GANGLIOSIDE ALTERATIONS IN THE CENTRAL AND PERIPHERAL NERVOUS SYSTEMSOF PATIENTS WITH CREUTZFELDT-JAKOB-DISEASE

We have examined the distribution and composition of gangliosides in c entral and peripheral nervous tissues from two patients with Creutzfel dt-Jakob disease (CJD). There were marked decreases in total gangliosi de levels in CJD, with reductions in the cerebral cortex and cerebellu m in the order of 20-30% and 50% of control values, respectively, thou gh in spinal cord and sciatic nerve total gangliosides were not signif icantly altered. The percentage distribution of individual ganglioside s was characterized by marked increases in GD3 and GD2, contrasting wi th severe decreases in GD1a, GD1b, GT1b and GQ1b; such changes were fo und throughout the patients' nervous tissues. An abnormal long-chain b ase composition was detected with the d20:1 component being decreased to less than 50% of control values, in the cerebrum, cerebellum and sp inal cord. Changes in gangliosides occurred even in those tissues not severely affected neuropathologically. These ganglioside abnormalities are discussed in relationship to the pathogenesis of CJD. (C) 1996 Ac ademic Press Limited