Familial idiocy with spongy degeneration of the central nervous system of van Bogaert—Bertrand type

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Abstract
A case is presented. The salient clinical features of this disease are onset in early infancy, atonia of the neck muscles, hyperextension of legs and flexion of arms, blindness, severely defective mental development, megalocephaly, and death at an average age of 18 months. Principal pathologic findings include a spongy degeneration of the white matter extending through the U-fibers into the cerebral cortex, a severely defective myelin pattern, proliferation of astroglial cells, presence of Alzheimer II cells, and lack of glial sclerosis.