Pediatrics. . d inother cases of amaurotic family idiocy is that the morbid processin this- disease affects primarily, or at least to a great extent, thtentire gray matter of the brain and of the spinal cord. The degen-eration of the white fibers of the anterior and lateral pyramidaltracts is in all probability secondary, but not nearly so great or somarked as one would expect with such advanced disease of the ON AMAUROTIC FAMILY IDIOCY 15 ganglionic tissue. It is already probable that the relative involve-ment of the gray and white matter may vary in different subjectsof this disease. The neu
Pediatrics. . d inother cases of amaurotic family idiocy is that the morbid processin this- disease affects primarily, or at least to a great extent, thtentire gray matter of the brain and of the spinal cord. The degen-eration of the white fibers of the anterior and lateral pyramidaltracts is in all probability secondary, but not nearly so great or somarked as one would expect with such advanced disease of the ON AMAUROTIC FAMILY IDIOCY 15 ganglionic tissue. It is already probable that the relative involve-ment of the gray and white matter may vary in different subjectsof this disease. The neurone theory has received so many severe blows of latethat the somewhat anomalous condition of affairs as observed inthis case need not be especially dilated upon. The theory may haveto go to the wall in part or as a whole, but the facts will stand andwill call for further elucidation. In 1887 I spoke of these changes as representing an ageneticcondition pure and simple affecting the highest cortical nerve ele-. Fig. 5—Section through a lumbar ganglion. Nissl stain showingmarked cell changes. ments. At that time I was not aware of the widespread character ofthe morbid process. Later researches, and particularly those ofKingdon and Russell and of Hirsch, have laid stress upon the de-generative character of the disease. Much has been made of the factthat the present author has recorded the process as essentially anarrest of development, while the other writers just mentioned haveinsisted upon the purely degenerative and acquired character ofthe disease. Some writers have accepted these expressions of opin-ion as though they indicated a widely differing conception of 16 B. SACHS amaurotic family idiocy, but the difference between the views ofthe authors just mentioned and of myself is not so great as itwould appear at first sight. A degenerative process does not necessarily represent an ac-quired or an acute affection. It is my contention now, as in formeryears, that degeneration
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