Progressive Muscular Dystrophy Involving the Extra-ocular Muscles with Notes concerning Operative Treatment of the Associated Divergent Squint.

THE earliest sign in progressive muscular dystrophy of involvement of the extraocular muscles is usually a bilateral ptosis which is followed by slowly progressing immobility of the eyes. The central nervous system as well as the skeletal muscles may be affected. Furthermore, heart disorders have been reported. At present, the disorder is attributed to progressive dystrophy of the extra-ocular muscles and not to degeneration of the nuclei of the third, fourth, and sixth nerves as was thought before. The reader is referred to the papers on the subject by Kiloh and Nevin (1951) and Papst, Esslen, and Mertens (1958), and more recently, Lind and Prame (1963). The present case showed dystrophic changes primarily in the extra-ocular muscles which led to marked restrictions of the ocular movements as well as to divergent squint. The condition was improved by squint surgery. The presenting sign was continuous lacrimation, and practically no ptosis could be observed.