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PT  - JOURNAL ARTICLE
AU  - Brismar, J
AU  - Brismar, G
AU  - Coates, R
AU  - Gascon, G
AU  - Ozand, P
TI  - Increased density of the thalamus on CT scans in patients with GM2 gangliosidoses.
DP  - 1990 Jan 01
TA  - American Journal of Neuroradiology
PG  - 125--130
VI  - 11
IP  - 1
4099  - http://www.ajnr.org/content/11/1/125.short
4100  - http://www.ajnr.org/content/11/1/125.full
SO  - Am. J. Neuroradiol.1990 Jan 01; 11
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.