Spongy degeneration of the white matter of the central nervous system associated with hyperglycinuria.
نویسنده
چکیده
Five infants from two families with the clinical features of hyperglycinaemia and hyperglycinuria are described. In four of these cases spongy degeneration of the central nervous system is associated with lipid-filled glial cells and retarded myelination. The origin of these changes is discussed and the relationship of the lesions to the metabolic defect is reviewed. The importance of such cases in the understanding of the normal metabolism of the nervous system is stressed.
منابع مشابه
Infantile spongy degeneration of the central nervous system associated with glycogen storage and markedly fatty liver.
The clinical, biochemical, and pathological features of an unusual expression of infantile spongy degeneration of the central white matter are presented with emphasis on neuropathological observations. The topographical distribution of the spongy change along with the observed defect in myelination were such as to suggest an arrest in development of the white matter in late foetal life. Of addi...
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عنوان ژورنال:
- Journal of clinical pathology
دوره 21 4 شماره
صفحات -
تاریخ انتشار 1968