Progressive External Ophthalmoplegia - Some Clinical Associations

Three cases of progressive weakness of the external ocular muscles are described. Although there were many common factors, each seemed to represent a distinct clinical entity. Several associations such as cardiac conduction defects, malabsorption, athetosis, myopathy, peripheral neuropathy, optic atrophy, posterior column dysfunction and cerebellar ataxia were noted in different cases. Although muscle biopsies were done, the paper illustrates the difficulty in establishing the cause of the muscle atrophy. In this context the term progressive external ophthalmoplegia is appropriate, as it implies a clinical state and does not presume the underlying pathological change. In view of the absence of a clear genetic basis for disease processes under scrutiny, I wondered whether this progressive assault on the anatomy could be the result of an infection with some slow virus. There was nothing to support an infective basis for the illness in any of the investigations. It is of interest that the C.S.F. protein was 92 mg/100 ml in one case. The mechanism of an attack by slow viruses is still a matter of conjecture.