Pigment-filled appendages of the small spiny neurons: a severe pathological change of the striatum in neuronal ceroid lipofuscinosis.

In neuronal ceroid lipofuscinosis, the small spinous nerve cells of the striatum show a conspicuous pathological change in that they develop spindle-shaped and pigment-filled appendages of the soma. The axon emerges from the tip of these expansions, the volume of which often exceeds that of the cell body. The aspiny neurons of the striatum do not show this alteration. The pigment-filled expansions close to the axon may be considered an early sign of neuronal degeneration. The small spinous nerve cell is the predominant cell type of the striatum which receives inputs from various sources. Its slowly progressive destruction might account for extra-pyramidal motor disturbances in the course of neuronal ceroid lipofuscinosis.