Cerebellar pathology in ataxia-telangiectasia: the significance of basket cells.

The unique neuropathology of AT is discussed, focusing on changes in the cerebellum--site of the most consistent and severe findings. In the cerebellum, despite marked Purkinje cell and granule cell loss and thinning of the molecular layer, the basket cells are relatively preserved, as demonstrated by the Bielschowsky staining. To document that basket cells do, indeed, represent a "footprint" of where Purkinje cells once existed, we examined the cerebellum of patients with chronic alcohol abuse. We again found normal numbers of "empty" basket cells. This suggests that AT is a degenerative condition in which the Purkinje cell layer forms, perhaps abnormally, but then undergoes neuronal depletion.