Summary
The neuroradiological findings in four patients with Tay-Sachs disease are described in three phases of the clinical course. The basal ganglia and cerebral white matter show low density on computed tomography and high signal intensity on T2-weighted magnetic resonance imaging in the initial phase. The caudate nuclei are characteristically enlarged and protrude into the lateral ventricles in the first and second phases. The cerebral white matter shows low density on the CT which varies in extent from the second to third phases, and the whole brain becomes atrophic in the last phase. Thus, central nervous system involvement in the disease may begin in basal ganglia as well as in cerebral white matter.
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References
Kanof A, Aronsen SM, Volk BW (1959) Clinical progression of amaurotic idiocy. Anthropometric studies. Am J Dis Child 97: 656–662
Shimoizumi H, Miyao M, Ichihashi K, Sawa R, Yamamoto Y, Tanaka O, Yanagisawa M, Kamoshita S (1986) Serial CT scans of Tay-Sachs disease (in Japanese). No to Hattatsu 18: 464–469
Brismar J, Brismar G, Coates R, Gascon G, Ozand P (1990) Increased density of the thalamus on CT scans in patients with GM2 gangliosidosis. AJNR 11: 125–130
Kendall BE, Kingsley D (1978) The value of computerized axial tomography (CAT) in cranio-cerebral malformations. Br J Radiol 51: 171–190
Friede RL (1989) Sphingolipidosis. In: Friede RL (ed) Developmental neuropathology. Springer, Berlin Heidelberg New York, pp 426–447
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Fukumizu, M., Yoshikawa, H., Takashima, S. et al. Tay-Sachs disease: progression of changes on neuroimaging in four cases. Neuroradiology 34, 483–486 (1992). https://doi.org/10.1007/BF00598955
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DOI: https://doi.org/10.1007/BF00598955