11/172. susac syndrome.PURPOSE: To report the first Japanese case of the susac syndrome characterized by microangiopathy of the brain, retina, and cochlea. methods: Case report. A 36-year-old Japanese man underwent extensive clinical and laboratory examination for encephalopathy, bilateral recurrent branch retinal arterial occlusion, and hearing loss. RESULTS: This case was initially diagnosed as acute disseminated encephalomyelitis. Despite exhaustive clinical and laboratory examination of the patient, no evidence of a known systemic disease was found. The bilateral recurrent branch retinal arterial occlusions were widely disseminated. Remission occurred 1 year after symptom onset. This patient demonstrated all of the clinical features associated with the susac syndrome. CONCLUSION: susac syndrome has been considered rare, but it is probably more common than previously thought and seems to occur in any race. This disorder usually occurs in young women, but it can affect men.- - - - - - - - - - ranking = 1keywords = microangiopathy (Clic here for more details about this article) |
12/172. Diagnostic features of the Favre-Goldmann syndrome.Extensive retinal vascular disease was noted in three patients from two families with Favre-Goldmann syndrome. In addition to classical features they had pronounced leakage from some retinal vessels. Vessels were either opaque ('sclerotic') or non-perfused. Cystoid macular oedema was a contributing cause of decreased vision. Two of the three patients showed a discrepancy on electroretinography between single-flash photopic amplitudes and flicker fusion frequency. This may be characteristic of Favre-Goldmann syndrome.- - - - - - - - - - ranking = 0.18684426562035keywords = vascular disease (Clic here for more details about this article) |
13/172. Thrombotic thrombocytopenic purpura in pulmonary-renal syndromes.Thrombotic thrombocytopenic purpura pathologically consists of a thrombotic microangiopathy that classically spares lung tissues. We describe a case of TTP that presented as a pulmonary-renal syndrome. In reviewing the international literature, pulmonary involvement is not as rare as once was thought, and the diagnosis of TTP should be considered in the differential diagnosis of pulmonary-renal syndromes.- - - - - - - - - - ranking = 1keywords = microangiopathy (Clic here for more details about this article) |
14/172. Raynaud's phenomenon, anticentromere antibodies, and digital necrosis without sclerodactyly: an entity independent of scleroderma?We describe 4 women of 43, 73, 84, and 86 years with Raynaud's phenomenon, severe digital necrosis, and high serum levels of anticentromere antibodies without skin thickening or internal organ sclerosis. Investigations revealed no diabetes or arterial vascular disease leading to arterial obstruction. Histologic examination did not show any dermal sclerosis or calcinosis. The intravenous infusions of prostaglandin reversed the ischemic lesions in 3 patients. These cases suggest that the triad Raynaud's phenomenon, anticentromere antibodies and necrosis of digits without sclerodactyly and sclerosis of internal organ should be considered as an entity distinct from scleroderma with sclerosis. For this entity we propose the name RACAND syndrome.- - - - - - - - - - ranking = 0.18684426562035keywords = vascular disease (Clic here for more details about this article) |
15/172. Superior orbital fissure syndrome in a latent type 2 diabetic patient.Although isolated cranial nerve palsies are common in diabetic patients, multiple, simultaneous cranial neuropathies are rare. We describe the second case of a complete superior orbital fissure syndrome including the optic nerve in a middle-aged Papuan man with newly diagnosed diabetes mellitus. The differential diagnosis included septic cavernous sinus thrombosis and Tolosa Hunt syndrome, and management was initially directed at excluding these serious, treatable conditions.- - - - - - - - - - ranking = 3.1699825409955keywords = diabetic (Clic here for more details about this article) |
16/172. Progressive appearance of overlap syndrome together with autoantibodies in a patient with fatal thrombotic microangiopathy.We describe an extraordinary patient with overlap syndrome (systemic lupus erythematosus, systemic sclerosis, and rheumatoid arthritis) having positive autoantibodies against Sm, double stranded dna, dna topoisomerase I, and centromere, together with rheumatoid factor. The patient had multiple organ involvement resulting from thrombotic microangiopathy that mimicked so-called normotensive scleroderma renal crisis, and died mainly of massive pulmonary hemorrhage caused by thrombotic thrombocytopenic purpura. The clinical presentations of the case support the concept of strong associations between disease-specific autoantibodies and clinical features.- - - - - - - - - - ranking = 5keywords = microangiopathy (Clic here for more details about this article) |
17/172. susac syndrome: microangiopathy of the retina, cochlea and brain.BACKGROUND: susac syndrome is characterized by the triad of branch retinal arterial occlusions, encephalopathy and cochlear microangiopathy. The underlying process is believed to be a small vessel vasculitis causing microinfarcts in the retina, brain and cochlea. methods: Analysis of two male and two female cases of susac syndrome recognized in australia. RESULTS: In this series the epidemiology, mode of presentation, ophthalmologic features, neurologic and cochleo-vestibular features, radiologic characteristics, cerebrospinal fluid findings, therapeutic interventions, clinical course and outcome of susac syndrome is examined. Key ophthalmologic differential diagnoses include systemic lupus erythematosis (SLE), Behcet's syndrome and other vasculitides such as sarcoidosis, tuberculosis, syphilis and lymphoma. Neuro-otologic features are most frequently misdiagnosed as multiple sclerosis. CONCLUSION: susac syndrome, first described in 1979, is becoming an increasingly recognized condition. Early recognition of the syndrome is important because treatment with systemic immunosuppression may minimize permanent cognitive, audiologic and visual sequelae.- - - - - - - - - - ranking = 5keywords = microangiopathy (Clic here for more details about this article) |
18/172. Uveitis masquerade syndromes.BACKGROUND: Incorrect diagnosis of the uveitic masquerade syndromes (UMS) may have severe consequences. In this study, the frequency, clinical manifestations, and informative diagnostic tests for UMS are described. DESIGN: Retrospective observational case series. PARTICIPANTS: Forty patients with UMS were identified in a cohort of 828 consecutive patients with uveitis. The mean follow-up was 4.5 years. methods: A review of clinical, laboratory, photographic, and angiographic records was performed. MAIN OUTCOME MEASURES: Clinical features, associated systemic diseases, diagnostic procedures and their role in the diagnostic process, and systemic and visual outcomes. RESULTS: Nineteen patients had intraocular malignancy (48% of all with UMS; 2.3% of all with uveitis), mainly intraocular lymphoma (n = 13) and leukemia (n = 3). The ophthalmologist was the first to recognize malignant disease in 11 of 19 patients (58%). Of 21 patients with nonmalignant UMS, 10 had an ocular vascular disease and 5 a hereditary ocular disorder. The patients with malignant UMS were older than those with nonmalignant UMS (average age, 50 vs 44 years, P: < 0.05). During follow-up, 9 of 19 patients with malignant UMS died. The most informative diagnostic procedure for malignant UMS was intraocular fluid analysis; for nonmalignant UMS, fluorescein angiography. The cytologic analysis of intraocular fluids yielded the best results for diagnosing intraocular malignancies (7 of 11 patients, 64%); the association of negative cytologic results with the recent administration of systemic corticosteroids was noted. immunophenotyping of the aqueous confirmed the diagnosis of hematologic malignancy for 3 of 5 patients examined. panuveitis was the most frequent manifestation of malignant UMS. Intraocular lymphomas presented with isolated vitreitis (n = 6), chorioretinal lesions (n = 5) and iris infiltration (n = 2). Clinical presentation of nonmalignant UMS was diverse but consisted mainly of abnormalities of the retinal vasculature. CONCLUSIONS: UMS was diagnosed in 5% of the patients with uveitis at a tertiary center. Despite the variety of underlying disorders and different clinical presentations, a high frequency of malignant and vascular diseases was found. awareness of the clinical manifestations of UMS and application of the correct diagnostic procedures should promote timely diagnosis and treatment, which are essential not only for visual acuity but also for the life of the patient.- - - - - - - - - - ranking = 0.3736885312407keywords = vascular disease (Clic here for more details about this article) |
19/172. Type a syndrome of insulin resistance: anterior chamber anomalies of the eye and effects of insulin-like growth factor-I on the retina.BACKGROUND: The purpose of this work was to describe the anterior chamber and iris anomalies as well as to evaluate the effects of recombinant human insulin-like growth factor-I (rhIGF-I) on the retinal vessels in 2 diabetic patients with type A syndrome of insulin resistance, a rare condition associated with acanthosis nigricans. methods: Ophthalmologic examinations, including photographs and fluorescein angiograms, were performed before, and 2 and 4 weeks after starting subcutaneous rhIGF-I treatment, and 3 months after withdrawal of rhIGF-I treatment. RESULTS: Both patients had goniodysgenesis with mild elevation of the intraocular pressure. Before and after 2 weeks of treatment with rhIGF-I, the fundus and the fluorescein angiograms were mainly normal. After 4 weeks of rhIGF-I treatment both patients' retinas revealed leakage of fluorescein. Three (case 1) and 4 months (case 2) after withdrawal of rhIGF-I, the fundus of all four eyes were again without leakage. CONCLUSIONS: The anterior chamber anomalies found in these patients may be part of the type A syndrome of insulin resistance and could alert clinicians that these patients might not have the usual type of diabetes. Moreover, the data show that exogenous rhIGF-I administration in patients with type A syndrome of insulin resistance alters the permeability of the superficial layer of retinal capillaries which is comparable to the earliest angiographic changes in childhood diabetic retinopathy. Whether this is a direct effect of rhIGF-I, as suggested by experiments in an animal model, or an indirect effect due to the near-normalization of the glucose levels by rhIGF-I warrants further investigations. Finally, this work points to an important caveat regarding the therapeutic use of rhIGF-I in this patient population.- - - - - - - - - - ranking = 1.2679930163982keywords = diabetic (Clic here for more details about this article) |
20/172. Susac's syndrome: beneficial effects of corticosteroid therapy in a Japanese case.Susac's syndrome is a rare disorder characterized by the triad of microangiopathy of the brain and retina with hearing loss. More than 50 affected individuals have been reported worldwide, all Caucasians. We herein identify the first Japanese patient with Susac's syndrome. A 36-year-old man developed recurrent subacute encephalopathy, bi- a lateral sensorineural hearing loss, and retinal arteriolar occlusions, caused by microangiopathy from a year previously. T2-weighted MRI showed multiple high-signal lesions ti predominantly in the periventricular white matter. During the exacerbated phase both high-dose intravenous methyl-prednisolone and oral prednisone therapy produced beneficial effects. He showed definite remission within 2 years from the disease onset.- - - - - - - - - - ranking = 2keywords = microangiopathy (Clic here for more details about this article) |
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