amaurotic familial idiocy


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amaurotic

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

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An out-of-date term for a range of rare hereditary diseases, now known as the GANGLIOSIDOSES.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.

amaurotic

pertaining to, or of the nature of, amaurosis.

amaurotic familial idiocy
a group of hereditary disorders in humans characterized by cerebromacular degeneration, blindness, progressive dementia, and progressive and unremitting paralysis. The term has been applied to ceroid lipofuscinosis in English setters and Chihuahuas which have very similar clinical features.