BOWMAN LECTURE. CXVII 



vision very bad in all, and they all Lave more or less 

 nystagmus; O.Ds. much alike in all, pale all over in the 

 adults, but especially so on Y.S. side; in the children 

 nasal side is fairly coloured, but Y.S. side quite pale. 

 None of those affected, from the grandmother (II, 2) to 

 the youngest (IV, 10) are getting either worse or better. 

 No consanguinity. 



Apart from early deaths, we do not meet with any 

 very prevalent morbid tendencies in these families. The 

 most frequent seem to be epilepsy and aggravated 

 hysteria ; such conditions are recorded in several families, 

 sometimes in the subject of the Leber's disease, some- 

 times in a sibling or a maternal relative. In one case 

 Basedow's disease occurred in the sister of a man with 

 Leber's disease.^ Insanity or mental defect is recorded 

 in three affected brothers, and in one other affected 

 male, whose affected brother was epileptic. Diabetes is 

 mentioned in two or three cases. At least two males, one 

 affected, the other normal, and perhaps a third affected 

 male, were congenitally colour-blindt probably a normal 

 coincidence ; likewise, the association of retinitis pigmen- 

 tosa with Leber's disease, observed by Wider, Coppez, 

 and H. Schmidt, appears to have been purely accidental. 



As to sex, I find about 60 females against about 300 

 males. J It has been said that in females the disease 

 tends to come on with special frequency at about the 

 climacteric, but little evidence of this can be found. 

 Of the 57 affected females the disease came on before the 

 age of 13 in 13 cases, almost equally spread over the 



* Case mentioned (on Liebreich's authority) by A. Terson in his 

 article " Maladies de 1'CEil " in the Traite de Chirurgic of Dentu-Delbct, 

 T.V., 1897, p. 198. 



t Cases 106, 117, 147. 



+ The exact number depends upon the inchision or exclusion of a few 

 doubtful cases. There are probably more than 300 males to 60 females ; 

 small pedigrees with only males affected are relatively common and not 

 always recorded, but cases in females have been more generally published 

 on account of their rarity. Perhaps some few of the cases in females that 

 I have included would be rejected as atypical by others. 



