Familial Ocular Myopathy and External Ophthalmoplegia

INVOLVEMENT of the oculomotor muscles in the course of a generalized muscular dystrophy is not common. Even more unusual is a dystrophy limited to the ocular muscles without evidence of muscular involvement elsewhere in the body. A few such cases have been described by Kiloh and Nevin.¹We are reporting the clinical and pathologic observations on a patient who had this syndrome, and also the clinical findings in four members of his family. These cases form an entity, little recognized clinically, and in addition have a bearing on the subject of "chronic progressive nuclear ophthalmoplegia." REPORT OF CASES Case 1.— A. X., a white man aged 68, had been well until about 20 years of age, when he noted the onset of slight ocular ptosis. This became worse slowly and progressively over the course of many years. Sometime after the onset of ptosis, slight external ophthalmoplegia occurred, which also