TY - JOUR
T1 - MR findings in Tay-Sachs disease
AU - Mugikura, Shunji
AU - Takahashi, Shoki
AU - Higano, Shuichi
AU - Kurihara, Noriko
AU - Kon, Kimiya
AU - Sakamoto, Kiyohiko
PY - 1996
Y1 - 1996
N2 - Objective: MR and CT findings in two patients of Tay-Sachs disease are reported. Materials and Methods: In two patients of Tay-Sachs disease, MR and CT studies were conducted in the early phase (11 and 10 months of age, respectively) and one was followed-up with MR in the late phase at the age of 5 years and 8 months. Six MR studies on this disease (including 3 other cases documented in the literature) were reviewed and correlated with the pathological phases of this disease. Results: MRI findings drastically changed during the phases of disease process in one case. In the early phase, T2-weighted MR images demonstrated hyperintensity in both basal ganglia and thalamus. MR images obtained in the late phase demonstrated markedly advanced brain atrophy and diffuse white matter lesions, which were hyperintense on the T2-weighted images. T1-weighted images exhibited hyperintensity in the basal ganglia, thalamus, and along the cortical layer of the cerebrum, which appeared to be hypointense on T2-weighted images. Conclusion: MR was apparently superior to CT in clearly delineating lesions of this disease. These abnormal signal intensities in the late phase may reflect the pathological findings, marked accumulation of GM2 ganglioside in the basal ganglia and thalamus and along the cerebral gray matter.
AB - Objective: MR and CT findings in two patients of Tay-Sachs disease are reported. Materials and Methods: In two patients of Tay-Sachs disease, MR and CT studies were conducted in the early phase (11 and 10 months of age, respectively) and one was followed-up with MR in the late phase at the age of 5 years and 8 months. Six MR studies on this disease (including 3 other cases documented in the literature) were reviewed and correlated with the pathological phases of this disease. Results: MRI findings drastically changed during the phases of disease process in one case. In the early phase, T2-weighted MR images demonstrated hyperintensity in both basal ganglia and thalamus. MR images obtained in the late phase demonstrated markedly advanced brain atrophy and diffuse white matter lesions, which were hyperintense on the T2-weighted images. T1-weighted images exhibited hyperintensity in the basal ganglia, thalamus, and along the cortical layer of the cerebrum, which appeared to be hypointense on T2-weighted images. Conclusion: MR was apparently superior to CT in clearly delineating lesions of this disease. These abnormal signal intensities in the late phase may reflect the pathological findings, marked accumulation of GM2 ganglioside in the basal ganglia and thalamus and along the cerebral gray matter.
KW - Computed tomography
KW - Magnetic resonance
KW - Tay-Sachs disease
UR - http://www.scopus.com/inward/record.url?scp=0030054671&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0030054671&partnerID=8YFLogxK
U2 - 10.1097/00004728-199607000-00009
DO - 10.1097/00004728-199607000-00009
M3 - Article
C2 - 8708054
AN - SCOPUS:0030054671
VL - 20
SP - 551
EP - 555
JO - Journal of Computer Assisted Tomography
JF - Journal of Computer Assisted Tomography
SN - 0363-8715
IS - 4
ER -