Abstract
Electronmicroscopic examination of brain tissue from patients with juvenile amaurotic idiocy (Batten-Spielmeyer-Vogt disease) revealed a highly characteristic fine architecture of the lipid bodies accumulated in the perikarya. These pleomorphic bodies present two different types of internal structure, either a densely granular stroma, or round, oval and tubular membranes giving the cross section a multilocular appearance. There is some suggestion that the bodies might derive from degenerating mitochondria. In all four patients, regardless of age, the lipid bodies were similar in structure but they were distinctly different from the membranous cytoplasmic bodies of infantile amaurotic idiocy (Tay-Sachs disease) as described byTerry andKorey.
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