Abstract

Spinocerebellar degeneration is a heredofamilial disease of unknown aetiology. The shape of erythrocytes as revealed by scanning electron microscopy was studied in this disease. Echinocytes I, as defined by Bessis, were seen more frequently in spinocerebellar degeneration than in age and sex matched controls (7.2 +/- 1.5% in spinocerebellar degeneration, 3.4 +/- 1.2% in controls, p less than 0.001), Parkinson's disease, motor neuron disease, myopathy, and Huntington's chorea. Erythrocyte deformability was impaired to a greater extent in spinocerebellar degeneration than in the controls when the pH was raised from 7.2 to 8.0; Echinocytes I in spinocerebellar degeneration increased from 8.4 +/- 0.6 to 15.4 +/- 2.4%, in the control group from 2.8 +/- 1.2 to 13.3 +/- 2.1%. In spinocerebellar degeneration no significant correlation was found between the level of serum low density lipoprotein and the number of Echinocytes I. In both groups there was a significant correlation between the occurrence of Echinocytes I and age, and the difference of Echinocytes I was greater in aged subjects in spinocerebellar degeneration. The data suggest that membrane abnormality in erythrocytes exists in spinocerebellar degeneration and may be accelerated with the advance of age.