Pediatric radiology · 2005
Proton MR spectroscopy in three children with Tay-Sachs disease.
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Aydin Kubilay, Bakir Baris, Tatli Burak, Terzibasioglu Ege, Ozmen Meral
Аннотация
BACKGROUND: Tay-Sachs disease is an inherited metabolic disease caused by the accumulation of GM(2) gangliosides in the central nervous system. Deficiency of hexosaminidase A leads to the accumulation of gangliosides in neurons, axons and glial cells.
OBJECTIVE: To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease.
MATERIALS AND METHODS: Three children aged 10, 20 and 21 months were examined.
RESULTS: On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio.
CONCLUSIONS: The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease.
OBJECTIVE: To present the cranial MRI and proton MR spectroscopy findings of children of Tay-Sachs disease.
MATERIALS AND METHODS: Three children aged 10, 20 and 21 months were examined.
RESULTS: On T2-weighted MR images there were hyperintense signal changes in the basal ganglia and cerebral white matter. MR spectroscopy demonstrated an increase in myoinositol/creatine and choline/creatine ratios with a decrease in the N-acetyl aspartate/creatine ratio.
CONCLUSIONS: The spectroscopy findings support demyelination, gliosis and neuronal loss in the neuropathological process of Tay-Sachs disease.