Tay-Sachs disease is a lysosomal storage disease due to inability to metabolize GM2 gangliosides.
What is Tay-Sachs Disease?

A 10-month-old male patient with GM2-gangliosidosis with bilateral thalamic involvement in brain imaging. GM2-Gangliosidosis (Sandhoff and Tay Sachs disease): Diagnosis and Neuroimaging Findings (An Iranian Pediatric Case Series): Karimzadeh P, Jafari N, Nejad Biglari H, Jabbeh Dari S, Ahmad Abadi F, Alaee MR, Nemati H, Saket S, Tonekaboni SH, Taghdiri MM, Ghofrani M - Iranian journal of child neurology (2014). Not altered. CC.