Atypical juvenile neurolipidosis. Ultrastructural study of a cerebral biopsy

The description is given of a structural and ultrastructural aspect of a cerebral biopsy specimen collected from a 17-year-old patient, with very frequent convulsive seizures, dementia, motor aphasia and spastic tetraparesis. The disease started at the age of 9 years and evolved very slowly. The morphologic diagnosis was neurolopidosis, the ultrastructural one was atypical juvenile lipofuscinosis, the electron microscopic aspect being identical to that of another 3 cases published in the literature: highly polymorphous membranogranulovesicular cytosomes.