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amaurotic familial idiocy |
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amaurotic familial idiocy. See Tay-Sachs disease. 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. 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
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