Spongy Degeneration of the Central Nervous System and Other Forms of Spongy Myelinopathy

摘要: The hereditary infantile disease known as spongy degeneration of the central nervous system van Bogaert-Bertrand type belongs to a group conditions characterized by microvacuolar, state affecting predominantly, but not exclusively, white matter. Sponginess tissue, or status spongiosus, revealed light microscope, is specific tissue alteration. Taken on its own merit, it an arbitrary criterion for classification entities. However, spongiosus was shown electron microscopic examination be result alteration in fine structure myelin, consisting accumulation large pockets fluid between lamellae sheaths. This change needs distinguished from splitting without outpouching sheaths preparative artefact with retrogressive changes Intramyelinic accumulation, myelinopathy, shared otherwise heterogeneous diseases described present chapter.