Affiliation: | 1 Clarke Institute of Psychiatry, Toronto Ont., Canada 2 Rotman Research Institute of Baycrest Centre, Toronto, Ont., Canada 3 Montreal Neurological Institute, Quebec, Que., Canada 4 Oregon Health Sciences Center, Portland, OR, U.S.A. 5 Victoria Hospital, London, Ont., Canada 6 Toronto General Hospital, Toronto, Ont., Canada 7 University of Minnesota, Minneapolis, MN, U.S.A. |
Abstract: | We measured the concentration of glycerophosphoethanolamine (GPEA), a membrane breakdown product, in autopsied brain of 10 patients with dominantly inherited olivopontocerebellar atrophy (OPCA), a cerebellar ataxia disorder. As compared with the controls, mean GPEA levels were significantly elevated by 37–69% in 11 of the 15 brain areas examined, including extracerebellar brain regions in which no neuronal cell loss could be detected by semiquantitative estimation. Our data suggest the possibility of altered membrane phospholipid metabolism in OPCA which could be a contributing factor in the neuronal cell death. |