眼睛之间的比较显示，内部视网膜异常和与经典视网膜宽的ON-BPC功能障碍相关的IPL异常分层，和局部中央杆介导的敏感性损失。在两个cCSNB病例和一个抗TRPM1自身抗体阴性MAR病例中，几乎相同的结构表型支持这些具有ON-BPC功能障碍的病症的特定结构功能表型。
