neuronal ceroid lipofuscinoses

A group of rare autosomal recessive neurodegenerative disorders characterized by the abnormal accumulation of the pigmented material LIPOFUSCIN in tissues. The group forms the commonest neurodegenerative disorder of childhood. There is progressive destruction of retinal cell function with visual loss and deterioration of general brain function. The disease may start in early infancy, childhood or in adult life. In the latter case vision is retained but there is slow progressive dementia.

单词	neuronal ceroid lipofuscinoses
释义	neuronal ceroid lipofuscinoses neuronal ceroid lipofuscinoses A group of rare autosomal recessive neurodegenerative disorders characterized by the abnormal accumulation of the pigmented material LIPOFUSCIN in tissues. The group forms the commonest neurodegenerative disorder of childhood. There is progressive destruction of retinal cell function with visual loss and deterioration of general brain function. The disease may start in early infancy, childhood or in adult life. In the latter case vision is retained but there is slow progressive dementia. AcronymsSeeNCLS
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