INCREASED DENSITY OF THE THALAMUS ON CT SCANS IN PATIENTS WITH GM2-GANGLIOSIDOSES

  • 1 January 1990
    • journal article
    • research article
    • Vol. 11  (1) , 125-130
Abstract
In 13 patients, the GM2 gangliosidoses, Sandhoff disease and Tay-Sachs disease, were found to be constantly associated wth homogeneously and symmetrically increased CT attenuation within the thalami. In the only patient examined with MR imaging, a T2-weighted sequence showed hypointense thalami. It is suggested that this finding is caused by an accumulation of calcium, associated with the intracellular storage of GM2 ganglioside. The finding of dense thalami may be useful as a specific diagnostic criterion for GM2 gangliosidoses. In a few patients with blocks in adjacent steps in the sphingolipid metabolism, this finding was not present.

This publication has 1 reference indexed in Scilit: