[Skip to Navigation]
May 1977

The Cellular Pathology of Neuronal Ceroid-Lipofuscinosis: A Golgi-Electronmicroscopic Study

Author Affiliations

From the Departments of Neurology and Neuropathology, Massachusetts General Hospital and Harvard Medical School, Boston, and the Eunice Kennedy Shriver Center for Mental Retardation, Inc, Waltham, Mass.

Arch Neurol. 1977;34(5):298-305. doi:10.1001/archneur.1977.00500170052010

• A cerebral biopsy specimen from a 4-year-old girl with a moderately advanced stage of the late infantile form of neuronal ceroid-lipofuscinosis was observed in routine cell and fiber stains and in Golgi and electronmicroscopic preparations. There was no evidence of neuronal degeneration or loss. Golgi impregnations identified a fusiform enlargement of proximal axon segments of most pyramidal neurons and some polymorphic neurons but not of other cortical neuronal classes. Typical curvilinear inclusions were found in all cells and appeared to be impacted within the dilated proximal axon segments of pyramidal neurons. The numbers of type II synapses on the axon hillock and dilated proximal axon segments of pyramidal neurons were much reduced, whereas type I synapses remained abundant in the neuropil.

Add or change institution