The hallmark of degenerative disorders of the nervous system is the progressive loss of previously acquired abilities. In young children, deceleration in the rate of development is often the first sign of degeneration. The patient falls progressively behind other children, and subsequently there is actual loss of previously acquired achievements. This is quantitated as a declining developmental quotient. By definition, this deterioration is not due to an exogenous agent or event, or to secondary involvement of the nervous system by a generalized systemic disease. Most of the degenerative disorders are hereditary, although the biochemical basis of many of these conditions is not fully understood.
KeywordsBiliary Atresia Optic Atrophy Degenerative Disorder Neuronal Ceroid Lipofuscinosis Developmental Quotient
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