Megalencephaly

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Abstract
In 2 mentally subnormal children dying at ages 2 1/2 and 3 1/2 yrs., the brains were "dry," covered with large, plump convolutions, and weighed 1,644 and 1,502 gm. respectively (normal 1080 gm.) ; gray and white matter preserved normal proportions. The outstanding pathologic features were histologic and consisted of a well-proportioned and harmonious growth of nerve-cells and fibers, without gliosis or other interstitial reaction, but with a peculiar, generalized dysplasia of the ganglion-cells of the different cortical layers, notably the 3d and 4th; this consisted in a flattening or lateral shrinkage and elongation of the cytoplasm, its homogeneous coloration and the absence of Nissl granules, and also in a similar oval lengthening of the nucleus, which stained very deeply and was not normal in structure. The facts that the brain is seldom enlarged (and when enlarged is not functionally impaired) in acromegaly, and that megalencephaly is seldom accompanied by macrosomia, or anencephaly or micrencephaly by microsomia, indicate that the growth of the brain is not subject to the same endocrine influences as the growth of the body. Developing G. H. Parker''s theory that neural control may be exerted by means of hormones secreted by the nerve terminals, the author suggests that the brain may contain within itself the apparatus for regulating its own growth, thus acting, in fact, as "its own gland.".

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