Kimura S, Goebel H H
Division of Neuropathology, University of Mainz, Federal Republic of Germany.
Brain Dev. 1987;9(6):576-80. doi: 10.1016/s0387-7604(87)80088-8.
Skin and lymphocytes of three patients with early juvenile neuronal ceroid-lipofuscinosis (NCL) were ultrastructurally investigated. Fingerprint profiles (FPP), isolated and/or mixed with curvilinear profiles (CLP), in various dermal cells and large, usually single lipopigments delineated by a trilaminar membrane and filled with a granular matrix, FPP and occasionally lipid droplets in lymphocytes were observed in all three patients. Characteristic lipopigments in lymphocytes are an important feature to differentiate between early juvenile NCL and late infantile and juvenile NCL.
对三名早发性青少年神经元蜡样脂褐质沉积症（NCL）患者的皮肤和淋巴细胞进行了超微结构研究。在所有三名患者中，均观察到在各种真皮细胞中存在指纹样结构（FPP），其可单独存在和/或与曲线样结构（CLP）混合，还有大的、通常为单个的、由三层膜包绕并充满颗粒状基质的脂色素，以及淋巴细胞中的FPP和偶尔出现的脂滴。淋巴细胞中的特征性脂色素是区分早发性青少年NCL与晚发性婴儿型和青少年型NCL的一个重要特征。
