Straatsma B R, Foos R Y, Heckenlively J R, Taylor G N
Am J Ophthalmol. 1981 Jan;91(1):25-38. doi: 10.1016/0002-9394(81)90345-7.
In a series of 3,968 consecutive autopsies, myelinated nerve fibers of the retina were presented in 39 (0.98%) cases and bilateral in three (7.7%) affected cases; thus, 42 (0.54%) of the 7,936 eyes examined were affected. Myelinated nerve fiber lesions appeared as white or gray-white striated patches corresponding in shape to the distribution of retinal nerve fibers and demonstrated frayed borders. Myelinated retinal nerve fibers were continuous with the optic nerve head in 14 (33%) and discontinuous with the optic nerve head in 28 (66%) affected eyes. By light microscopy and electron microscopy, myelinated retinal fibers were marked by a ganglion cell axon surrounded by concentric lipoprotein lamellae that formed the myelin sheath. Glial cells were often prominent near the myelin sheaths, but other components of the sensory retina were morphologically normal. Clinically, 32 patients with myelinated retinal nerve fibers had comparable overall features, visual field defects less extensive than expected on the basis of ophthalmoscopic appearance, and normal findings on fluorescein angiography. Four patients had a syndrome characterized by ipsilateral extensive myelinated retinal nerve fibers, anisometropic myopia, amblyopia, and strabismus.
在连续3968例尸检中，39例（0.98%）视网膜出现有髓神经纤维，其中3例（7.7%）为双侧受累；因此，在检查的7936只眼中，42只（0.54%）受累。有髓神经纤维病变表现为白色或灰白色条纹状斑块，其形状与视网膜神经纤维分布相对应，边界呈参差不齐状。在14只（33%）受累眼中，有髓视网膜神经纤维与视神经乳头相连，28只（66%）受累眼中则与视神经乳头不相连。通过光学显微镜和电子显微镜观察，有髓视网膜纤维的特征是神经节细胞轴突被形成髓鞘的同心脂蛋白层包围。神经胶质细胞在髓鞘附近通常很明显，但感觉视网膜的其他成分在形态上是正常的。临床上，32例有髓视网膜神经纤维患者具有相似的总体特征，视野缺损范围比根据检眼镜检查外观预期的要小，荧光素血管造影检查结果正常。4例患者有一种综合征，其特征为同侧广泛的有髓视网膜神经纤维、屈光参差性近视、弱视和斜视。
