RT Journal Article
SR Electronic
T1 Increased density of the thalamus on CT scans in patients with GM2 gangliosidoses.
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 125
OP 130
VO 11
IS 1
A1 J Brismar
A1 G Brismar
A1 R Coates
A1 G Gascon
A1 P Ozand
YR 1990
UL http://www.ajnr.org/content/11/1/125.abstract
AB In 13 patients, the GM2 gangliosidoses, Sandhoff disease and Tay-Sachs disease, were found to be constantly associated with 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.