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18 AMAUROSIS FUGAX CAUSED BY THROMBOPHILIA-HYPOFIBRINOLYSIS IN CASES WITHOUT CAROTID ATHEROSCLEROSIS: THERAPY WITH COUMADIN-LOVENOX OR FOLIC ACID-B6-B12 PREVENTS SUBSEQUENT TRANSIENT MONOCULAR PARTIAL BLINDNESS.
  1. C. J. Glueck,
  2. K. Golnik,
  3. P. Wang
  1. Cholesterol Center, Jewish Hospital, Department of Ophthalmology, University of Cincinnati Medical Center, Cincinnati, OH

Abstract

In 8 men and 9 women (age 60 ± 14, all white) without ipsilateral atherosclerotic carotid plaque and with no other known causes of amaurosis fugax (AF), whose AF was associated with thrombophilia-hypofibrinolysis, we hypothesized that case-specific thromboprophylaxis (Coumadin-Lovenox, folic acid [5 mg]-B6 [100 mg]-B12 [2,000 μg], aspirin, cessation of exogenous estrogens, glucophage [2.5 g]) would prevent subsequent episodes of transient monocular partial or total blindness. All 17 cases had $ 1 thrombophilic-hypofibrinolytic disorder thought to be etiologic for AF. Seven cases had MTHFR C677T homozygosity or C677T-A1298C compound heterozygosity, 5 4G4G PAI-1 homozygosity, 4 high factor VIII, 4 lupus anticoagulant, 4 the platelet glycoprotein PL A1/A2 mutation, 2 low free protein S, 2 high plasminogen activator inhibitor activity (PAI-Fx), 1 V Leiden heterozygosity, 1 prothrombin gene (PTG) heterozygosity, and 1 protein C deficiency. In 4 cases on Coumadin for 16,16, 21, and 98 months (1 PTG heterozygote, 1 protein C deficient [41%], 1 free protein S deficient [53%] with high factor VIII [157%], and 1 with high factor VIII [207%]), AF resolved, usually within 1 month of starting Coumadin, and the patients remained asymptomatic provided that the INR remained 2.5 or higher. In 1 case with low free protein S (28%), treated for 8 months of pregnancy and 1 month postpartum on Lovenox 80 mg/day, AF disappeared within 1 week, and she remained asymptomatic. In 3 cases with MTHFR C677T homozygosity treated with folic acid-B6-B12 for 10, 15, and 29 months, AF resolved, usually within 1 month of starting therapy, and has not recurred. AF stopped within 1 month in 2 cases (1 high factor VIII 157%, 1 PAI-1 4G4G, PL A1/A2) when exogenous estrogens-SERMS were discontinued and has not recurred. The frequency of AF events was reduced in 2 cases on aspirin alone (1 PL A1/A2, I high VIII 157%) and AF events have remained less frequent over 5 and 11 months on aspirin. In 1 case with hypofibrinolysis (4G4G PAI-1 polymorphism, high PAI Fx, 31.7 IU/mL) treated with Glucophage (2.55 g/day), symptoms resolved within 1 month and have not recurred in 9 months on Glucophage. When AF occurs in the absence of carotid artery atherosclerosis or other known causes of AF, thrombophilia and/or hypofibrinolysis are nearly universal, reversible pathoetiologies.

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