Lees (1960) described congenital static familial ophthalmoplegia. Ptosis, almost completely fixed eyes, nystagmoid movements, and unequal pupils were features. Males in 3 successive generations and 7 persons in all were affected. Lees (1960) thought the lesion to be ... Lees (1960) described congenital static familial ophthalmoplegia. Ptosis, almost completely fixed eyes, nystagmoid movements, and unequal pupils were features. Males in 3 successive generations and 7 persons in all were affected. Lees (1960) thought the lesion to be in the posterior longitudinal bundle and its connections with the oculomotor nuclei. Transmission through several generations with male-to-male transmission has been noted by Bradburne (1912) and many others. The palsy is thought to be of nuclear origin. Holmes (1956) described 9 affected members with congenital onset in 4 generations of a family. The families of Bradburne (1912), Holmes (1956), and Lees (1960) suffered from total ophthalmoplegia. Mollica et al. (1980) described a Sicilian family in which congenital external ophthalmoplegia showed wide variability in expressivity, as well as reduced penetrance with 'skipped generations.' They distinguished 3 forms: 1) ophthalmoplegia interna (paralysis of the ciliary muscles and iris); 2) ophthalmoplegia externa (paralysis of all the muscles innervated by the 3rd, 4th and 6th cranial nerves); and 3) total ophthalmoplegia (paralysis of both intrinsic and extrinsic muscles of the eye). Ptosis was the only manifestation in some affected members of the Sicilian family. Some families reported as 'hereditary ptosis' (178300) may represent external ophthalmoplegia. Mace et al. (1971) reported a family consistent with either autosomal or x-linked dominant inheritance. Because the males and females were apparently equally affected and presumably no myopia was present, this was probably the autosomal and not the X-linked disorder (311000).