Fine Structure of Spongy Degeneration of the Central Nervous System (van Bogaert and Bertrand Type)*†

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Abstract
The present study describes the fine structure of cortex and white matter of a one year old Jewish boy afflicted with spongy degeneration of the brain. The most characteristic clinical and pathologic feature of this disease is megalencephaly which is due to increased intracellular water content in the brain, mainly in the subcortical white matter. The light microscopic picture was similar to that previously reported. Electron microscopically, multiple vacuoles in the subcortical white matter and deep cortical lamina (fusiform layer) were due to separation of the lamellae of myelin. The vacuoles were present between the major dense lines of the myelin membranes, and it is conjectured that the obscured intraperiod lines might be the origin of these vacuoles. The extracellular spaces in the subcortical white matter were widened due to the rupture of the myelin lamellae and astrocytic cell membranes. It is hypothesized that a secondarily increased extracellular water content in the white matter might have accelerated further degeneration in the white matter at the advanced stage. There were smaller vacuoles present in the cortex, especially in the ganglionic layer which were the result of marked enlargement of astrocytes and their processes, although no increased extracellular spaces or ruptured cell membranes were present. The large and pale astrocytic nuclei described as Alzheimer type II by light microscopy appeared ultramicroscopically also enlarged and were seen to contain sparse nucleoplasmic granules, loss of chromatin and distinct nucleoli with preserved nucleolonema. Marked changes in size, and frequently in appearance, of mitochondria were observed in the processes of the astrocytes in the cortex, especially in the ganglionic layer. Histochemical studies showed that the activity of ATPase within these mitochondria was decreased when compared with that of normal brain tissue. The fine structure of the neurons, oligodendroglia and blood vessels was not altered.

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