Spongy degeneration of the central nervous system (van Bogaert-Bertrand type?) in a newborn infant. A light and electron microscopic study.
Autopsy findings on a 5 day old infant with hypotonia from birth showed extensive spongy changes of the myelinating tracts within the cerebrum, cerebellum and brain stem. The spongy changes, similar to Van Bogaert-Bertrand disease, resulted from intramyelinic edema. However, unlike the typical forms of this disease, swollen astrocytes with abnormal mitochondria were not found. The relationship of this case to typical forms of Van Bogaert-Bertrand disease is discussed. This case may represent a very early form of Van Bogaert-Bertrand disease or a new pathological entity.
D001253:Astrocytes / Q000473:pathology; D001921:Brain / Q000473:pathology*; D001929:Brain Edema; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008064:Lipidoses / Q000473:pathology*; D008297:Male; D009186:Myelin Sheath / Q000648:ultrastructure