170 OLIVER — BLINDNESS FROM MALFORMATION OF SKULL. [April 5, 



order. The pupils are but slightly, if at all, oversized. The irides 

 are prompt to light-stimulus, efforts for accommodation, and con- 

 vergence. The ciliary muscles are active. The eye-grounds, in 

 every detail of neuronic, vascular, and lymph structure, appear 

 normal j in fact, the eyeballs, with their entire adnexa, are healthy 

 and perform their functional duties properly. 



This complexus of symptoms, with its absolute blindness and 

 concomitants of slight globular protrusion, divergence and the 

 rotary nystagmus as the only ocular signs, constitute a most remark- 

 able clinical picture. In it is seen a blindness, the proving of which 

 necessitates a careful study of every possible direct and indirect 

 ocular detail ; a blindness that, from the ocular signs and associated 

 conditions, may be assumed as intracranial in type, and most 

 probable, until autopsy proves to the contrary, mainly cortical in 

 character. 



The accompanying reproduction of a photograph (Plate XX, Fig. 

 3) of a case recently studied by me and described in full elsewhere,^ 

 gives an excellent idea of the cranial deformation and the peculiar 

 facial appearances and expression in an American-born type of case 

 of this character. In this child the optical and receiving portions of 

 the visual apparatus were apparently perfect. No visual perception, 

 however, could be evolved in this case, no matter how centrally the 

 impression reached (surely in this case back to the midbrain). 

 Cortex sensation was lost ; the discharging station was functionless.^ 



The cases thus far described exhibit but little, if any, mental 

 involvement. The grossest of the resultant disturbances are mainly 

 basilar in character, and in measure affect the vascular channels, 

 the lymph cavities and the coarse nerve fibrils as they pass through 

 both the primary and the secondary foramina. Trophic ocular dis- 

 order soon takes place ; ophthalmic irritation signs and palsies early 

 appear ; sensory changes in the organs of vision quickly ensue ; and, 

 sooner or later, the main portions of the receiving, transmitting 

 and discharging parts of the visual apparatus degenerate and become 

 useless. Should the main distortions be situated in the anterior 

 and central portions of the cranial base, producing antero-midbrain 

 disorder, as in the first illustrative case, the more frequently in- 



^ The American Journal of the Medical Sciences, January, 1902. 



2 It is probable that cases of the badly termed condition " amaurotic fam- 

 liy idiocy," with their peculiar lesions in the fundus of each eye, have some such 

 similar origin. 



