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Branch artery occlusion in a young woman
SURVEY
OF OPHTHALMOLOGY
CLINICAL
VOLUME
30.
NUMBER
1 - JULY-AUGUST
1985
CHALLENGES
RONALD M. BURDE AND PAUL HENKIND, EDITORS
Branch Artery Occlusion JOHN W. GITTINGER, Comments
in a Young Woman
JR., M.D.
by Neil R. Miller, M.D., John L. Keltner,
(In keebing
with
thepur~ose
oj’a rlinicalpnthological
M.D., and Ronald
rorz~rence,
the abstract
M. Burde, M.D.
and kty n,ords a,t)pear at the end of
the article.)
Dilated fundus examination demonstrated a circumscribed area of retinal edema superior and temporal to the macula in the right eye (Fig. 2). In the left eye in a similar location were several punch-out lesions at the level of the retinal pigment epithelium (Fig. 2). Computerized tomography of the head was normal. Echocardiogram revealed reduplicated mitral echoes but no clear evidence of mitral prolapse. An electrocardiogram showed normal sinus rhythm with early repolarization. A complete blood count was normal. Platelet count was 259,000; erythrocyte sedimentation rate was 4 mm/hour. Test for antinuclear antibodies was negative. Serum cholesterol was 231 mg/dl. Prothrombin time and partial thromboplastin time were in the normal range. Blood cultures were negative. Platelet factor 4 was 83 ng/ml (normal range O-10.4 ng/ml). The patient was placed on 325 mg of aspirin and 25 mg ofdipyridamole, three times a day. When she returned for follow-up six months later, she had experienced no further episodes of visual loss and was no longer aware of a defect. Repeat visual field examination of the right eye revealed expansion of the I,e isopter and only an equivocal defect to the 1,e stimulus in the region of the previous scotoma. The right fundus was normal; the left was unchanged.
This 3 1-year-old white, married woman, who is a teacher and secretary, came to the emergency room at the University of Massachusetts Medical Center with the complaint that one-quarter of the vision in her right eye was missing. That morning she awoke, sat up in bed, and experienced sudden, bilateral visual loss lasting about Iive seconds. As this cleared, she was aware of a loss of vision in her right eye inferior and nasal to fixation. Over the next several hours this improved somewhat, but a defect persisted which prompted her visit to the hospital. There were no other associated symptoms. There was no history of migraine or cardiac disease. She had not taken oral contraceptives for over a year, and there was no history of recreational drug use. Two years previously she had undergone cholecystectomy for cholelithiasis. She had smoked over a pack of cigarettes daily for 1.5 years. Physical examination revealed a midsystolic click and late systolic murmur. Her neuroiogical examination was normal with the exception of her visual field. Her visual acuity was 20/15 in each eye. Kinetic visual fields performed on a Goldmann perimeter demonstrated a scotoma inferior and nasal to fixation in the right eye (Fig. 1); the visual field in the left eye was normal. Intraocular pressures were 15 mm Hg in the right eye and 16 mm Hg in the left. 52
BRANCH
ARTERY
OCCLUSION
53
Fig. 1. Left: Relatively normal visual field of the patient’s left eye. Nonspecific constriction of the 1,e isopter to inside the blindspot should be noted. Right: Inferior nasal central quadrantic defect is present to Ile test object. The depth of the scotoma to a Ipe and 14e isopter is demonstrated.
What is the SigniJcance of a branch retinal artery occlusion in a young person? What evaluation do you undertake? What significance doyou attach to a diagnosis of mitral valve prolapse?
Comments Comments by Neil R. Miller, M.D., The Wilmer Ophthalmological Institute, Johns Hopkins University, Baltimore, Maryland This is a case of a young, healthy woman who developed permanent visual field loss in her right eye, thought to have occurred as a result of a branch retinal artery occlusion. The patient’s workup reveals only a nonspecifically abnormal echocardiogram and an elevated platelet factor 4. She is placed on aspirin and dipyridamole and has no further attacks over the next six months. As in all patients with visual loss, the first step in the evaluation is the history. This patient’s visual loss begins suddenly with painless, bilateral visual loss lasting about five seconds and then clearing
Fig. 2. Left: Circumscribed area of edema is noted superior temporal to the macula of the right eye. Right: Punched-out circular lesion of the pigment epithelium is noted superior and temporal in the left eye.
except for an inferior nasal field defect in the right eye. The bilaterality of visual loss immediately implicates a multifocal process - either vasospasm or multiple emboli. Migraine is probably the most common cause of vasospastic ocular and intracranBrown and associates’ reial vascular disease. 7.‘2.23.38 port nine of 27 patients (one-third) who developed retinal arterial obstruction prior to the age of 30 years had a history of migraine. Although Dr. Gitthere tinger’s patient has “no history of migraine,” is no comment regarding whether she had a history of either car sickness, ice cream headache, or other abnormalities thought by many to be migraine prodromes.“,lq Since migraine can begin at any age, this disorder must be considered strongly in this patient. Coagulation abnormalities are also reported to be common among young patients who suffer retinal artery occ1usions,7~4’~42and this patient does have an elevated platelet factor 4; however, in my experience, it is rarely clear how such abnormalities actually relate pathophysiologically to obstruction. Such patients rarely have a history of other vascular
54
Surv Ophthalmol
30( 1) July-August
1985
events, and it has been impossible to pin down our hematologists with respect to a specific role of the factors or a method of preventing further occlusions from occurring. In addition to platelet factor abnormalities, one must also consider other factors that might contribute to abnormal clotting, such as hemoglobinopathies. Although the patient is white, and thus presumably does not have either sickle cell disease or sickle cell trait, both of which may be associated with retinal artery obstruction,7~27~34~3g~45 other hemoglobinopathies could be present. Doctor Gittinger is quite correct in attempting to obtain a history for recreational drug use and oral contraceptives in his patient. Some drugs, particularly heroin, may be mixed with substances like talc and cornstarch that, when injected intramuscularly, may serve as emboli that occlude small vessels in various organs of the body including the eye.3,28 In addition, oral contraceptives are reported to be associated with retinal artery occlusions in young womqn.2’t40 Although three women in the series reported by Brown and associates’ were taking oral contraceptives at the time of their visual loss, each woman had other potentially contributory factors. While the connective tissue (collagen vascular) disorders such as systemic lupus erythematosus and periarteritis nodosa have been associated with retinal artery occlusions in young individuals, usually women 24 there is no serologic evidence of either disordei in this patient (normal antinuclear antibody titer, normal erythrocyte sedimentation rate). Cardiac disorders are well recognized as etiologic factors of retinal artery obstruction in young individuals. Atria1 myxoma is a well-known but, in my experience, very rare cause.‘4*‘* This tumor is usually diagnosed by echocardiography. Similarly, patients with rheumatic heart disease and subacute bacterial endocarditis are candidates for embolic retinal disease. This patient has no history or physical findings implicating any of these disorders. The most common cardiac disease that appears to be responsible for amaurosis fugax as well as retinal and intracranial artery occlusions in young individuals, however, is mitral leaflet prolapse (Barlow’s syndrome). 5,47,48 The diagnosis of mitral valve prolapse is usually suspected by cardiac auscultation when a midsystolic click is heard, and the diagnosis is verified by echocardiography. In Dr. Gittinger’s patient, a midsystolic click is indeed heard, but echocardiography is nondiagnostic. Despite the inconclusive echocardiogram in this patient, I believe Barlow’s syndrome may be the most likely diagnosis (although migraine is still a distinct possibility). Although other system disorders (e.g., homocystinuria, Fabry’s disease) and ocular anomalies (e.g., optic disc drusen, preretinal arterial loops) are re-
GITTINGER ET AL ported in association with retinal arterial occlusion >6.7,36,37,46 they are rare and usually identified by a careful history and examination. In the final analysis, all young patients with retinal artery occlusion should be evaluated carefully from an ocular and systemic standpoint. The patient should be questioned regarding a history of migraine or its accompaniments (e.g., car sickness as a child), drug abuse, use of oral contraceptives, previous vascular disorders, and cardiac disease. When a history is unrevealing, a careful cardiac workup and a serologic screen are probably the most important portions of the workup. The role of coagulation defects (other than hemoglobinopathies) is unclear. When all else fails, I would agree with placing the patient on antiplatelet aggregant medications such as aspirin and dipyridamole, although some patients might benefit from proprano101 and other antimigraine drugs. Comments by John L. Keltner, M.D., Departments of Ophthalmology, Neurology, and Neurosurgery, Universi9 of California at Davis, Davis, Calzfornia Dr. Gittinger describes a 3 1-year-old white woman who awakes with bilateral visual loss lasting five seconds. Her vision clears except for a persistent inferior nasal defect in the right eye. The patient has 20/15 vision in each eye. Visual fields demonstrate a scotoma inferior nasal to fixation in the right eye; the left visual field is normal. Ophthalmoscopic examination reveals a branch retinal artery occlusion in the right eye. In the left eye, in a similar area, are several punched-out lesions suggesting, perhaps, there was a previous artery occlusion in the left eye. Pertinent laboratory findings in this patient are an echocardiogram which reveals reduplicated mitral valve echoes but no clear evidence of a mitral valve prolapse, and platelet factor 4 is found to be elevated. The patient is placed on aspirin and dipyridamole and for the ensuing six months experiences no further episodes. Repeat visual field examination six months later shows only a small defect in the area of the previously noted scotoma. If the history is correct, the patient experienced bilateral loss of vision for a few seconds prior to noting persistent visual loss in her right eye. In order to explain this occurrence, it is necessary to postulate that more than one event took place, likely on a vascular basis. In addition, the fundus findings on the left suggest that she may have had a previous small artery occlusion in that eye, although visual fields appear to be fairly normal at the time of testing. Brown and associates, as well as numerous other authors, have written about vascular occlusive dis-
BRANCH ARTERY
OCCLUSION
ease in young individuals.7.8.25 A history of migraine was found in approximately one-third of the patients, and coagulation abnormalities were also common. Other causes included trauma, sickle cell hemoglobinopathies, cardiac disorders, use of oral contraceptives, pregnancy, systemic lupus erythematosus, and intravenous drug abuse. Ocular abnormalities included increased intraocular pressure, subtle buried drusen of the optic nerve head, and congenital peripapillary arterial 10ops.“~~.~~Very rare causes of arterial occlusion have been described in homocystinuria, Fabry’s disease, Sydenham’s chorea, and a variety of other isolated instances.7 In contrast to older patients with retinal artery occlusions, there was no evidence of atheromatous disease in the young patients.7 In a recent article by and hemLacy and coworkers 3oon brain infarction orrhage in young and middle-age adults (ages 17-45 years), the most common causative factors were rheumatic heart disease, migraine, and oral contraceptives in the younger group; atherosclerosis, hypertension, and diabetes were most common in the middle-age group. Numerous authors have pointed out that etiologic causes for artery occlusion may be multifactorial.2,7~‘5~35~@Thus, both systemic and ocular abnormalities must be fully excluded in order to allow for appropriate diagnosis and management. The finding of one abnormality should not preclude a search for additional contributing factors. What facts are provided in this case that may put this young patient at risk for an artery occlusion? First, the patient has smoked over a pack of cigarettes a day for 15 years. Although there is no history of migraine or cardiac disease, one might wonder if there is any history of migraine-like phenomena. Oral contraceptives, which are known to produce a risk factor for arterial occlusion, have not been taken for over one year, and there is no history of recreational drug use, which also is known to produce arterial occlusive disease. Two years previously she underwent cholecystectomy for cholelithiasis. This might indicate a problem with lipid metabolism. Her cholesterol is reported at 23 1, which is presumably normal, but none of the rest of her lipid profiles are mentioned. While she has no history of heart disease, her echocardiogram reveals reduplicated mitral echoes suggestive, but not diagnostic, of mitral valve prolapse. Mitral valve prolapse is associated with an increased incidence of the “platelet and embolic phenomena.25~2g~3’~44 sticky syndrome”” Blood coagulation studies are normal in this patient except for a very elevated platelet factor 4 (83 ng/ml with a normal range of O-10.4 ng/ml). While embolic phenomena, as well as the “sticky may both be associated with platelet syndrome,” mitral valve prolapse, the possibility of endocarditis
55 is considerably higher in patients who have late systolic murmurs with mitral valve prolapse. There is no evidence in this patient of endocarditis. In addition, it is suggested that patients with mitral valve prolapse with a late systolic murmur should receive antibiotic prophylaxis before procedures which produce bacteremia.35 Fisher and associateszO report finding that mitral valve prolapse was a predisposing factor for cerebral ischemia, especially in young adults, and platelets played a key role in the development of thrombi. They report that platelet factor 4 was a marker protein of platelet activation and was elevated in 12 of 33 patients with mitral valve prolapse without a history of stroke. Platelet activation in vivo can now be assessed easily by measuring plasma level of platelet factor 4, a marker protein of platelet aggregation. Fisher and associates believe this finding indicates that platelet abnormalities are found frequently in patients with asymptomatic mitral valve prolapse and thus may identify a group of mitral valve prolapse patients who are at risk for emboli. Gonder and colleaguesz2 report 50 patients under age 50 years with central retinal vein obstruction. Seventy-three percent of the patients were found to have abnormalities of platelet function, usually platelet coagulative hyperactivity. Sixty-four percent were found to have mitral valve prolapse, and 60% had abnormalities of platelet function. The findings suggest that mitral valve prolapse contributes to platelet hyperactivity and, ultimately, to central retinal vein occlusion. Jackson and coworkersz6 report 32 patients with mitral valve prolapse as the only recognizable cause for cerebral ischemia. Littman and Friedman33 report a series of 230 patients with mitral valve prolapse, 64 (20%) ofwhom had migraine. In another series of patients with mitral valve prolapse, Amat and associates’ report finding 20% of the patients had a vascular headache syndrome. According to the history of Dr. Gittinger’s patient, she does not have evidence for migraine, but certainly migraine is one risk factor which needs to be considered,17 particularly in a patient with a possible history of mitral valve prolapse. Migraine was present in 22% of the patients reported by Jackson and coworkers. They noted that the association of migraine and mitral valve prolapse is speculative until a controlled study is completed. They found that recurrent ischemic events may be prevented by the empirical use of platelet antiaggregants, and anticoagulants should only be used for recurrent events. Bergen and colleagues5 reported a case of bilateral retinal artery occlusion with an echocardiogram which indicated clear mitral valve prolapse. Several
56
Surv Ophthalmol
30( 1) July-August
1985
authors have reported retinal emboli from mitral valve prolapse. “z~,~’ Lesser and coworkers3’ reported 59 patients with mitral valve prolapse. They looked for the incidence of amaurosis fugax and found 22% had symptoms, while 27 controls had only one attack of amaurosis fugax. They concluded that the patients with mitral valve prolapse had an increased risk of ocular and cerebral ischemia. Researchers at Wayne State University suggest that young patients with cerebral infarction have part of what is called a “sticky platelet syndrome.“13 They report finding a family history that tends to indicate there might be a genetic explanation for the “sticky platelet syndrome.” In their studies there is an increased family history ofvascular events occurring in several members at a young age. Also, there is a higher association of migraine headaches. In addition, according to their studies, smoking evidently can aggravate the “sticky platelet syndrome.” In their series, they report finding 20 patients with a history of angina or myocardial infarction and also an equal number with visual loss caused by occlusion of the retinal arteries. They believe it is extremely important to identify patients with this “sticky platelet syndrome,” and that these patients should have their platelets analyzed. They state that their patients responded quite well to aspirin or dipyridamole with a decrease in platelet aggregability.‘3,‘6 The lipid profile of Dr. Gittinger’s patient is not presented, but we do know that she had a cholecystectomy for cholelithiasis and may well have had lipid abnormalities possibly contributing to a “sticky platelet syndrome.” Although there is no history ofdrug use in this patient, certainly intravenous drug use should be kept in mind in any adult who suffers retinal artery emboli. Talc and cornstarch emboli from filler mixed with crushed methylphenidate tablets are reported in retinal arteries of drug abusers.3 These emboli often lodge in multiple retinal arteries or in the macula. In these cases, continued intravenous injections may produce extensive pulmonary capillary obstruction. This leads to arteriovenous shunt formation, possibly facilitating passage of emboli into the arterial circulation.7 Finally, what evaluation should be considered in a young patient with an arterial occlusion? First, a careful history should be elicited in patients and their relatives as to other preceding vascular events which might indicate a genetic predisposition to the “sticky platelet syndrome.” A careful history of migraine, not only headache symptoms but also for migraine related phenomena, in the patient as well as family members may be important. Past medical history related to cardiac disease, particularly a history of rheumatic fever has great importance. One
GITTINGER ET AL should also inquire about the use of oral contraceptives and intravenous drugs. After a thorough eye examination, looking for any abnormalities which may help to identify contributing factors such as high intraocular pressure, optic nerve head drusen, prepapillary arterial loops, appropriate laboratory studies should be performed. A complete blood count, erythrocyte sedimentation rate, antinuclear antibody test, hemoglobin electrophoresis (if the patient is black), serology and coagulation studies, including factor assays as well as platelet coagulation activity, should be studied. In addition, a two-hour postprandial blood sugar and a screen and lipid profile should be obtained. Finally, a cardiac evaluation, including an echocardiogram or appropriate cardiologic consultation, should be performed. With our present state of knowledge, we do not understand all the platelet abnormalities and coagulation factors which contribute to an arterial occlusion in the young patient. If, after screening the patient for all of the above-mentioned factors, no etiology is apparent, it probably is advisable to place the patient on platelet antiaggregant therapy or anticoagulant therapy if there is no response to the former. In summary, mitral valve prolapse, with or without a history of migraine, a history of cigarette smoking and gall bladder disease, all may predispose Dr. Gittinger’s patient either to the “sticky platelet syndrome” or to embolic disease from the mitral valve prolapse itself.“,‘3 The fact that the patient has had no further ischemic episodes is encouraging. We need to know more about coagulation abnormalities and their importance in this and similar patients. Antiaggregant platelet medication would seem appropriate. With recurrent transient ischemic episodes, despite the use ofplatelet antiaggregants, anticoagulation therapy may be considered.‘3B44 Comments by Ronald M. Burde, M.D., Departments of Ophthalmology and Neurology and Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri Drs. Miller and Keltner discuss in great detail the differential diagnosis and therapeutic options available. Thus, rather than reduplicating such an effort, let us reexamine the case presentation. The patient experiences sudden bilateral visual loss lasting about five seconds. Under almost all circumstances this implies posterior circulatory insufficiency. The simultaneous occurrence of a retinal artery occlusion, indicating involvement of the anterior circulation, almost certainly points to the presence of em-
BRANCH ARTERY
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57
bolic disease. It is of interest that the fundus findings in the other eye appear to me to indicate choroidal infarction involving one of the short posterior ciliary arteries. The only reasonable explanation is that the heart is serving as a source of emboli. Drs. Miller and Keltner, as well as Brown and associateq6s7 variously mention that a history of migraine was present in one-third of patients under age 30 years with retinal artery obstruction. This incidence probably does not differ from the occurrence of migraine in the population as a whole (15%-17% of men and 25%-29% of women),43 and therefore adds very little toward invoking migraine as a cause. I have discussed an approach to patients presenting with transient visual 10~s.~ I would modify this approach slightly. First, determine if the visual loss is unilateral or bilateral. If the loss is unilateral, the duration is critical. If the duration exceeds 20 minutes, microscopic hyphema (idiopathic or following cataract surgery), preeruptive ischemic optic neuropathy, and migraine phenomena should be considered. If the visual loss is less than 20 minutes but more than seconds, an embolic source must be excluded and, in a young women, fibromuscular dysplasia should be included in the differential diagnosis. Retinal or ophthalmic migraine remains a diagnostic possibility. If the visual loss is fleeting, optic nerve drusen, papilledema, and obscurations of myopia should be excuded. An important historical fact often overlooked is whether the visual loss is related to the position of the eye within the orbit. Such a relationship is diagnostic of an orbital mass lesion. If the visual loss is bilateral, the presence or absence of scintillations with their characteristic march will implicate or exclude a wide variety of migraine phenomena. If there is no history of scintillations, then it is necessary to exclude the presence of nonembolic causes of transient visual loss, such as arteriosclerotic disease, autonomic dysfunction, escause of trogen overload, etc. lo Once a nonembolic transient visual loss is reasonably excluded, attention should be directed toward differentiating between a cardiac and intraarterial source of emboli. In the differential diagnosis of Dr. Gittinger’s patient, it is possible that a momentary, arrhythmia caused the simultaneous visual loss (hypoperfusion), and she was only experiencing emboli in the anterior circulation. In that case, fibromuscular disease may also be a diagnostic possibility in a young woman.
References 1, Amat G, Louis PJ, L&y C, et al: Migraine and the mitral valve prolapse syndrome. Adu New01 33:27-29, 1982 2. Anonymous: The floppy mitral valve. Lancet lrl38-139,
1979
3. Atlee WE Jr: Talc and cornstarch emboli in eyes in drug abusers. JAMA 219:49-51, 1972 4. Barnett HJM, Boughner DR, Taylor DW, et al: Further evidence relating mitral-valve prolapse to cerebral ischemic events. New Engl J Med 302: 139-144, 1980 5. Bergen RL, Cangemi FE, Glassman R: Bilateral arterial occlusion secondary to Barlow’s syndrome. Ann Ophthalmol 14:67> 675, 1982 6. Brown GC, Magargal L, Augsburger JJ, Shields JA: Preretinal arterial loops and retinal arterial occlusion. Am J Ophthalmol 87:646-651, 1979 7. Brown GC, Magargal LE, Shelds JA, et al: Retinal arterial obstruction in children and young adults. Ophthalmology 88. 18-25, 1981 8. Brown GC, Magargal LE: Central retinal artery obstruction and visual acuity. Opfithutmology 89: 14-19, 1982 9. Burde RM: Discussion. Ophthalmology 91:441L442, 1984 10. Burde RM, Savino PJ, Trobe JD: Transient visual loss, in Burde RM, Savino PJ, Trobe JD: Clinical Dccisionr in NeuroOphthalmology. St Louis, CV Mosby, 1985, pp 91-l 15 11. Caltrider ND, Irvine AR, Kline JH, Rosenblatt A: Retinal emboli in patients with mitral valve prolapse. Am J Ophthalmol 90:534-539, 1980 12. Carroll D: Retinal migraine. Headache 10:%13, 1970 13. Check WA: Possible platelet syndrome suggested in early stroke. JAMA 252:597-598, 1984 14. Cogan DG, Wray SH: Vascular occlusions in the eye from cardiac myxomas. Am J Ophthalmol 80:39&403, 1975 15. Coppeto JR, Currie JN, Monteiro MLR, Lessell S: A syndrome of arterial-occlusive retinopathy and encephalopathy. Am J Ophthalmol 98: 189-202, 1984 16. Couch .JR, Hassanein RS: Platelet aggregability in migraine. -- Ncurolo& 27:843-848, 1977 17. D’Andrea G. Toldo M. Cortelazzo S. Milone FF: Platelet activity in migraine. HcadoLhe 22:207-212, 1982 18. Donoso LA, Magargal LE, Eiferman RA, Jaegers KR: Recurrent vascular obstructions from left atrial myxoma. Neuro-ophthalmology 2:X%65, I 98 1 19. Fisher CM: Late-life migraine accompaniments as a cause of unexplained transient ischemic attacks. Can J Neurof Sci 7rS17, 1980 20. Fisher M, Weiner B, Ockene IS, et al: Platelet activation and mitral valve prolapse. Neurology 33:384-386, 1983 21. Friedman S, Golan A, Shoenfeld A, Goldman J: Acute ophthalmologic complications during the use of oral contraceptives. Contraception 10:68>692, 1974 22. Gondor JR, Magargal LE, Walsh PN, et al: Central retinal vein obstruction associated with mitral valve prolapse. Can JOphthalmol 18:22O-222, 1983 23. Graveson GS: Retinal arterial occlusion in migraine. Br Med J 2:83&840, 1949 24. Henkind P, Gold DH: Ocular manifestations of rheumatic disorders: Natural and iatrogenic. Rheumatology 4: 13-59, 1973 25. Hirsowitz GS, Saffer D: Hemiplegia and the billowing mitral leaflet syndrome. J New01 Ncurosurg Psychiatry 41:81-383, 1978 26. ,Jackson AC, Boughner DR, Barnett HJM: Mitral valve proiapse and cerebraiischemic events in young patients. Neurology 34:784787, 1984 27. Kabakow B, Van Weimokly SS, Lyons HA: Bilateral central retinal artery occlusion. Arch Ophthalmol 54:670-676, 1955 from carotid artery self-injec28. Keane JR: Embolic retinopathy tion. J Clin Ncuro-ophthalmol I: 119- 12 1, 1981 29. Kimball RW, Hedges TR: Amaurosis fugax caused by a prolapsed mitral valve leaflet in the midsystolic click, late systolic murmur syndrome. Am J Ophthalmol83:46%470, 1977 NR: Brain 30. Lacy JR, Filley CM, Arnest MP, GrafT-Radford infarction and hemorrhage in young and middle-aged adults. West J Med 141:32%334, 1984 31. Lesser RL, Yeinemann M-H, Borkowski H Jr, Cohen LS: Mitral valve prolapse and amaurosis fugax. J Clin Neuro-ophthalmol 1:15~160, 1981 circulation 32 Limaye SR, Tang RA, Pilkerton AR: Cilioretinal and branch arterial occlusion associated with preretinal arterial loops. Am J Ophthalmol89:834-839, 1980
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42. Walsh PN, Mills DCB, Pareti F, et al: Hereditary giant platelet syndrome: Absence of collagen-induced coagulant activity and deficiency of factor-XI binding to platelets. Br J Hacmatol 29:639-655, 1975 43. Waters WE: Prevalence of migraine. JNeurol Neurosurg Pyhiatry 38:613-616, 1975 44. Watson RT: TIA, stroke, and mitral valve prolapse. Neurology 29:886-889, 1979 45. Weissman H, Nadel AJ, Dunn M: Simultaneous bilateral retinal arterial occlusions treated by exchange transfusions. Arch Ophthalmol97:2151-2153, 1979 46. Wilson LA, Keeling PWN, Malcolm AD, et al: Visual complications of mitral leaflet prolapse. Br Med J 2:8&88, 1977 47. Wilson RS, Ruiz RS: Bilateral central retinal artery occlusion in homocystinuria. A case report. Arch Ophthalmol 82:267-278, 1969 48. WoldofFHS, Gerber M, Desser KB, Benchimol A: Retinal vascular lesions in two patients with prolapsed mitral valve leaflets. Am J Ophthalmol 79:382-385, 1975
33. Litman GI, Friedman HM: Migraine and mitral valve prolapse syndrome. Am Heart J 96:610-614, 1978 34. Michaelson PE, Pfaffenbach D: Retinal arterial occlusion following ocular trauma in youths with sickle-trait hemoglobinopathy. Am J Ophthalmol 74:494-497, 1972 35. Mills P, Rose J, Hollingsworth J, et al: Long-term prognosis of mitral-valve prolapse. New Engl J Med 297: 13-18, 1977 36. Purcell JJ Jr, Goldberg RE: Hyaline bodies of the optic papilla and bilateral acute vascular occlusions. Ann Ophthalmol 6: 1069-1076, 1974 37. Sher NA, Reiff W, Letson RD, Desnick RJ: Central retinal artery occlusion complicating Fabry’s disease. Arch Ophthalmol %:815-817, 1978 38. Silberberg DH, Laties AM: Occlusive migraine. Tram PA Acad Ophthalmol Otolarpgol 27:34-38, 1974 39. Sorr EM, Goldberg RE: Traumatic central retinal artery occlusion with sickle cell trait. Am J Ophthalmol 80:648-652, 1975 40. Walsh FB, Clark DB, Thompson RS, Nicholson DH: Oral contraceptives and neuro-ophthalmologic interest. Arch Ophthalmol 74:62%640, 1965 41. Walsh PN, Kansu T, Savino PJ, et al: Platelet coagulant activities in arterial occlusive disease of the eye. Stroke 10:589-594, 1979
Reprints
are not available.
Abstract. A 31-year-old
woman presented with signs and symptoms of anterior and posterior cerebral circulatory insufftciency. The differential diagnosis and therapeutic options are discussed. The most likely cause is a cardiogenic source of emboli. Surv Ophthalmol 30:52-58, 1985)
Key words. vasospasm
artery
occlusion
l
emboli
l
migraine
l
transient
visual
loss
l
OF OPHTHALMOLOGY
CLINICAL
VOLUME
30.
NUMBER
1 - JULY-AUGUST
1985
CHALLENGES
RONALD M. BURDE AND PAUL HENKIND, EDITORS
Branch Artery Occlusion JOHN W. GITTINGER, Comments
in a Young Woman
JR., M.D.
by Neil R. Miller, M.D., John L. Keltner,
(In keebing
with
thepur~ose
oj’a rlinicalpnthological
M.D., and Ronald
rorz~rence,
the abstract
M. Burde, M.D.
and kty n,ords a,t)pear at the end of
the article.)
Dilated fundus examination demonstrated a circumscribed area of retinal edema superior and temporal to the macula in the right eye (Fig. 2). In the left eye in a similar location were several punch-out lesions at the level of the retinal pigment epithelium (Fig. 2). Computerized tomography of the head was normal. Echocardiogram revealed reduplicated mitral echoes but no clear evidence of mitral prolapse. An electrocardiogram showed normal sinus rhythm with early repolarization. A complete blood count was normal. Platelet count was 259,000; erythrocyte sedimentation rate was 4 mm/hour. Test for antinuclear antibodies was negative. Serum cholesterol was 231 mg/dl. Prothrombin time and partial thromboplastin time were in the normal range. Blood cultures were negative. Platelet factor 4 was 83 ng/ml (normal range O-10.4 ng/ml). The patient was placed on 325 mg of aspirin and 25 mg ofdipyridamole, three times a day. When she returned for follow-up six months later, she had experienced no further episodes of visual loss and was no longer aware of a defect. Repeat visual field examination of the right eye revealed expansion of the I,e isopter and only an equivocal defect to the 1,e stimulus in the region of the previous scotoma. The right fundus was normal; the left was unchanged.
This 3 1-year-old white, married woman, who is a teacher and secretary, came to the emergency room at the University of Massachusetts Medical Center with the complaint that one-quarter of the vision in her right eye was missing. That morning she awoke, sat up in bed, and experienced sudden, bilateral visual loss lasting about Iive seconds. As this cleared, she was aware of a loss of vision in her right eye inferior and nasal to fixation. Over the next several hours this improved somewhat, but a defect persisted which prompted her visit to the hospital. There were no other associated symptoms. There was no history of migraine or cardiac disease. She had not taken oral contraceptives for over a year, and there was no history of recreational drug use. Two years previously she had undergone cholecystectomy for cholelithiasis. She had smoked over a pack of cigarettes daily for 1.5 years. Physical examination revealed a midsystolic click and late systolic murmur. Her neuroiogical examination was normal with the exception of her visual field. Her visual acuity was 20/15 in each eye. Kinetic visual fields performed on a Goldmann perimeter demonstrated a scotoma inferior and nasal to fixation in the right eye (Fig. 1); the visual field in the left eye was normal. Intraocular pressures were 15 mm Hg in the right eye and 16 mm Hg in the left. 52
BRANCH
ARTERY
OCCLUSION
53
Fig. 1. Left: Relatively normal visual field of the patient’s left eye. Nonspecific constriction of the 1,e isopter to inside the blindspot should be noted. Right: Inferior nasal central quadrantic defect is present to Ile test object. The depth of the scotoma to a Ipe and 14e isopter is demonstrated.
What is the SigniJcance of a branch retinal artery occlusion in a young person? What evaluation do you undertake? What significance doyou attach to a diagnosis of mitral valve prolapse?
Comments Comments by Neil R. Miller, M.D., The Wilmer Ophthalmological Institute, Johns Hopkins University, Baltimore, Maryland This is a case of a young, healthy woman who developed permanent visual field loss in her right eye, thought to have occurred as a result of a branch retinal artery occlusion. The patient’s workup reveals only a nonspecifically abnormal echocardiogram and an elevated platelet factor 4. She is placed on aspirin and dipyridamole and has no further attacks over the next six months. As in all patients with visual loss, the first step in the evaluation is the history. This patient’s visual loss begins suddenly with painless, bilateral visual loss lasting about five seconds and then clearing
Fig. 2. Left: Circumscribed area of edema is noted superior temporal to the macula of the right eye. Right: Punched-out circular lesion of the pigment epithelium is noted superior and temporal in the left eye.
except for an inferior nasal field defect in the right eye. The bilaterality of visual loss immediately implicates a multifocal process - either vasospasm or multiple emboli. Migraine is probably the most common cause of vasospastic ocular and intracranBrown and associates’ reial vascular disease. 7.‘2.23.38 port nine of 27 patients (one-third) who developed retinal arterial obstruction prior to the age of 30 years had a history of migraine. Although Dr. Gitthere tinger’s patient has “no history of migraine,” is no comment regarding whether she had a history of either car sickness, ice cream headache, or other abnormalities thought by many to be migraine prodromes.“,lq Since migraine can begin at any age, this disorder must be considered strongly in this patient. Coagulation abnormalities are also reported to be common among young patients who suffer retinal artery occ1usions,7~4’~42and this patient does have an elevated platelet factor 4; however, in my experience, it is rarely clear how such abnormalities actually relate pathophysiologically to obstruction. Such patients rarely have a history of other vascular
54
Surv Ophthalmol
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1985
events, and it has been impossible to pin down our hematologists with respect to a specific role of the factors or a method of preventing further occlusions from occurring. In addition to platelet factor abnormalities, one must also consider other factors that might contribute to abnormal clotting, such as hemoglobinopathies. Although the patient is white, and thus presumably does not have either sickle cell disease or sickle cell trait, both of which may be associated with retinal artery obstruction,7~27~34~3g~45 other hemoglobinopathies could be present. Doctor Gittinger is quite correct in attempting to obtain a history for recreational drug use and oral contraceptives in his patient. Some drugs, particularly heroin, may be mixed with substances like talc and cornstarch that, when injected intramuscularly, may serve as emboli that occlude small vessels in various organs of the body including the eye.3,28 In addition, oral contraceptives are reported to be associated with retinal artery occlusions in young womqn.2’t40 Although three women in the series reported by Brown and associates’ were taking oral contraceptives at the time of their visual loss, each woman had other potentially contributory factors. While the connective tissue (collagen vascular) disorders such as systemic lupus erythematosus and periarteritis nodosa have been associated with retinal artery occlusions in young individuals, usually women 24 there is no serologic evidence of either disordei in this patient (normal antinuclear antibody titer, normal erythrocyte sedimentation rate). Cardiac disorders are well recognized as etiologic factors of retinal artery obstruction in young individuals. Atria1 myxoma is a well-known but, in my experience, very rare cause.‘4*‘* This tumor is usually diagnosed by echocardiography. Similarly, patients with rheumatic heart disease and subacute bacterial endocarditis are candidates for embolic retinal disease. This patient has no history or physical findings implicating any of these disorders. The most common cardiac disease that appears to be responsible for amaurosis fugax as well as retinal and intracranial artery occlusions in young individuals, however, is mitral leaflet prolapse (Barlow’s syndrome). 5,47,48 The diagnosis of mitral valve prolapse is usually suspected by cardiac auscultation when a midsystolic click is heard, and the diagnosis is verified by echocardiography. In Dr. Gittinger’s patient, a midsystolic click is indeed heard, but echocardiography is nondiagnostic. Despite the inconclusive echocardiogram in this patient, I believe Barlow’s syndrome may be the most likely diagnosis (although migraine is still a distinct possibility). Although other system disorders (e.g., homocystinuria, Fabry’s disease) and ocular anomalies (e.g., optic disc drusen, preretinal arterial loops) are re-
GITTINGER ET AL ported in association with retinal arterial occlusion >6.7,36,37,46 they are rare and usually identified by a careful history and examination. In the final analysis, all young patients with retinal artery occlusion should be evaluated carefully from an ocular and systemic standpoint. The patient should be questioned regarding a history of migraine or its accompaniments (e.g., car sickness as a child), drug abuse, use of oral contraceptives, previous vascular disorders, and cardiac disease. When a history is unrevealing, a careful cardiac workup and a serologic screen are probably the most important portions of the workup. The role of coagulation defects (other than hemoglobinopathies) is unclear. When all else fails, I would agree with placing the patient on antiplatelet aggregant medications such as aspirin and dipyridamole, although some patients might benefit from proprano101 and other antimigraine drugs. Comments by John L. Keltner, M.D., Departments of Ophthalmology, Neurology, and Neurosurgery, Universi9 of California at Davis, Davis, Calzfornia Dr. Gittinger describes a 3 1-year-old white woman who awakes with bilateral visual loss lasting five seconds. Her vision clears except for a persistent inferior nasal defect in the right eye. The patient has 20/15 vision in each eye. Visual fields demonstrate a scotoma inferior nasal to fixation in the right eye; the left visual field is normal. Ophthalmoscopic examination reveals a branch retinal artery occlusion in the right eye. In the left eye, in a similar area, are several punched-out lesions suggesting, perhaps, there was a previous artery occlusion in the left eye. Pertinent laboratory findings in this patient are an echocardiogram which reveals reduplicated mitral valve echoes but no clear evidence of a mitral valve prolapse, and platelet factor 4 is found to be elevated. The patient is placed on aspirin and dipyridamole and for the ensuing six months experiences no further episodes. Repeat visual field examination six months later shows only a small defect in the area of the previously noted scotoma. If the history is correct, the patient experienced bilateral loss of vision for a few seconds prior to noting persistent visual loss in her right eye. In order to explain this occurrence, it is necessary to postulate that more than one event took place, likely on a vascular basis. In addition, the fundus findings on the left suggest that she may have had a previous small artery occlusion in that eye, although visual fields appear to be fairly normal at the time of testing. Brown and associates, as well as numerous other authors, have written about vascular occlusive dis-
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ease in young individuals.7.8.25 A history of migraine was found in approximately one-third of the patients, and coagulation abnormalities were also common. Other causes included trauma, sickle cell hemoglobinopathies, cardiac disorders, use of oral contraceptives, pregnancy, systemic lupus erythematosus, and intravenous drug abuse. Ocular abnormalities included increased intraocular pressure, subtle buried drusen of the optic nerve head, and congenital peripapillary arterial 10ops.“~~.~~Very rare causes of arterial occlusion have been described in homocystinuria, Fabry’s disease, Sydenham’s chorea, and a variety of other isolated instances.7 In contrast to older patients with retinal artery occlusions, there was no evidence of atheromatous disease in the young patients.7 In a recent article by and hemLacy and coworkers 3oon brain infarction orrhage in young and middle-age adults (ages 17-45 years), the most common causative factors were rheumatic heart disease, migraine, and oral contraceptives in the younger group; atherosclerosis, hypertension, and diabetes were most common in the middle-age group. Numerous authors have pointed out that etiologic causes for artery occlusion may be multifactorial.2,7~‘5~35~@Thus, both systemic and ocular abnormalities must be fully excluded in order to allow for appropriate diagnosis and management. The finding of one abnormality should not preclude a search for additional contributing factors. What facts are provided in this case that may put this young patient at risk for an artery occlusion? First, the patient has smoked over a pack of cigarettes a day for 15 years. Although there is no history of migraine or cardiac disease, one might wonder if there is any history of migraine-like phenomena. Oral contraceptives, which are known to produce a risk factor for arterial occlusion, have not been taken for over one year, and there is no history of recreational drug use, which also is known to produce arterial occlusive disease. Two years previously she underwent cholecystectomy for cholelithiasis. This might indicate a problem with lipid metabolism. Her cholesterol is reported at 23 1, which is presumably normal, but none of the rest of her lipid profiles are mentioned. While she has no history of heart disease, her echocardiogram reveals reduplicated mitral echoes suggestive, but not diagnostic, of mitral valve prolapse. Mitral valve prolapse is associated with an increased incidence of the “platelet and embolic phenomena.25~2g~3’~44 sticky syndrome”” Blood coagulation studies are normal in this patient except for a very elevated platelet factor 4 (83 ng/ml with a normal range of O-10.4 ng/ml). While embolic phenomena, as well as the “sticky may both be associated with platelet syndrome,” mitral valve prolapse, the possibility of endocarditis
55 is considerably higher in patients who have late systolic murmurs with mitral valve prolapse. There is no evidence in this patient of endocarditis. In addition, it is suggested that patients with mitral valve prolapse with a late systolic murmur should receive antibiotic prophylaxis before procedures which produce bacteremia.35 Fisher and associateszO report finding that mitral valve prolapse was a predisposing factor for cerebral ischemia, especially in young adults, and platelets played a key role in the development of thrombi. They report that platelet factor 4 was a marker protein of platelet activation and was elevated in 12 of 33 patients with mitral valve prolapse without a history of stroke. Platelet activation in vivo can now be assessed easily by measuring plasma level of platelet factor 4, a marker protein of platelet aggregation. Fisher and associates believe this finding indicates that platelet abnormalities are found frequently in patients with asymptomatic mitral valve prolapse and thus may identify a group of mitral valve prolapse patients who are at risk for emboli. Gonder and colleaguesz2 report 50 patients under age 50 years with central retinal vein obstruction. Seventy-three percent of the patients were found to have abnormalities of platelet function, usually platelet coagulative hyperactivity. Sixty-four percent were found to have mitral valve prolapse, and 60% had abnormalities of platelet function. The findings suggest that mitral valve prolapse contributes to platelet hyperactivity and, ultimately, to central retinal vein occlusion. Jackson and coworkersz6 report 32 patients with mitral valve prolapse as the only recognizable cause for cerebral ischemia. Littman and Friedman33 report a series of 230 patients with mitral valve prolapse, 64 (20%) ofwhom had migraine. In another series of patients with mitral valve prolapse, Amat and associates’ report finding 20% of the patients had a vascular headache syndrome. According to the history of Dr. Gittinger’s patient, she does not have evidence for migraine, but certainly migraine is one risk factor which needs to be considered,17 particularly in a patient with a possible history of mitral valve prolapse. Migraine was present in 22% of the patients reported by Jackson and coworkers. They noted that the association of migraine and mitral valve prolapse is speculative until a controlled study is completed. They found that recurrent ischemic events may be prevented by the empirical use of platelet antiaggregants, and anticoagulants should only be used for recurrent events. Bergen and colleagues5 reported a case of bilateral retinal artery occlusion with an echocardiogram which indicated clear mitral valve prolapse. Several
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1985
authors have reported retinal emboli from mitral valve prolapse. “z~,~’ Lesser and coworkers3’ reported 59 patients with mitral valve prolapse. They looked for the incidence of amaurosis fugax and found 22% had symptoms, while 27 controls had only one attack of amaurosis fugax. They concluded that the patients with mitral valve prolapse had an increased risk of ocular and cerebral ischemia. Researchers at Wayne State University suggest that young patients with cerebral infarction have part of what is called a “sticky platelet syndrome.“13 They report finding a family history that tends to indicate there might be a genetic explanation for the “sticky platelet syndrome.” In their studies there is an increased family history ofvascular events occurring in several members at a young age. Also, there is a higher association of migraine headaches. In addition, according to their studies, smoking evidently can aggravate the “sticky platelet syndrome.” In their series, they report finding 20 patients with a history of angina or myocardial infarction and also an equal number with visual loss caused by occlusion of the retinal arteries. They believe it is extremely important to identify patients with this “sticky platelet syndrome,” and that these patients should have their platelets analyzed. They state that their patients responded quite well to aspirin or dipyridamole with a decrease in platelet aggregability.‘3,‘6 The lipid profile of Dr. Gittinger’s patient is not presented, but we do know that she had a cholecystectomy for cholelithiasis and may well have had lipid abnormalities possibly contributing to a “sticky platelet syndrome.” Although there is no history ofdrug use in this patient, certainly intravenous drug use should be kept in mind in any adult who suffers retinal artery emboli. Talc and cornstarch emboli from filler mixed with crushed methylphenidate tablets are reported in retinal arteries of drug abusers.3 These emboli often lodge in multiple retinal arteries or in the macula. In these cases, continued intravenous injections may produce extensive pulmonary capillary obstruction. This leads to arteriovenous shunt formation, possibly facilitating passage of emboli into the arterial circulation.7 Finally, what evaluation should be considered in a young patient with an arterial occlusion? First, a careful history should be elicited in patients and their relatives as to other preceding vascular events which might indicate a genetic predisposition to the “sticky platelet syndrome.” A careful history of migraine, not only headache symptoms but also for migraine related phenomena, in the patient as well as family members may be important. Past medical history related to cardiac disease, particularly a history of rheumatic fever has great importance. One
GITTINGER ET AL should also inquire about the use of oral contraceptives and intravenous drugs. After a thorough eye examination, looking for any abnormalities which may help to identify contributing factors such as high intraocular pressure, optic nerve head drusen, prepapillary arterial loops, appropriate laboratory studies should be performed. A complete blood count, erythrocyte sedimentation rate, antinuclear antibody test, hemoglobin electrophoresis (if the patient is black), serology and coagulation studies, including factor assays as well as platelet coagulation activity, should be studied. In addition, a two-hour postprandial blood sugar and a screen and lipid profile should be obtained. Finally, a cardiac evaluation, including an echocardiogram or appropriate cardiologic consultation, should be performed. With our present state of knowledge, we do not understand all the platelet abnormalities and coagulation factors which contribute to an arterial occlusion in the young patient. If, after screening the patient for all of the above-mentioned factors, no etiology is apparent, it probably is advisable to place the patient on platelet antiaggregant therapy or anticoagulant therapy if there is no response to the former. In summary, mitral valve prolapse, with or without a history of migraine, a history of cigarette smoking and gall bladder disease, all may predispose Dr. Gittinger’s patient either to the “sticky platelet syndrome” or to embolic disease from the mitral valve prolapse itself.“,‘3 The fact that the patient has had no further ischemic episodes is encouraging. We need to know more about coagulation abnormalities and their importance in this and similar patients. Antiaggregant platelet medication would seem appropriate. With recurrent transient ischemic episodes, despite the use ofplatelet antiaggregants, anticoagulation therapy may be considered.‘3B44 Comments by Ronald M. Burde, M.D., Departments of Ophthalmology and Neurology and Neurological Surgery, Washington University School of Medicine, St. Louis, Missouri Drs. Miller and Keltner discuss in great detail the differential diagnosis and therapeutic options available. Thus, rather than reduplicating such an effort, let us reexamine the case presentation. The patient experiences sudden bilateral visual loss lasting about five seconds. Under almost all circumstances this implies posterior circulatory insufficiency. The simultaneous occurrence of a retinal artery occlusion, indicating involvement of the anterior circulation, almost certainly points to the presence of em-
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57
bolic disease. It is of interest that the fundus findings in the other eye appear to me to indicate choroidal infarction involving one of the short posterior ciliary arteries. The only reasonable explanation is that the heart is serving as a source of emboli. Drs. Miller and Keltner, as well as Brown and associateq6s7 variously mention that a history of migraine was present in one-third of patients under age 30 years with retinal artery obstruction. This incidence probably does not differ from the occurrence of migraine in the population as a whole (15%-17% of men and 25%-29% of women),43 and therefore adds very little toward invoking migraine as a cause. I have discussed an approach to patients presenting with transient visual 10~s.~ I would modify this approach slightly. First, determine if the visual loss is unilateral or bilateral. If the loss is unilateral, the duration is critical. If the duration exceeds 20 minutes, microscopic hyphema (idiopathic or following cataract surgery), preeruptive ischemic optic neuropathy, and migraine phenomena should be considered. If the visual loss is less than 20 minutes but more than seconds, an embolic source must be excluded and, in a young women, fibromuscular dysplasia should be included in the differential diagnosis. Retinal or ophthalmic migraine remains a diagnostic possibility. If the visual loss is fleeting, optic nerve drusen, papilledema, and obscurations of myopia should be excuded. An important historical fact often overlooked is whether the visual loss is related to the position of the eye within the orbit. Such a relationship is diagnostic of an orbital mass lesion. If the visual loss is bilateral, the presence or absence of scintillations with their characteristic march will implicate or exclude a wide variety of migraine phenomena. If there is no history of scintillations, then it is necessary to exclude the presence of nonembolic causes of transient visual loss, such as arteriosclerotic disease, autonomic dysfunction, escause of trogen overload, etc. lo Once a nonembolic transient visual loss is reasonably excluded, attention should be directed toward differentiating between a cardiac and intraarterial source of emboli. In the differential diagnosis of Dr. Gittinger’s patient, it is possible that a momentary, arrhythmia caused the simultaneous visual loss (hypoperfusion), and she was only experiencing emboli in the anterior circulation. In that case, fibromuscular disease may also be a diagnostic possibility in a young woman.
References 1, Amat G, Louis PJ, L&y C, et al: Migraine and the mitral valve prolapse syndrome. Adu New01 33:27-29, 1982 2. Anonymous: The floppy mitral valve. Lancet lrl38-139,
1979
3. Atlee WE Jr: Talc and cornstarch emboli in eyes in drug abusers. JAMA 219:49-51, 1972 4. Barnett HJM, Boughner DR, Taylor DW, et al: Further evidence relating mitral-valve prolapse to cerebral ischemic events. New Engl J Med 302: 139-144, 1980 5. Bergen RL, Cangemi FE, Glassman R: Bilateral arterial occlusion secondary to Barlow’s syndrome. Ann Ophthalmol 14:67> 675, 1982 6. Brown GC, Magargal L, Augsburger JJ, Shields JA: Preretinal arterial loops and retinal arterial occlusion. Am J Ophthalmol 87:646-651, 1979 7. Brown GC, Magargal LE, Shelds JA, et al: Retinal arterial obstruction in children and young adults. Ophthalmology 88. 18-25, 1981 8. Brown GC, Magargal LE: Central retinal artery obstruction and visual acuity. Opfithutmology 89: 14-19, 1982 9. Burde RM: Discussion. Ophthalmology 91:441L442, 1984 10. Burde RM, Savino PJ, Trobe JD: Transient visual loss, in Burde RM, Savino PJ, Trobe JD: Clinical Dccisionr in NeuroOphthalmology. St Louis, CV Mosby, 1985, pp 91-l 15 11. Caltrider ND, Irvine AR, Kline JH, Rosenblatt A: Retinal emboli in patients with mitral valve prolapse. Am J Ophthalmol 90:534-539, 1980 12. Carroll D: Retinal migraine. Headache 10:%13, 1970 13. Check WA: Possible platelet syndrome suggested in early stroke. JAMA 252:597-598, 1984 14. Cogan DG, Wray SH: Vascular occlusions in the eye from cardiac myxomas. Am J Ophthalmol 80:39&403, 1975 15. Coppeto JR, Currie JN, Monteiro MLR, Lessell S: A syndrome of arterial-occlusive retinopathy and encephalopathy. Am J Ophthalmol 98: 189-202, 1984 16. Couch .JR, Hassanein RS: Platelet aggregability in migraine. -- Ncurolo& 27:843-848, 1977 17. D’Andrea G. Toldo M. Cortelazzo S. Milone FF: Platelet activity in migraine. HcadoLhe 22:207-212, 1982 18. Donoso LA, Magargal LE, Eiferman RA, Jaegers KR: Recurrent vascular obstructions from left atrial myxoma. Neuro-ophthalmology 2:X%65, I 98 1 19. Fisher CM: Late-life migraine accompaniments as a cause of unexplained transient ischemic attacks. Can J Neurof Sci 7rS17, 1980 20. Fisher M, Weiner B, Ockene IS, et al: Platelet activation and mitral valve prolapse. Neurology 33:384-386, 1983 21. Friedman S, Golan A, Shoenfeld A, Goldman J: Acute ophthalmologic complications during the use of oral contraceptives. Contraception 10:68>692, 1974 22. Gondor JR, Magargal LE, Walsh PN, et al: Central retinal vein obstruction associated with mitral valve prolapse. Can JOphthalmol 18:22O-222, 1983 23. Graveson GS: Retinal arterial occlusion in migraine. Br Med J 2:83&840, 1949 24. Henkind P, Gold DH: Ocular manifestations of rheumatic disorders: Natural and iatrogenic. Rheumatology 4: 13-59, 1973 25. Hirsowitz GS, Saffer D: Hemiplegia and the billowing mitral leaflet syndrome. J New01 Ncurosurg Psychiatry 41:81-383, 1978 26. ,Jackson AC, Boughner DR, Barnett HJM: Mitral valve proiapse and cerebraiischemic events in young patients. Neurology 34:784787, 1984 27. Kabakow B, Van Weimokly SS, Lyons HA: Bilateral central retinal artery occlusion. Arch Ophthalmol 54:670-676, 1955 from carotid artery self-injec28. Keane JR: Embolic retinopathy tion. J Clin Ncuro-ophthalmol I: 119- 12 1, 1981 29. Kimball RW, Hedges TR: Amaurosis fugax caused by a prolapsed mitral valve leaflet in the midsystolic click, late systolic murmur syndrome. Am J Ophthalmol83:46%470, 1977 NR: Brain 30. Lacy JR, Filley CM, Arnest MP, GrafT-Radford infarction and hemorrhage in young and middle-aged adults. West J Med 141:32%334, 1984 31. Lesser RL, Yeinemann M-H, Borkowski H Jr, Cohen LS: Mitral valve prolapse and amaurosis fugax. J Clin Neuro-ophthalmol 1:15~160, 1981 circulation 32 Limaye SR, Tang RA, Pilkerton AR: Cilioretinal and branch arterial occlusion associated with preretinal arterial loops. Am J Ophthalmol89:834-839, 1980
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42. Walsh PN, Mills DCB, Pareti F, et al: Hereditary giant platelet syndrome: Absence of collagen-induced coagulant activity and deficiency of factor-XI binding to platelets. Br J Hacmatol 29:639-655, 1975 43. Waters WE: Prevalence of migraine. JNeurol Neurosurg Pyhiatry 38:613-616, 1975 44. Watson RT: TIA, stroke, and mitral valve prolapse. Neurology 29:886-889, 1979 45. Weissman H, Nadel AJ, Dunn M: Simultaneous bilateral retinal arterial occlusions treated by exchange transfusions. Arch Ophthalmol97:2151-2153, 1979 46. Wilson LA, Keeling PWN, Malcolm AD, et al: Visual complications of mitral leaflet prolapse. Br Med J 2:8&88, 1977 47. Wilson RS, Ruiz RS: Bilateral central retinal artery occlusion in homocystinuria. A case report. Arch Ophthalmol 82:267-278, 1969 48. WoldofFHS, Gerber M, Desser KB, Benchimol A: Retinal vascular lesions in two patients with prolapsed mitral valve leaflets. Am J Ophthalmol 79:382-385, 1975
33. Litman GI, Friedman HM: Migraine and mitral valve prolapse syndrome. Am Heart J 96:610-614, 1978 34. Michaelson PE, Pfaffenbach D: Retinal arterial occlusion following ocular trauma in youths with sickle-trait hemoglobinopathy. Am J Ophthalmol 74:494-497, 1972 35. Mills P, Rose J, Hollingsworth J, et al: Long-term prognosis of mitral-valve prolapse. New Engl J Med 297: 13-18, 1977 36. Purcell JJ Jr, Goldberg RE: Hyaline bodies of the optic papilla and bilateral acute vascular occlusions. Ann Ophthalmol 6: 1069-1076, 1974 37. Sher NA, Reiff W, Letson RD, Desnick RJ: Central retinal artery occlusion complicating Fabry’s disease. Arch Ophthalmol %:815-817, 1978 38. Silberberg DH, Laties AM: Occlusive migraine. Tram PA Acad Ophthalmol Otolarpgol 27:34-38, 1974 39. Sorr EM, Goldberg RE: Traumatic central retinal artery occlusion with sickle cell trait. Am J Ophthalmol 80:648-652, 1975 40. Walsh FB, Clark DB, Thompson RS, Nicholson DH: Oral contraceptives and neuro-ophthalmologic interest. Arch Ophthalmol 74:62%640, 1965 41. Walsh PN, Kansu T, Savino PJ, et al: Platelet coagulant activities in arterial occlusive disease of the eye. Stroke 10:589-594, 1979
Reprints
are not available.
Abstract. A 31-year-old
woman presented with signs and symptoms of anterior and posterior cerebral circulatory insufftciency. The differential diagnosis and therapeutic options are discussed. The most likely cause is a cardiogenic source of emboli. Surv Ophthalmol 30:52-58, 1985)
Key words. vasospasm
artery
occlusion
l
emboli
l
migraine
l
transient
visual
loss
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