Pediatrics. . entire nucleusappears to have dropped out. The disintegration of the cell-bodyis so complete that in many specimens the contour alone enablesone to infer that these round bodies are the remnants of ganglioncells. These metamorphosed ganglion cells are frequently sur-rounded by distinct pericellular spaces and are relatively and ab- 14 B. SACHS solutely so much enlarged that they give a very striking appear-ance to the cross section. There is no doubt an entire agreement between the findings inthis last case of mine and those so well reported by Dr. Hirsch be-fore this Association
Pediatrics. . entire nucleusappears to have dropped out. The disintegration of the cell-bodyis so complete that in many specimens the contour alone enablesone to infer that these round bodies are the remnants of ganglioncells. These metamorphosed ganglion cells are frequently sur-rounded by distinct pericellular spaces and are relatively and ab- 14 B. SACHS solutely so much enlarged that they give a very striking appear-ance to the cross section. There is no doubt an entire agreement between the findings inthis last case of mine and those so well reported by Dr. Hirsch be-fore this Association a number of years ago. Curiously enough,other recent writers have not been struck by these changes in thegray matter of the brain and spinal cord. In a recent and prelim-inary report by Prof. SchafTer of Buda-Pest there is the account ofa typical case and in the report of the post-mortem findings specialst ress is laid upon changes of the white fibers and nothing is said • ? •? ? - v •*. * Wr^^^,-. Fig. 4—Section through cervical enlargement showing cell changes inanterior gray matter. (Van Gieson stain.) about the gray matter, except that it is entirely normal, and yet,from the cut (Fig. 2) which accompanies his article, although it isreproduced at a low power, I suspect that these same large cell-bodies are present, such as Hirsch and myself have found and de-scribed. The chief inference to be drawn from a study of sections laidthrough every part of the central nervous system in this and 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
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