IS PURKINJE-CELL LOSS IN LEIGHS DISEASE AN EXCITOTOXIC EVENT SECONDARY TO DAMAGE TO INFERIOR OLIVARY NUCLEI

In a series of 17 cases of Leigh's disease it has been observed that t here is a close correlation between damage to the inferior olivary nuc lei by vasculo-necrotic change and loss of Purkinje cells in the cereb ellar cortex. It is suggested that this association may be explained o n the basis of the selective loss of climbing fibres causing increased firing activity of Purkinje cells with consequent excessive entry of calcium ions. In these circumstances control of calcium ion regulation in the presence of reduced energy production, which is the basis of t his metabolic disease, would be expected to put these cells' survival seriously at risk.