January 1975

Multilamellar Cytosomes in a Particular Form of Late-Infantile Amaurotic Idiocy

Author Affiliations

From the Dr. C. A. Janeway Child Health Centre (Drs. Jacob, Kutty, and Islam), and the departments of pediatrics (Dr. Jacob) and cell sciences (Dr. Rowden), Memorial University Medical School, St. John's, Newfoundland, Canada. Dr. Rowden is now with the McGill University Cancer Research Unit, Montreal.

Am J Dis Child. 1975;129(1):109-112. doi:10.1001/archpedi.1975.02120380079018

There is a particular form of late-infantile amaurotic idiocy in which no clear chemical-pathological or unique enzyme abnormalities have been identified to date. A distinctive morphological feature has been recognized on electron microscopical examination of tissues from these patients, which has been descriptively labeled with various terms, including "multilamellar cytosomes" (MLC).

Illustrations of MLC in a patient with this late-infantile form of cerebroretinal degeneration show their reactivity with the periodic acid-silver methenamine reaction for glycoproteins. The MLC are shown to be morphologically identical in cerebral tissue obtained at biopsy, in the same tissue obtained three years later at autopsy, and in skeletal muscle.