A form of sphingolipidosis, transmitted as an autosomal recessive, in which there is an accumulation in neuronal cells of the neuraminic fraction of gangliosides; manifested clinically within the first few months of life by hypotonia progressing to spasticity, convulsions, and visual loss accompanied by the appearance of a cherry-red spot at the macula lutea. Also known as infantile amaurotic familial idiocy.