Neuronal ceroid lipofuscinoses (NCL) is the general name for a family of at least eight genetically separate neurodegenerative disorders that result from excessive accumulation of lipopigments (lipofuscin) in the body`s tissues. These lipopigments are made up of fats and proteins. Their name comes from the word stem lipo-, which is a variation on....
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http://en.wikipedia.org/wiki/Neuronal_ceroid_lipofuscinosis
A heritable lipidosis with cytoplasmic inclusions staining for ceroid and lipofuscin. Clinically the patient has progressive dementia, retinal degeneration, seizures, and myoclonic jerks. It was formerly thought to be related to tay-sachs disease but the biochemical defect is as yet undetermined. Named according to age of onset: jansky-bielschowsky...
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http://www.encyclo.co.uk/local/20973
Type: Term Definitions: 1. a group of diseases characterized by accumulation of abnormal pigments in tissue (previously classified as cerebral sphingolipidoses). Major subtypes include chronic juvenile form (Batten disease), slowly progressive behavior and visual symptoms, autosomal recessive inheritance; acute, late infantile form (Bielschowsky di...
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http://www.medilexicon.com/medicaldictionary.php?t=50781
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