[A clinicopathological study of neuronal ceroid lipofuscinosis].

Abstract

4 unrelated patients with infantile type (one case), late infantile type (one case), juvenile type (two case) of neuronal ceroid-lipofuscinosis were reported. The pathological study by brain biopsy was carried out with light and electron microscopes as well as histochemical methods. Ultrastructurally, the infantile type showed deposits of lipofuscin bodies, fingerprint bodies, and the late infantile type showed a lot of curvilinear bodies and paracrystalline bodies. The ultrastructural configuration in the juvenile type is more heterogeneous and consisted of combination of lipofuscin bodies, fingerprint bodies and multilamellar structures. It was emphasized that fingerprint bodies were first discovered in infantile type, which might challenge the view that only lipofuscin bodies can be found in infantile type.