CLXXXIV BOWMAN LECTURE. 



atrophy ; the uncle improved. Also a great uncle on mother's side was 

 affected. Four cases, all males. 



Case 181. E. N. (Littlechild). St. Thomas's Hospital (Out-Patient's 

 Boole, v, p. 133), February to May, 1890. 



Blindness from birth with optic neuritis and large skull in three sib- 

 lings. Probably not a true case of Leber's disease. Parents, first cousins, 

 had 8 children and no miscarriages. 



No history of blindness in rest of family, except, perhaps, in a male, 

 "second cousin" of I, 3 said to have been blind all his life. I, 1 and 2 

 good health. No history of syphilis. 



II, 1 and 2 good sight and health ; one of them squints ; 6 and the 

 next born, which by mistake is not shown and should be 1, also healthy and 

 see well. II, 4 died at 7 weeks, but could see. 



II, 3, quite blind from birth ; taken to Middlesex Hospital when a baby 

 and told "the nerve was inflamed." February 28th, 1890, set. 7 years; 

 Ps. motionless before mydriatic, but dilate widely after its use ; L. O. D. 

 seen with difficulty ; it is ha.zy, and one vein decidedly enlarged, but 

 no swelling and no visible atrophy; E,. not seen; shadows some H., 

 but degree not measured. Cranium rather large, forehead broad and 

 prominent, the eyebrows overhanging the orbits very much, so that the 

 eyes are extremely sunken and look small, although really of normal 

 size ; nose short ; face well formed ; speaks well, and seems intelligent. 



II, 5 was blind from birth ; died at 15 months of age ; no particulars. 



II, 8 (erroneously marked 7) brought in February, 1890, set. 7 months. 

 Appeared to have no p.L, and mother said she was certain the child 

 had never seen. Her seeing children had all noticed the light very 

 soon after birth ; this one never did so at all. February 28th, 

 1890 : Pupils small, equal and motionless to light ; irregular slow 

 nystagmic movements and frequent strong convergence of eyes. O.Ds. 

 swollen and very hazy, and veins tortuous. Head large and square, 

 fontanelle open, frontal eminences square ; ribs slightly beaded ; spleen 

 1 }, in. below costal margin ; for some weeks past head sweating ; suckled, 

 but for the last two months some bread and oatmeal in addition ; has 

 had no illness and no fits. Thoiigh quite blind the child screws up 

 her eyes in sunlight, but takes no notice of lamplight. Last seen in 

 May, 1890, in statu quo. 



Case 181a.* Eampoldi, Ann. di Ott., xii, pp. 269-271. 



1, 1 blind of " gutta serena " at 35, dead at date of record ; II, 1 good 

 sight ; II, 2 became blind at 35 and II, 3 between 35 and 40, also of 

 " gutta serena " ; III, 3, optic atrophy came on in E., soon followed by L. 

 early in 1883, set. 67 years, E. going to complete blindness, L. not so 

 severe ; had an attack of gastro-enteritis with some loss of blood two or 

 three years before eyes failed. Ill, 4 living, good sight ; III, 5 living 

 but blind, probably of same disease ; III, 6, set. 73 years at record and 

 quite blind ; sight failed from the same disease at 65. IV, 1 set. 33 

 years, good sight ; IV, 3, set. 31 years, nearly blind, age of onset not 

 stated ; is married. 



