amaurotic familial idiocy


amaurotic

 [am″aw-rot´ik] pertaining to, or of the nature of, amaurosis.amaurotic familial idiocy former name for lipofuscinosis." >neuronal ceroid lipofuscinosis.

idiocy

 [id´e-ah-se] old term for profound mental retardation, now considered offensive.amaurotic familial idiocy former name for neuronal ceroid-lipofuscinosis.mongolian idiocy former name for Down syndrome or the marked mental retardation associated with it. The term is now considered offensive.

amaurotic familial idiocy

Batten-Mayou disease, neuronal ceroid-lipofuscinosis Molecular medicine An AR inborn error of metabolism characterized by neuronal deterioration Onset Age 5 to 10, rapid deterioration of vision and slower deterioration of intellect, accompanied by seizures and psychotic behavior

am·au·rot·ic fa·mil·i·al id·i·ocy

(am'aw-rot'ik fă-mil'ē-ăl id'ē-ŏ-sē) Family of recessive genetic diseases marked by accumulation of lipid-containing cells in viscera and nervous system, and by mental retardation and impaired vision.

amaurotic familial idiocy

An out-of-date term for a range of rare hereditary diseases, now known as the GANGLIOSIDOSES. Progressive degeneration occurs in the brain and the retinas.