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320 References Case Report exclude the development of generalised myasthenia in We report here a patient who presented with severe the future. vaso-occlusive retinal disease in the absence of any ocular symptoms. References 1. Millichap JG, Dodge PR. Diagnosis and treatment of Case report myasthenia gravis in infancy, childhood and adolescence. Neurology 1960;10:1007-14. A 48-year-old white man was referred to us by his 2. Chan-Lui WY, Leung NK, Lau TTY. Myasthenia gravis in optician who noted several 'haemorrhages' in both Chinese children. Dev Med Child NeuroI1984;26:717-24. retinae with new vessels at the left optic disc. He had no 3. Blundy S. A genetic study of infantile and juvenile myasthenia ocular symptoms other than presbyopia. Eleven months gravis. J Neurol Neurosurg Psychiatry 1972;35:41-51. 4. Weinberg DA, Lesser RL, Vollmer TL. Ocular myasthenia: a earlier, he had been investigated by the physicians for protean disorder. Surv Ophthalmol 1994;39:169-210. fatigue, sleeplessness and weight loss, associated with 5. Seybold ME, Lindstrom JM. Myasthenia gravis in infancy. hypertension. Neurology 1981;31:476-80. The ESR was 30 mm in the first hour (Westergren). Fasting blood sugar was normal. The platelet count was 79 X 109/1 (normal 120-400 X 109/1). Prothrombin time, v.s.y. Geh � fibrinogen and fibrinogen degradation products were JA Bradbury normal but the augmented partial thromboplastin test Department of Ophthalmology (APTT) was raised at 47 s (normal 29-37 s). The Dilute Bradford Royal Infirmary Russell Viper Venom Time Ratio was 1.50 (normal Duckworth Lane 0.8-1.12). The following tests were normal/negative: Bradford BD9 6RJ UK autoantibodies to glomerular basement membrane, anti­ double-stranded DNA, complement C3 and C4, c-ANCA and p-ANCA, serum electrophoresis, and Bence-Jones Sir, protein, He had a positive antinuclear antibody and raised titre of anticardiolipin antibody (IgG, 23 GPL u/ Asymptomatic vaso-occlusive retinopathy in Hughes' ml; and IgM, 3 MPL/ml). With a diagnosis of accelerated syndrome hypertension secondary to the antiphospholipid The antiphospholipid (APL) syndrome, first described in syndrome, antihypertensive medication was started. 1983/ is a disorder associated with specific antibodies At presentation to the eye clinic, the patient was well directed against phospholipids, particularly cardiolipin and his blood pressure was 155/85 mmHg. Visual acuity and phosphatidyl serine. Present also are elevated levels was 6/6 and N5 either eye. There was no afferent of the lupus anticoagulant. Although originally studied pupillary defect. Anterior segments were normal, and in patients with systemic lupus erythematosus intraocular pressures were 14 mmHg in both eyes, (secondary APL), APL is now known to occur as a Fundus examination showed occluded and sheathed primary (Hughes') syndrome? The main clinical feature arterioles in the mid-periphery of both eyes, together is thrombosis, both venous and arterial, and the major with frank neovascularisation at the left optic disc feature of the syndrome in women is recurrent (Fig. 1). Fluorescein angiography showed peripheral miscarriages? The eye may be involved symptomatically arteriolar attenuation with frank occlusion at some points as a feature of transient ischaemia,4,5 or as a direct, local, (Fig. 2). We performed left panretinal argon laser ischaemic event.6-9 photocoagulation. Simultaneously, the patient was referred to the haematologist who commenced him on warfarin 5 mg o.d. to prevent further vaso-occlusion, Fig. 1, Fundus photograph of left eye at presentation showing disc Fig. 2, Fluorescein angiogram of the right eye showing a clipped neovascuiarisation and occlusion of an arteriole superior to tl1e fovea. arteriole temporal to the macula. 320 particularly in the right eye. Two months following laser 7. Manzanares jM, Conget I, Rodriguez-Villar e, Tassie 0, photocoagulation and 6 weeks after commencement of Fernandez-Fernandez F, Cervera R, Levy I, Gomis R. Antiphospholipid syndrome in a patient with type I diabetes warfarin, the disc new vessels had regressed. Six months presenting as retinal artery occlusion. Diabetes Care into follow-up, his condition is stable. 1996;19:92-4. 8. Jonas MD, Kolble K, Volcker HE, Kalden JR. Central retinal artery occlusion in Sneddon's disease associated with CommeJlt antiphospholipid antibodies. Am J Ophthalmol 1986;102:37-40. Antiphospholipid antibodies were first detected as a 9. Pulido jS, Ward LM, Fishman GA, Goodwin JA, Froelich q, result of disturbances they cause in routine biological Sanghvi JP, Antiphospholipid antibodies associated with retinal vascular disease. Retina 1987;7:215-8. tests. These effects include lupus anticoagulant in the 10. Angles-Cano E, Sultan y, Clauvel j-R. Predisposing factors to augmented partial thromboplastin test (APTT) and false thrombosis in systemic lupus erythematosus: possible serological tests for syphilis in the VDRL test. The relation to endothelial cell damage. J Lab Clin Med systemic clinical features are mild thrombocytopenia, 1979;94:312-23. chorea, heart valve disease, livedo reticularis and, most 11. Castanon e, Amigo Me, Banales JL, Nava A, Reyes P A. Ocular vaso-occlusive disease in primary antiphospholipid commonly, recurrent pregnancy loss. The mechanism by syndrome. OphthalmOlogy 1995;102:256-62. which they paradoxically cause thrombosis ill viuo has 12. Snyers B, Lambert M, Hardy J-P. Retinal and choroidal vaso­ been debated. A direct antigen-antibody interaction in occlusive disease in systemic lupus erythematosus associated the vascular wall seems to be an attractive concept.IO with anti phospholipid antibodies, Retina 1990;10:255-60. Symptomatic central and branch retinal arteriolar 13. Rai R, Cohen H, Dave M, Regan L. Randomised controlled trial of aspirin and aspirin plus heparin in pregnant women occlusions, ischaemic and non-ischaemic central retinal with recurrent miscarriage associated with phospholipid (or vein occlusions and ischaemic optic neuropathy have all antiphospholipid) antibodies, BMJ 1997;31 4:253-7. been described in the primary and secondary APL syndromes.5-� Our patient was asymptomatic and even reluctant to undergo panretinal scatter photocoagulation. Prasad Pall mar k!Sl Castanon et Ill.]] studied 17 patients with the primary Nolan Cota syndrome and found vascular changes in all, although Warrington Hospital Lovely Lane only 10 patients described visual symptoms. This study Warrington and our case report highlight the importance of ocular Cheshire WA5 1 QG screening in such individuals. UK Aggressive steroid therapy, aspirin and dipyridamole have not universally shown beneficial effects in controlling visual symptoms and preventing progression Sir, of vaso-occlusion.6,H,9,l2 Recently, in a randomised Endogenous Candida endophthalmitis with no controlled clinical trial, low-dose aspirin (75 mg) together apparent predisposing factors with heparin has been shown to be more effective than Endogenous Crnzdidn endophthalmitis (EeE) is well aspirin alone in improving outcome of pregnancy in recognised in patients with certain predisposing factors recurrent fetal loss associated with this syndromeY The such as indwelling intravenous catheters, total parenteral patient described by Manzanares et Ill? experienced a nutrition, abdominal surgery, broad spectrum similar beneficial effect on switching from aspirin to antibiotics, immunosuppressive therapy and intravenous l acenocoumarol. Oral anticoagulants probably need to be drug use. The case recently reported in Eye illustrates continued for at least 6 months or, in the secondary this well. However, occaSionally no obvious syndrome, until the PTT becomes normal. predisposing factors exist and we report a patient who did not, apparently, have any of these risk factors. The diagnosis was suggested by clinical signs and confirmed References at vitrectomy. 1. Hughes GRV. Thrombosis, abortion, cerebral disease and the lupus anticoagulant. BMJ 1983;287:1088-9, Case report 2. Hughes GRV, The anticardiolipin syndrome, Clin Exp Rheumatol 1985;3:285-6, A 65-year-old man presented with a 2 week history of a 3. McNeil HP, Chesterman CN, Krills SA. Immunology and red, aching right eye associated with reduced vision. He clinical importance of antiphospholipid antibodies, Adv Immunol 1991 ;49:193-280. had no problems with his left eye and no past ocular 4..Rumi V, Angelini L, Scaioli V, D'Angelo A, Besana C history. In his past medical history he had eczema and Primary antiphospholipid syndrome and neurologic events, had become asthmatic 2 years previously; the asthma Paediatr Neurol 1993;9:473-5, initially required oral steroids but more recently had II. Levine SR, Crofts JW, Lesser GR, Floberg J, Welch KMA. been controlled with inhaled steroids and a beta-agonist. Visual symptoms associated with the presence of a lupus Two years previously he 'had required surgery for an anticoagulant. Ophthalmology 1988;95:686-92, ft.Acheson JF, Gregson RMe, Merry P, Schulenburgh WE. inguinal hernia, He was a retired gastroenterologist and Vaso-occlusive retinopathy in the primary anti phospholipid his latest travel abroad had been to Egypt 2 years syndrome. Eye 1991;5:48-55. previously. 321 .
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