Abetalipoproteinemia (Bassen-Kornzweig syndrome)
- 1 January 1977
- journal article
- research article
- Published by Springer Nature in Acta Neuropathologica
- Vol. 37 (3) , 255-258
- https://doi.org/10.1007/bf00686887
Abstract
A 26-year old male suffered from Abetalipoproteinemia (Bassen-Kornzweig disease). In addition to C.N.S. lesions, he had myopathy. A muscle biopsy revealed accumulation of ceroid pigment in the striated muscle. A few muscle fibers showed severe degeneration of the myofibrils. Fibroblasts and macrophages in the interstitial tissue contained abundant ceroid. The relation between ceroid accumulation, abetalipoproteinemia and vitamin E-deficiency is discussed.Keywords
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