Abstract
Making the point that subsequent involvement of the ocular muscles rarely complicates the regional or generalized weakness with which progressive muscular dystrophy presents, and then only in cases of Landouzy-Dejerine myopathy (facioscapulohumeral dystrophy) or myotonic dystrophy, (Leslie) Kiloh (1917–1997) and (Sam) Nevin (1905–1979) have noted ‘a number of cases of myopathy … in which ocular involvement was the primary and predominant feature’. ‘In some … only the levators of the upper lids were affected, but in others a complete external ophthalmoplegia developed over many years. The question arises whether these cases constitute a clinical group justifying the title ocular myopathy or whether they are merely rare atypical forms of muscular dystrophy … the pathology of those cases described as chronic progressive external ophthalmoplegia [is] considered by most authors to result from degeneration of the oculomotor nuclei, although no clear pathological anatomy has been established …’. They describe five cases.