An autosomal recessive metabolic disease caused by a deficiency of cerebroside-sulfatase leading to intralysosomal accumulation of cerebroside sulfate (sulfoglycosphingolipids) in the nervous system and other organs. Pathological features include diffuse demyelination, and metachromatically-staining granules in many cell types such as the GLIAL cells. There are several allelic and nonallelic forms with a variety of neurological symptoms.


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