Abiotrophic ophthalmoplegia externa.

THE various names given to the condition of ptosis and progressive ophthalmoplegia first described by von Graefe (1868), reflect views regarding the undecided aetiology of this condition, e.g. chronic progressive nuclear ophthalmoplegia, ocular myopathy, progressive muscular dystrophy involving the extra-ocular muscles, etc. M6bius (1900) had named the condition "progressive nuclear ophthalmoplegia", as he believed there was an absence of the nuclei of the ocular nerves, but Fuchs (1890), 10 years earlier, basing his opinion on biopsy material, had propounded a myopathic cause, as did Gourfein (1896). Langdon and Cadwalader (1928) and Jedlowski (1943) thought that the condition was due to degeneration of the nuclei, and McMullen and Hine (1921) suggested defective nuclear development. Trauma (Senita and Fisher, 1958) and autonomic dysfunction (Sunaga, 1927) have also been put forward. In recent years, however, several cases have been reported where biopsy material from the extra-ocular muscles has indicated that the condition is a myopathy (Sandifer, 1946; Beckett and Netsky, 1953; Schwarz and Liu, 1954; Kirschbaum and Holland, 1958) and most recently, on the basis of simultaneous biopsies from the extra-ocular and skeletal muscles, it has been suggested that the myopathy in the ocular muscles is a local manifestation of a generalized muscular dystrophy (Gartner and Billet, 1949; Kiloh and Nevin, 1951; McAuley, 1956; Nicolaissen and Brodal, 1959). Mention might be made at this point of a case reported by Elliot (1939) of generalized muscular wasting associated with ptosis and external ophthalmoplegia. Elliot considered this to be a myopathy though biopsy evidence was lacking. In general, the hereditary features of dystrophic ophthalmoplegia are weak and uncertain (Adams, Denny-Brown, and Pearson, 1953). Holmes (1956) suggested that the pathology of this interesting condition might be elucidated by electromyography of the affected muscles, and recent