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Show J. Clin. Neuro-ophthalmol. 4: 85-90, 1984. Migrainous Optic Neuropathy MARY O'HARA, CAPT., USA, MC PATRICK S. O'CONNOR, M.D. Abstract Two patients with histories of classic migraine developed ischemic optic neuropathy during a severe headache. Clinical and radiologic studies excluded other causes for the visual field loss. In both cases, the field loss was permanent. No further visual or neurologic disturbances have occurred in either patient during a 31/z- and 21/z-year period of followup, respectively. Acute monocular visual loss due to anterior ischemic optic neuropathy (AlaN) is a well-defined clinical syndrome. The importance and typical clinical presentation of this condition was first emphasized in the American literature by Miller and Smith in 1966, I and the term anterior ischemic optic neuropathy was coined by Hayreh in 1974." The association of AlaN with medium and small vessel angiopathic disorders such as cranial arteritis/.j collagen vascular disease," diabetes mellitus," atherosclerosis, and hypertension? have been well-documented. The association of AlaN with migraine has been rarely reported. K - 11 The clinical features in two such patients are reviewed and compared with previously described cases. Case Reports Case 1 A 27-year-old, right-handed white female had the acute onset of a severe, throbbing headache "like someone cracked my skull with a hammer," in August 1979. The headache lasted for approximately 27 hours, and as it cleared, the patient noticed a "bolt of lightning" in her right temporal field of vision which remained for the next 3 days. Since that time, she has had the feeling that "someone was standing on my right side" and was unable to see in her right temporal field. She continued having intermittent bifrontal head- From the Ophthalmology Service, Department of Surgery, Brooke Army Medical Center, Fort Sam Houston, San Antonio, Texas (MOH); and the Department of Ophthalmology, University of Texas Health Sciences Center, San Antonio, Texas (PSOC). June 1984 aches two to three times weekly. The patient had never taken oral contraceptives. Her past history was of great interest in that she had typical migraine headaches consisting of nausea, photophobia, a pounding headache and occasional vomiting intermittently over the last 10 years. Her mother also suffered from similar headaches. A complete medical and neurological examination carried out elsewhere was normal. Because of persistent field loss and recurrent bifrontal headaches, the patient was referred to us for further evaluation in October 1979. X-rays, including a sinus and skull series, optic canal tomography, and a CT scan with and without contrast, were normal. Laboratory examination, including CBC, sedimentation rate, RPR; SMA-20, FTA-ABS; ANA, and VER was normal. On examination, the patient's best-corrected vision was 20/20 +2 on the right and 20/20 +2 on the left. A 1+ Marcus Gunn pupil was present on the right. Color vision testing, including Ishihara plates and a DIS panel was normal in both eyes. Dilated fundus examination revealed a subtle nerve fiber layer dropout involving the nasal radiating fibers. Goldmann visual field testing demonstrated an absolute temporal defect breaking out of the blind spot. The left visual field was normal (Fig. 1). The patient was started on Inderal 20 mg four times/day. On follow-up in June 1980, her examination was unchanged, except for a marked decrease in the frequency of her headaches. Telephone contact in April 1983 revealed no further difficulties, except for an occasional headache. Case 2 A 64-year-old white woman, with a history of classic migraine dating from puberty, had the acute onset of a severe throbbing headache with transient loss of vision for 10 minutes in November 1980. Subsequently, she was seen for a routine eye examination in November 1981 complaining of a mild persistent blurring in her left eye, especially while reading, which she attributed to a need for new glasses. In the past 20 years, her headaches had become less hemicrania!' and more often, associated with a dull retroorbital ache behind the left eye. There was also a family history of migraine. Skull x-rays and a CT 85 Migraine Figure 1. Case 1: Visual fields demonstrating an absolute wedge-shaped defect breaking out of the blind spot on the right. The left visual field is normal. Figure 2. Case 2: Visual fields demonstrating an incomplete, inferior arcuate, defect on the left. The visual field on the right is normal. scan with and without enhancement were normal. Laboratory evaluation, including a CBC, sedimentation rate; VORL, FTA-ABS, ANA; RA factor, and VER was within normal limits. On examination, the patient's best-corrected acuity was 20/20 on the right and 20/25 on the left, with a 1+ Marcus Gunn pupil present on the left. Fundus examination revealed a subtle superior nerve fiber bundle defect corresponding to 81i the arcuate scotoma found on Goldmann perimetry (Fig. 2). Follow-up examination through May 1983 revealed no change in the optic nerve, vision, or fields. Discussion While anterior ischemic optic neuropathy is a common cause of visual loss in the elderly, it is Journal of Clinical Neuro-ophthalmology Vasculitides Giant cell arteritis Polyarteritis nodosum Systemic lupus erythematosus Buerger's disease Allergic vasculitis Postviral vasculitis Postimmunization Syphilis Radiation necrosis Systemic vasculopathies Hypertension Atherosclerosis Diabetes mellitus Migraine Takayashu's disease Cartoid occlusive disease Hematologic Polycythemia vera Pernicious anemia Sickle cell disease (trait) Acute hypotension (shOCk) G-6-P-D deficiency Ocular Postcataract Low-tension Glaucoma O'Hara, O'Connor Figure 3. Reported causes of anterior ischemic optic neuropathy. (Adapted from Miller·) rare in the young patient. The majority of elderly patients with this disorder fall into two distinct categories, those with cranial arteritis and a larger "idiopathic· group.3,4 In the latter group, hypertension may be an important predisposing factor and is present in more than 50% of the patients. 4 . 7 Diabetes and carotid atheromatous disease, although sometimes present, have not been demonstrated to predispose to idiopathic AION.3.4 Other recognized and potentially preventable causes of AlaN include massive blood loss with hypotension,12 toxemia of pregnancy,13 collagen vascular disease,s and cataract extraction. 14 The typical patient with idiopathic AlaN is 60 years of age or older and experiences the abrupt onset of monocular visual loss. Except in patients with cranial arteritis, symptoms of headache or periocular pain, and other systemic or neurologic signs, do not occur. Such symptoms should suggest an alternative diagnosis. The attendant visual field defect is often altitudinal or arcuate in nature and generally involves fixation. A pallid swelling of the optic nerve is characteristically found in a segmental distribution corresponding to the visual field defect.] Fluorescein angiography has been felt by some to be helpful in demonstrating hypoperfusion of the affected disc and peripapillary choroid.3.10 While simultaneous involvement of both optic nerves is rare in the absence of cranial arteritis, 40% of the patients with the idiopathic form will suffer a similar episode in the second eye within months to several years.!. 4 The diagnosis of migrainous AlaN in our patients is based on a history of long-standing classic migraine headaches, the occurrence of unilateral visual loss during one of these headaches, follow-up of 31f2 years and 21f2 years, respectively, in which no further visual difficulty developed; and a family history of migraine headaches. Other causes of anterior ischemic optic neuropathy (Fig. 3) were reasonably excluded by history and laboratory examination. Numerous other visual complications have been described in association with migraine. These have included bilateral disc edema without June 1984 visual loss or increased intracranial pressure, 15 vitreous and retinal hemorrha§es,10 central retinal arterl 7 and vein occlusion,1 permanent homonymous hemianopia,IY and transient monocular visualloss. 20 To our knowledge, only seven other cases of migrainous anterior ischemic optic neuropathy have been reported.~-l1 Figure 4 summarizes our and previously reported cases of migrainous ischemic optic neuropathy. Although the ages of these cases ranged from 22 to 64 years, there is a preponderance of younger patients. Females outnumber males 4.5 to 1, and the majority had a strong family history of migraine. All except one, had a typical history of migraine headaches for 7-50 years. The one exception was patient no. 4, a 53-year-old male who had a several-year history of zigzag lines and light flashes in his peripheral visual field, consistent with an acephalgic variety of migraine. The right and left eyes were equally affected, and a Marcus Gunn pupil was found in all patients in which this was tested. While two patients were left with a final visual acuity of 20/200, five had vision of 20/40 or better. Persistent visual field defects were present in all patients and included involvement of the papillomacular bundle, arcuate nerve fiber layer, and nasal radiating fibers. Six patients developed optic nerve pallor, while three of the four patients with the best final visual acuity had only nerve fiber layer defects identified. Three patients had pallid swelling when seen shortly after the onset of their visual loss. The remaining six, however, were seen from 2 months to 3 years after the initial episode, and no pallid swelling was identified. Although the precise pathogenesis of these defects is still speculative, a regional ischemic mechanism is generally believed to be present,IY.21 possibly mediated by abnormal platelet aRgregability and/or plasma hypercoagulability.2- Arteriovenous shunting2J 24 and vasospasm at the microcirculary level have been implicated in the ischemic symptomatology of migraine. Vasospasm involving the retinaV" conjunctivaVo and carotid circulation27 have been observed during 87 '0 t: :3 e:o... o enS:°-' z III .t.:. o6 'U:r :r III 3" 2- ~ Age Sex Family Migraine Hislory Eye Marcus Gunn Initial Final Fields Optic Nerve Hislory Pupil Vision Vision Appearance . . o • o • 53 Female PosItive ClassIc migraine 19 years as No comment 619 6/6 ·....~.... G Pallid swelling made 22 Male Unknown ClassIc migraine 7 years as Positive 20120 .~ Nerve I,ber layer defect only Marcus Gunn 36 WMe Unknown 15 year hlSlOry of aD POSItive 201200 20/60 Central scotoma to 212000 W Pallor w,th vessel sheathing Female b,-monthly headaches Marcus Gunn Several year history of 53 White Unknown Zigzag lines and light aD POSitive Poorer 20/40 5' central scotoma to 9 mm red test object Pallor With narrowing of Male flashes Purple haze Marcus Gunn InIllally retinal vessels lasllng 10-15 minutes 46 White Unknown ClaSSIC migraine as POSitive 20160 20160 Generalized contraction With 3' central scotoma to 6/2000 R Temporal pallor Female 27 years Marcus Gunn Female POSitive ClaSSIC mIgraine as PosIlive 36 20 years Marcus Gunn 201200 201200 ~:~ Pallid swelling ClaSSIC migraine aD POSitive 49 Female POSitive 201200 ~. Pallid swelling 20 years Marcus Gunn . . Common migraine POSitIve @Y~ Nerve fIber bundle defect 26 Female POSitive aD 20/20 20120 _...@............ 10 years Marcus Gunn ' ,.- . .. In nasal radlallng fibers -: '. . . White Classic migraine 50 years as POSitive 20/25 20/25 ..~~. Nerve fiber bundle defecl 64 Female POSitive Marcus Gunn supenor arcuate fibers Figure 4. Summary of reported cases of anterior ischemic optic neuropathy secondary to migraine. :s:: Oil' ~ S' III acute episodes. The anatomic basis for the peculiar susceptibility to ischemia at the prelaminar portion of the optic nerve, remains controversial, due to differences of opinion regarding the vascular architecture of the normal optic nerve. Most authors agree that the surface of the disc derives its nutrition primarily from branches of the central retinal artery. The remainder of the prelamina: and laminar portions of the anterior optic nerve is supplied by a richly anastomotic system with contributions from the pial vessels, choroidal arterioles, and the short posterior ciliary arteries.~"- 3J There is no universal agreement, however, on the relative importance of each of these potential blood supplies under normal conditions. Hayreh's~" experimental studies suggest that ligation of a single short posterior ciliary artery will produce segmental infarction of the distal portion of the optic nerve and adjacent peripapillary choroid. Conversely, Anderson and Davis3~ failed to produce substantial circulatory disturbances after ligation of a single posterior ciliary artery. Others have suggested that AlaN, especially when accompanied by hypertension, may be the result of multifocal vascular lesions, such as seen in lacunar hypertensive cerebral vascular disease.? Until the importance of the various sources of blood supply to the normal optic nerve is established, the pathogenesis of anterior ischemic optic neuropathy will remain uncertain. Clinically, however, when one sees a young or middle-aged patient with a history of typical migraine headaches who develops an anterior ischemic optic neuropathy during one of these headaches, migraine should be considered etiologic. References 1. Miller, G.5., and Smith, j.L.: Ischemic optic neuropathy. Am. j. Ophthalmol. 62: 103-115, 1966. 2. Hayreh, S..5.: Anterior ischemic optic neuropathy. Br. j. Ophthalmol. 58: 955-963, 1974. 3. Sanders, M.D.: Ischemic papillopathy. Trans. Ophthalmol. Soc. U.K. 91: 369-386, 1971. 4. Boghen, D.R., and Glaser, j.5.: Ischemic optic neuropathy. Brain 98: 689-708, 1971. 5. Kimbrell, O.C, and Whellis, J,A: Anterior ischemic optic neuropathy in collagen vascular disease. f.A.M.A. 201: 61-62, 1967. 6. Miller, N.R.: AION: Diagnosis and management. Bull. N. Y. Acad. Med. 56(7): 643-654, 1980. 7. Ellenberger, C: Ischemic optic neuropathy and hypertension. Am. j. Ophthalmol. 88: 1045-1051, 1979. 8. McDonald, W.I., and Sanders, M.D.: Migraine complicated by ischemic papillopathy. Lancet 1: 521-523,1971. june 1984 O'Hara, O'Connor 9. Troost, B.T.: Migraine. In Clinical Ophthalmology, T.D. Duane, Ed. Harper & Row, Philadelphia, 1981, pp. 10. Weinstein, J,M., and Feman, S.5.: Ischemic optic neuropathy in Migraine. Arch. Ophthalmol. 100: 1097-1100,1982. 11. Cowan, CL., and Knox, D.L.: Migraine optic neuropathy. Ann. Ophthalmol. 14(2): 164-166, 1982. 12. Drance, S., Morgan, R.M., and Sweeny, V.P.: Shock-induced optic neuropathy. N. Engl. j. Med. 288: 392-394, 1973. 13. Beck, R.W., Gamel, J,W., Willcourt, R.j., et aI.: Acute ischemic optic neuropathy in severe preeclampsia. Am. j. Ophthalmol. 90: 342-346, 1980. 14. Carroll, F.D.: Optic nerve complications of cataract extraction. Trans. Am. Acad. Ophthalmol. Otolaryngol. 77: 623-629, 1973. 15. Victor, D.L., and Welch, R.B.: Bilateral retinal hemorrhages and disc edema in migraine. Am. f. Ophthalmol. 84: 555-558, 1977. 16. Wolff, H.G. (revised by Dalessio, D.j.): Headache and Other Head Pain (3rd ed.). Oxford University Press, New York, 1972. 17. Brown, e.c, Mogorgol, L.E., Shields, J,A, et aI.: Retinal arterial obstructions in children and young adults. Ophthalmology 88: 18-25, 1981. 18. Friedman, M.W.: Occlusion of retinal vein in migraine. Arch. Ophthalmol. 45: 678-682, 1951. 19. Dunning, H.5.: Intracranial and extracranial vascular accidents in migraine. Arch. Neurol. Psychiatry 48: 396-406, 1942. 20. Carroll, D.: Retinal migraine. Headache 9-13, 1970. 21. Boisen, E.: Stroke in migraine: Report on seven strokes associated with severe migraine attacks. Dan. Med. Bull. 22: 100-106, 1975. 22. Kalendovsky, Z., and Austin, j.H.: Complicated migraine: Its association with increased platelet aggregability and abnormal plasma coa~lability factors. Headache 15: 18-35, 1975. 23. Heyck, H.: The importance of arteriovenous shunts in the pathogenesis of migraine. In Background to Migraine: Third Migraine Symposium, AL. Cochrane, Ed. Springer-Verlag, New York, 1970, pp. 19-25. 24. Saxena, P.R.: Arteriovenous shunting and migraine. Res. Clill. Stud. Headache 6: 89-102, 1978. 25. joffe, j.N.: Retinal blood vessel diameter during migraine. Eye Ear Nose Throat MOIl. 52: 338-342, 1973. 26. Blau, j.N., and Davis, E.: Small blood vessels in migraine. Lancet 2: 740-742, 1970. 27. Otis, S.M., Smith, R.A, Kroll, AD., et a\.: Vasospasm and vascular headaches: Selective vasoconstriction in the carotid vascular system measured by the Doppler ophthalmic method in migraineurs. Headache 19: 200-203,1979. 28. Hayreh, S.5.: Anterior Ischemic Optic Neuropathy. Springer-Verlag, New York, 1975. 29. Hayreh, S.S.: Pathogenesis of visual field defects: Role of the ciliary circulation. Br. f. Ophthalmol. 54: 289-311, 1970. 89 Migraine 30. Lieberman, M.F., Maumenee, A.E., and Green, W.R.: Histologic studies of the vasculature of the anterior optic nerve. Am. ]. Ophthalmol. 82: 405423, 1976. 31. Ernest, JT, and Potts, A.M.: Pathophysiology of the distal portion of the optic nerve head: II. Vascular relationships. Am. ]. Ophthalmol. 66: 380387,1968. 32. Anderson, D.R., and Davis, E.B.: Retina and optic nerve after posterior ciliary artery occlusion. Arch. Ophthalmol. 92: 422-426, 1974. 90 Acknowledgment This study was supported in part by an unrestricted development grant from Research to Prevent Blindness. Write for reprints to: Patrick S. O'Connor, M.D., Department of Ophthalmology University of Texas Health Sciences Center, 7703 Floyd Curl Drive, San Antonio, Texas 78284. Journal of Clinical Neuro-ophthalmology |
