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Neuronal ceroid-lipofuscinosis

Ultrastructural study of lymphocytic dense bodies

From the Unidade de Microscopia Electrónica da Faculdade de Medicina de Lisboa (Dr. Aguas), Laboratório de Virologia, Instituto Português de Oncologia (Dr. Moura Nunes), and Centro de Neurocirurgia de Lisboa (Drs. Hasse Ferreira and Vital), Lisbon, Portugal.

We studied the ultrastructural alterations of the dense bodies of peripheral blood lymphocytes in one case of neuronal ceroid-lipofuscinosis. These cytoplasmic vesicles were characterized by vacuolar transformation and "fingerprint" content. When we tilted the specimens, the "fingerprint" arrangement was shown to be the same as the arrangement of the crystalloid inclusions found in the same cells. Both vacuoles and periodic cytoplasmic inclusions could be due to a disorder of secondary lysosomes—the lymphocytic dense bodies.

Address correspondence and reprint requests to Dr. Aguas, Unidade de Microscopia Electronica, Faculdade de Medicina de Lisboa, Hospital de Santa Maria, Lisbon, Portugal.

Accepted for publication November 9, 1979.