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Show Journal of Climcal Neuro- ophthalmology 9( 3) 181- 183, 1989. Ophthalmoplegic Migraine with Superior Ramus Oculomotor Paresis Barrett Katz, M. D., and Steve Rimmer, M. D. ( 1;) 1989 Raven Press, Ltd., New York We report a 13- year- old girl with recurrent paroxysmal superior division oculomotor palsy as a manifestation of ophthalmoplegic migraine. Neurodiagnostic tests ( including head computed tomography scan, cerebral angiography, and Tensilon tests) were normal. This case demonstrates that ophthalmoplegic migraine may selectively affect superior division fibers of the oculomotor nerve. Key Words: Migraine- Oculomotor nerve- Cranial neuropathy- Ophthalmoplegic migraine. From the Departments of Ophthalmology ( B. K., S. R.), and Neurosciences ( B. K.), University of California at San DIego, La JoIla, California. Address correspondence and reprint requests to Barrett Katz, M. D., UCSD Eye Center M- 018, La JoIla, CA 92093, U. S. A. 181 Ophthalmoplegic migraine is a syndrome characterized by the typical history of vascular headache presenting during childhood, ophthalmoplegia, and the exclusion of other causes by arteriography, surgical exploration, or autopsy ( 1). Ophthalmoplegia may involve the third, sixth, or fourth cranial nerves ( 2). Recurrent internal or external ophthalmoplegia without headache may also be a variant of ophthalmoplegic migraine ( 3,4). We describe a patient with ophthalmoplegic migraine characterized by superior division paresis of the oculomotor nerve. To the best of our knowledge, this presentation has not been previously reported. CASE REPORT A 13- year- old Hispanic girl developed the sudden onset of ptosis of the left upper lid and diplopia on upward gaze. Five days after the onset of ptosis she noted a mild, pounding, left periorbital headache accompanied by tearing and photophobia. She denied nausea, vomiting, sonophobia, visual disturbance, or other neurologic symptoms. She described three previous episodes of painless left ptosis without diplopia ( photographically documented) at ages 9, 10, and 11, each episode lasting approximately 48 h. Episodes resolved without sequelae or residual ophthalmoplegia. There was no family history of migraine. Her examination ( on the sixth day after onset of diplopia) revealed levator palpebrae superioris and superior rectus dysfunction on the left ( Figs. 1 and 2). Her ptosis was incomplete; there was no evidence for pupillary dysfunction or medial rectus involvement. Forced ductions and exophthalmometry were normal. There was no element of fatigueability, no lid twitch, and normal orbicularis strength. The remainder of her ophthalmologic, neurologic, and general physical exam was nor- 182 KATZ AND RIMMER FIG. 1. Demonstration of mild ptosis of the left upper lid and associated loss of elevation of the left eye apparent upon initial evaluation; patient is attempting supraduction. mal. Hemogram was normal, erythrocyte sedimentation rate was 4; monospot, anti- nuclear antibody ( ANA), and rapid plasma reagin ( RPR) were negative, with absence of detectable antibodies to skeletal muscle or acetylcholine receptors. Tensilon tests done in triple- blind fashion ( twice) were negative. Sinus films were normal. Computed tomography ( CT) scan of the head and orbit was normal except for minimal mucoperiosteal thickening of the sphenoid sinus and opacification of several ethmoid air cells. Digital cerebral angiography showed no abnormality of intracranial or orbital compartment. As the left levator and superior rectus dysfunction resolved over the next week, subtle involvement of the medial rectus and pupil became apparent. The patient recovered over the following 6 weeks, without residual ophthalmoplegia or signs of aberrant regeneration, although with 1 mm of ptosis. DISCUSSION In a study of 30 children with oculomotor nerve palsy seen in isolation, Miller ( 5) found the differential diagnosis to include congenital palsy ( 43%), trauma ( 20%), infection and inflammation ( 13%), FIG. 2. Patient as she appeared in primary position, rl,?"",":~,~ tr: otin( l sn; wnq of left medial rectus when first - '- l i riiplopia. I Clin NeuTo- ophthalmol, Vol. 9, No. 3, 1989 tumor ( 10%), aneurysm ( 7%), and ophthalmoplegic migraine ( 7%). Other entities reported to cause solitary oculomotor nerve palsy of childhood include myasthenia gravis, cyclic oculomotor palsy, infectious disease ( such as diphtheria, tetanus, syphilitic meningitis, viral syndromes, mumps, and tick paralysis), mucocele of the sphenoid sinus, Tolosa- Hunt syndrome, measles vaccine, branched- chain ketoaminoaciduria, and nonketotic hyperglycinemia. Could our observation of sinus mucosal thickening recognized on CT scan imply that our patient's oculomotor paresis was a manifestation of underlying inflammation? The clinical presentation of this patient with a history of prior periodic migrainous episodes beginning in late childhood and characterized by throbbing hemicranial headache followed by ophthalmoparesis and eventual recovery supports the diagnosis of ophthalmoplegic migraine. The combination of unilateral ptosis and ipsilaterally impaired elevation is consistent with a superior division oculomotor palsy. As the third nerve passes through the anterior portion of the cavernous sinus, it divides into a superior branch containing fibers to the levator palpebrae superioris and superior rectus muscles, and an inferior branch containing fibers to the medial rectus, inferior rectus, and inferior oblique muscles and parasympathetic fibers to the iris sphincter and ciliary muscle. Isolated paresis of the superior division is uncommon. Guy et al. ( 6) described five patients with an isolated superior division oculomotor nerve palsy in which diagnostic studies suggested oculomotor nerve involvement proximal to its expected anatomic bifurcation into superior and inferior divisions. These authors suggested the oculomotor nerve may undergo a functional organization of fibers into superior and inferior components prior to its anatomic bifurcation. The observation of medial rectus and pupillary involvement late in the course of our patient's ophthalmoplegia suggests that the insult was proximal to the nerve's bifurcation. While not likely to be nuclear in origin, the site of involvement could possibly be fascicular, as it is becoming increasingly clear that there is a superior- inferior division of function of the third nerve even within the brainstem. Nonetheless, the pathophysiologic mechanism causing oculomotor dysfunction in our patient first affected those fibers that eventually become the superior division, with later involvement of fibers carried within the inferior division. The mechanism by which migraine causes ophthalmoplegia is uncertain. Walsh and O'Doherty ( 1) suggested that ophthalmoplegia results from OPHTHALMOPLEGIC MIGRAINE 183 compression of the oculomotor nerve by a dilated or edematous vessel, specifically the intracavernous portion of the carotid artery. In this older model, neural ischemia would result from compression of the nerve itself, or from compression of its vasa nervorum. These authors described two cases in which cerebral angiography revealed a narrowing of the distal portion of the carotid artery, suggesting an edematous swollen vessel as the end result of the factors involved in migraine pathophysiology. Such findings, however, are not routinely observed in arteriography of ophthalmoplegic migraine. Ophthalmoplegic migraine may result from vasospasm of a small branch of the internal carotid artery that supplies the oculomotor, trochlear, or abducens nerves. A small meningohypophysial branch of the internal carotid supplies three distal branches: ( a) a tentorial branch that gives small perineural branches to oculomotor and trochlear nerves before it passes into the tentorium, ( b) a dorsal meningeal branch that gives perineural branches to the abducens nerve, and ( c) a medial branch to the posterior hypophysis ( 2). The intracavernous carotid artery gives rise to two small branches that may also supply neural tissue ( 7). Vasospasm of any of these small arteries might result in ischemia of the nerve. Alternatively, a dilated or edematous carotid artery might obstruct blood flow at the origin of one of these small arteries, or compress the artery along its course. In our patient, the initial presentation of superior division oculomotor palsy might have resulted from ischemia to that portion of the nerve that ultimately becomes the superior division, with subse-quent involvement of those portions of the nerve that supply the pupil and medial rectus. Whether the lesion in ophthalmoplegic migraine is central or peripheral is yet to be determined. O'Day et al. ( 8) described a patient with ophthalmoplegic migraine who developed aberrant regeneration of the oculomotor nerve, consisting of ipsilateral eyelid retraction on downward gaze and constriction of the pupil on attempted adduction and depression of the affected eye. These authors concluded that aberrant regeneration of the oculomotor nerve in ophthalmoplegic migraine suggests the lesion to be in the distal portion of the nerve. We present this case as further evidence that the site of the insult in ophthalmoplegic migraine is not nuclear and may selectively affect superior division fibers of the oculomotor nerve. REFERENCES 1. Walsh Jr, O'Doherty OS. A possible explanation of the mechanism of ophthalmoplegic migraine. Neurology 1960; 10: 1079-- 84. 2. Walsh FB, Hoyt WF. Clinical neuro- ophthalmology. 3rd ed., vol. 2. Baltimore: Williams & Wilkins, 1969: 1674- 8. 3. Edelson RN, Levy DE. Transient benign unilateral pupillary dilation in young adults. Arch Neurol 1974; 31: 12-- 4. 4. Durkan GP, Troost BT, Slamovits TL, Spoor Te Kennerdell JS. Recurrent painless oculomotor palsy in children. A variant of ophthalmoplegic migraine' Headache 1981; 21: 58- 62. 5. Miller NR. Solitary oculomotor nerve palsy in childhood. Am J Ophthalmol 1977; 83: 106-- 11. 6. Guy]. Savino PJ, Schatz N]. Cobbs WH, Day AL. Superior division paresis of the oculomotor nerve. Ophthalmology 1985; 92: 777-- 84. 7. Parkinson D. Collateral circulation of cavernous carotid artery: anatomy. Can J Surg 1964; 7: 251--{' 8. 8. O'Day J, Billson F, King J. Ophthalmoplegic migraine and aberrant regeneration of the oculomotor nerve. Br I Ophthalmol 1980; 64: 534- 6. J Clill Nellro- ophthalmol. Vol. 9. No. 3. 1989 |
