An autosomal recessive neurodegenerative disorder characterized by the onset in infancy of an exaggerated startle response, followed by paralysis, dementia, and blindness. It is caused by mutation in the alpha subunit of the HEXOSAMINIDASE A resulting in lipid-laden ganglion cells. It is also known as the B variant (with increased HEXOSAMINIDASE B but absence of hexosaminidase A) and is strongly associated with Ashkenazic Jewish ancestry.

Descriptor ID	D013661
MeSH Number(s)	C10.228.140.163.100.435.825.300.300.500 C16.320.565.189.435.825.300.300.500 C16.320.565.398.641.803.350.300.850 C16.320.565.595.554.825.300.300.840 C18.452.132.100.435.825.300.300.500 C18.452.584.687.803.350.300.850 C18.452.648.189.435.825.300.300.500 C18.452.648.398.641.803.350.300.850 C18.452.648.595.554.825.300.300.840
Concept/Terms	Tay-Sachs Disease Tay-Sachs Disease Tay Sachs Disease G(M2) Gangliosidosis, Type I Gangliosidosis GM2, B Variant Gangliosidosis GM2, Type I GM2 Gangliosidosis, B Variant GM2 Gangliosidosis, Type 1 GM2 Gangliosidosis, Type I Hexosaminidase A Deficiency Disease Sphingolipidosis, Tay-Sachs Sphingolipidosis, Tay Sachs Tay-Sachs Sphingolipidosis Tay-Sachs Disease, B Variant Tay Sachs Disease, B Variant B Variant GM2-Gangliosidosis B Variant GM2-Gangliosidoses GM2-Gangliosidoses, B Variant GM2-Gangliosidosis, B Variant Variant GM2-Gangliosidoses, B Variant GM2-Gangliosidosis, B GM2-Gangliosidosis, Type I GM2-Gangliosidoses, Type I I GM2-Gangliosidoses, Type I GM2-Gangliosidosis, Type Type I GM2-Gangliosidoses Type I GM2-Gangliosidosis B Variant GM2 Gangliosidosis Deficiency Disease Hexosaminidase A Gangliosidosis G(M2), Type I Gangliosidosis GM2 , Type 1 Hexosaminidase alpha-Subunit Deficiency (Variant B) Hexosaminidase alpha-Subunit Deficiency (Variant B) Deficiencies, Hexosaminidase alpha-Subunit (Variant B) Deficiency, Hexosaminidase alpha-Subunit (Variant B) Hexosaminidase alpha Subunit Deficiency (Variant B) Hexosaminidase alpha-Subunit Deficiencies (Variant B) alpha-Subunit Deficiencies, Hexosaminidase (Variant B) alpha-Subunit Deficiency, Hexosaminidase (Variant B) Hexosaminidase A Deficiency Deficiencies, Hexosaminidase A Deficiency, Hexosaminidase A Hexosaminidase A Deficiencies Amaurotic Familial Idiocy Amaurotic Familial Idiocy Familial Amaurotic Idiocy Amaurotic Idiocy, Familial Idiocies, Familial Amaurotic

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