Painful ophthalmoplegia

Abstract

Twenty-two cases of "painful ophthalmoplegia" are presented. In all cases the onset was characterized by pain. The condition was noted to be predominantly unilateral. Varying combinations of 3rd, 4th, 6th nerve, ophthalmic division of 5th nerve and optic nerve involvement were recorded. The pupils were relatively normal in most cases in spite of 3rd nerve involvement; the cocaine test was positive in some of these cases, demonstrating that the oculosympathetic was also involved. Spontaneous remissions and recurrences were features of this disease. The response to systemic steroid therapy was dramatic in all the cases so treated. It is suggested that this can be used as a diagnostic test, in cases presenting with such a clinical history. The differential diagnosis is discussed. Extensive investigations including angiography, pneumoencephalography and surgical exploration in 1 case did not reveal any lesion outside the cavernous sinus. Based on a single previous autopsy report and the angiographic findings in 1 of our cases it is presumed that the pathological lesion is in the cavernous sinus, and is a process of indolent inflammation. The raised ESR, the eosinophilia, the positive LE cell phenomenon, and the positive test for rheumatoid arthritis factor, found in some of these patients, and the dramatic response to steroids, suggest that the inflammatory process may be related to an altered immune mechanism. Whether this is related to co-existent infections and infestations such as tuberculosis or filariasis is a point to be further investigated. Detailed immunological study of these cases might well be fruitful.