Ceroid‐lipofuscinosis (Batten's Disease)

Abstract

Two patients with the clinical picture of a late infantile and juvenile amaurotic familial idiocy (Batten's disease) have been examined electron microscopically. The typical cytoplasmic inclusion bodies in the Schwann cells of the sural nerve supported this clinical diagnosis. The importance of sural nerve biopsy in patients with a cerebroretinal degeneration is discussed.

The Importance of Retinal Electrophysiology in Ceroid Lipofuscinosis

“…Les travaux de Duffy et al (1968), Eisner et Prensky (1969), Carpenter (1972, Bolmers et al (1973), revelent que les modifications ultrastructurales mentionnees ne sont pas confinees au systheme nerveux central, mais s'etendent egalement aux neurones des muscles lisses (rectum, appendice), aux cellules de Schwann des nerfs peripheriques et meme a la rate et aux glandes sudoripares.…”