Signs and symptoms of<unk>  are identical with those of infantile Tay–Sachs disease, except that enzyme assay testing shows normal levels of hexosaminidase A. Infantile Sandhoff disease has similar symptoms and prognosis, except that there is deficiency of both hexosaminidase A and hexosaminidase B. Infants with this disorder typically appear normal until the age of 3 to 6 months, when development slows and muscles used for movement weaken. Affected infants lose motor skills such as turning over, sitting, and crawling. As the disease progresses, infants develop seizures, vision and hearing loss, mental retardation, and paralysis. An ophthalmological<unk> normality called a cherry-red spot, which can be identified with an eye examination, is characteristic of this disorder. This cherry-red spot is the same finding that Warren Tay first reported in 1881, when he identified a case of Tay–Sachs disease, and it has the same etiology. The prognosis for<unk><unk>  is the same as for infantile Tay–Sachs disease. Children with<unk><unk>  die in infancy or early childhood.
GM2-gangliosidosis, AB variant
GM1 gangliosidoses
GM2 gangliosidoses
Adult polyglucosan body disease
Mucopolysaccharidosis type I
Glycogen storage disease type 0
Mucolipidosis type IV
Griscelli syndrome type 2