Abstract

A case is presented of the ataxic variety of Creutzfeldt-Jakob disease with particular reference to the cerebellar cortex. The main features were loss of granule cells, subtotal in the vermis, severe in the lateral lobes, mild to moderate loss of Purkinje cells and preservation of tangential and basket fibres. The Purkinje cell dendrites showed malorientation and hypertrophy of the primary and secondary branches, the so-called "antler" or "staghorn" deformity. These findings indicate that remodelling of the dendritic tree may start early in the course of the disease even in adults, the total length of history in this case being eight months. They do not throw any additional light on the pathogenesis of the dendritic abnormalities, in particular on the controversy whether they are a non-specific response of the Purkinje cell to a variety of noxious agents or a reaction to partial deafferentation. The authors favour the latter hypothesis.