成人型与青少年型神经元蜡样质脂褐质沉积症

Adult Neuronal Ceroid Lipofuscinosis and Juvenile Neuronal Ceroid Lipofuscinosis

目的 :报道 2例神经元蜡样质脂褐质沉积症 (NCL)。方法 :临床和病理学观察。结果 :2例病人均有智能障碍 ,但ANCL同时伴有运动障碍 ,JNCL则伴有癫及性格改变。电镜下ANCL胶质细胞及神经元胞质内沉积物形式主要为脂褐素体 ,而JNCL主要为指纹体 ,同时有较多脂褐素体。结论 :根据起病年龄。

Aim:To report a case of Adult Neuronal Ceroid Lipofuscinosis (ANCL) and a case of Juvenile Neuronal Ceroid Lipofuscinosis (JNCL). Methods:The clinicopathological studies of an ANCL and JNCL proved by electromicroscope were performed. Results:In clinical, the two types had dementia, but ANCL accompanied motor impairment, and JNCL accompanied seizures and personality changes. At ultrastructure level, most of the deposits of ANCL were lipofusion granules, but those of JNCL were fingerprint bodies and lipofusion granules. Conclusion:We could differ ANCL from JNCL according to age, clinical manifestations and ultrastructures.