Lymphocyte ultrastructure in two cases of neuronal ceroid‐lipofuscinosis

Abstract

Circulating blood lymphocytes from 2 patients with neuronal ceroid-lipofuscinosis (NCL) were investigated by transmission electronmicroscopy. Ultrastructural examination showed 2 forms of intracytoplasmic single membrane-limited inclusions. Contents of the 1st inclusion form were arranged in 5 distinct patterns: granules, membranous formations, paracrystalline forms, alternating electron-dense/electron-lucent arrangements, and admixtures of these components. These molecular morphologies suggest the usefulness of lymphocyte fine structure as a diagnostic tool in NCL. The 2nd inclusion form contained cylinder-like structures. These structures are not specific for NCL and have been identified in other diseases.