The significance of cytoplasmic inclusions in late infantile and juvenile amaurotic idiocy. An ultrastructural study.

Abstract

The electron microscopic findings of cerebral biopsies from two siblings with late infantile-juvenile amaurotic idiocy are presented. One patient had a mixture of “fingerprint” and multilamellar inclusions, often in the same cytosome. The second patient had only “fingerprint” inclusions. Electron microscopic findings in autopsied tissues from two other cases of late infantile-juvenile amaurotic idiocy with multilamellar cytosomes are also presented. Evolution of the multilamellar cytosomes into lipofuscin was not observed. We concluded that cases of late infantile-juvenile amaurotic idiocy with multilamellar and “fingerprint” inclusions should be distinguished from cases with lipofuscin.