Optic Nerve Projections in Patients with Primary Ciliary Dyskinesia
Optic Nerve Projections in Patients with Primary Ciliary Dyskinesia
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DOI：
10.1167/iovs.11-7194
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发表时间：
2011-06-01
影响因子：
4.4
通讯作者：
Golla, Franziska
中科院分区：
文献类型：
--
作者：
Hoffmann, Michael B.;Wolynski, Barbara;Golla, Franziska
目的。最近，有研究表明，颞视网膜向对侧半球的错误投射可能不是白化病患者所特有的，也可能与Kartagener综合征（即原发性纤毛运动障碍[PCD]时的全反位）有关。作者测试了这种投影异常是否与PCD和倒位有关。在10例PCD患者（5例完全性倒位）和10例年龄和性别匹配的对照中，单眼记录了视觉诱发电位（vep），如下：常规模式发作的vep （cvep）和60个位置的多焦点vep (mfvep)，构成44直径的视野。纳入13例白化病患者的cvep作为参考。对于每只眼睛，计算半球间差异电位并相互关联以评估反应的偏侧性：正相关系数和负相关系数分别表示在相同或相反的半球上偏侧化。视神经走路错误预计会产生负的眼间相关性。对于cvep和mfvep， PCD患者的相关系数分布大部分为正值，与对照组没有差异。因此，在PCD中没有观察到大或小的侧化异常。此外，视神经的投射并不依赖于视反位的存在。在一组10名PCD患者（其中5人患有Kartagener综合征）中，没有证据表明投射异常，这强调了视神经走错并不是这些患者的共同特征。（Invest Ophthalmol Vis Sci. 2011;52:4617-4625） DOI:10.1167/iovs.117194
PURPOSE. Recently, it has been suggested that misprojections of the temporal retina to the contralateral hemisphere might not be specific for patients with albinism and might also be associated with the Kartagener syndrome (i.e., with situs inversus totalis in the presence of primary ciliary dyskinesia [PCD]). The authors tested whether such projection abnormalities are associated with PCD and situs inversus.METHODS. In 10 patients with PCD (five with situs inversus totalis) and in 10 age-and sex-matched controls, visual evoked potentials (VEPs) were recorded monocularly, as follows: conventional pattern-onset VEPs (cVEPs) and multifocal VEPs (mfVEPs) for 60 locations constituting a visual field of 44 diameter. cVEPs from 13 albinotic subjects were included as a reference. For each eye, interhemispheric difference potentials were calculated and correlated with each other to assess the lateralization of the responses: positive and negative correlation coefficients indicated lateralizations on same or opposite hemispheres, respectively. Misrouted optic nerves are expected to yield negative interocular correlations.RESULTS. For both cVEPs and mfVEPs, the distribution of the correlation coefficients in the PCD patients yielded largely positive values and did not differ from that of the controls. Consequently, neither large-nor small-scale lateralization abnormalities were observed in PCD. Further, the optic nerve projection did not depend on the presence of situs inversus.CONCLUSIONS. The absence of evidence for projection abnormalities in a cohort of 10 subjects with PCD, five of whom had Kartagener syndrome, underscores that misrouting of the optic nerves is not a common trait of these subjects. (Invest Ophthalmol Vis Sci. 2011;52:4617-4625) DOI:10.1167/iovs.117194