cerebroside/asthenia

A 38-year-old man presented with weakness of the lower limbs. Electrophysiology revealed a pronounced demyelinating neuropathy. Nerve biopsy disclosed de- and remyelinating lesions and characteristic lamellar inclusions in Schwann cells and macrophages. There was no familial history of neurologic

Metachromatic leukodystrophy is a lysosomal storage disorder caused by deficiency in the sulfolipid degrading enzyme arylsulfatase A (ASA). In the absence of a functional ASA gene, 3-O-sulfogalactosylceramide (sulfatide; SGalCer) and other sulfolipids accumulate. The storage is associated with

Mammalian sulfoglycolipids comprise two major members, sulfatide (HSO3-3-galactosylceramide) and seminolipid (HSO3-3-monogalactosylalkylacylglycerol). Sulfatide is a major lipid component of the myelin sheath and serves as the epitope for the well known oligodendrocyte-marker antibody O4.