Presentación atípica de síndrome antifosfolípido

El síndrome antifosfolípido es considerado un trastorno del colágeno asociado a presentaciones clínicas a edades intermedias con incidencias no mayores de 5 casos por cada 100.000 personas. Su espectro clínico oscila en la presencia de manifestaciones trombóticas multisistémicas de origen vascular. Sin embargo, las manifestaciones no vasculares tienen un espectro raro en su presentación habitual. Se presenta el caso de un varón de 64 años con manifestaciones oculares relacionadas a amaurosis fugaz asociado a alteraciones estructurales retinianas. Los hallazgos de tamizajes realizados fueron fuertemente sugestivos de trombofilias primarias. Se aplicó perfil para síndrome antifosfolípido el cual fue positivo, por lo que se instauró anticoagulación plena.

Antiphospholipid syndrome is considered a collagen disorder associated with clinical presentations at intermediate ages with incidences of no more than 5 cases per 100,000 people. Its clinical spectrum ranges from the presence of multisystem thrombotic manifestations of vascular origin. However, non-vascular manifestations have a rare spectrum in their usual presentation. The case of a 64-year-old man with ocular manifestations related to amaurosis fugax associated with retinal structural alterations is presented. The findings of screening performed were strongly suggestive of primary thrombophilia. A profile for antiphospholipid syndrome was applied, which was positive and therefore, full anticoagulation was initiated.

Charles Miller Fisher: the 65th anniversary of the publication of his groundbreaking study "Transient Monocular Blindness Associated with Hemiplegia" / Charles Miller Fisher: 65 anos da publicação de seu estudo inovador "Cegueira monocular transitória associada com hemiplegia"

ABSTRACT Charles Miller Fisher is considered the father of modern vascular neurology and one of the giants of neurology in the 20th century. This historical review emphasizes Prof. Fisher's magnificent contribution to vascular neurology and celebrates the 65th anniversary of the publication of his groundbreaking study, "Transient Monocular Blindness Associated with Hemiplegia."

RESUMO Charles Miller Fisher é considerado o pai da neurologia vascular moderna, e um dos gigantes da neurologia no século XX. Esta revisão histórica enfatiza a magnífica contribuição de Miller Fisher na neurologia vascular, particularmente com a celebração dos 65 anos de publicação do seu estudo inovador intitulado "Cegueira monocular transitória associada com hemiplegia".

Amaurosis Fugax Associated with Ipsilateral Internal Carotid Artery Agenesis

PURPOSE: We report a case of amaurosis fugax associated with ipsilateral internal carotid artery agenesis. CASE SUMMARY: A 50-year-old woman presented with amaurosis fugax in her left eye; the frequency of episodes of the condition had recently increased to once a month. She had a history of hypertension and dyslipidemia, and was under medical therapy. The visual acuity of both eyes was 20/20. Slit-lamp examination was normal except for pseudophakia. Ophthalmoscopy revealed a myopic tigroid fundus and a myopic tilted disc. No abnormalities were evident in fluorescein fundus angiography. Brain computed tomography showed that the left bony carotid canal was absent, and magnetic resonance angiography showed that the left internal carotid artery was also absent. She was diagnosed with left internal carotid artery agenesis. Other neurological and hematological parameters were within normal ranges. The amaurosis fugax spontaneously disappeared and has not recurred over the past 12 months. Our case, although rare, suggests that amaurosis fugax may be associated with internal carotid artery agenesis.

Ocular and Systemic Manifestation of Amaurosis Fugax: Six-Year Observational Study

PURPOSE: To investigate the prevalence of ocular and systemic disease causing amaurosis fugax and to discuss the ocular and systemic manifestation of each disease. METHODS: Consecutive patients who had amaurosis fugax were retrospectively studied from 2007 to 2013. Carotid evaluation using Doppler was performed in all patients. Ocular and medical histories were taken and bilateral ophthalmic evaluation performed. RESULTS: This study included 35 patients. The mean age of patients was 63 years and 27 patients were male; 29 unilateral and 6 bilateral eyes were involved. Associated systemic disease included hypertension (54.3%) and diabetes mellitus (34.2%). The most frequent cause of amaurosis fugax was retinal artery occlusion (28.6%) followed by ocular ischemic syndrome (22.9%), other vascular diseases (11.4%), and retinal vein occlusion (5.7%). The remaining 31.4% patients with amaurosis fugax had no vascular disease. Clinically significant stenosis of the internal carotid artery was observed in 16 patients (45.7%) and 6 of these patients (37.5%) had retinal artery occlusion disease. CONCLUSIONS: Prevalence and clinical manifestation of amaurosis fugax is very complex. Patients with transient visual disturbance are at risk for retinal artery occlusion, ocular ischemic syndrome and other diseases which cause visual loss. Therefore, careful history taking and urgent systemic and ophthalmic evaluations should be performed.

Transient Visual Loss in Peripapillary Staphyloma

PURPOSE: To report the case of a patient with transient visual loss with a peripapillary staphyloma. CASE SUMMARY: The authors of the present study examined a 30-year-old woman who complained of transient visual loss in her right eye. The patient lost her vision for 5 seconds on average approximately 5 times a day. Her visual acuity was 20/20 in both eyes. There were no abnormalities on slit lamp examination. A deep excavation with choroidal atrophy in the peripapillary area of the right eye was found. The patient was diagnosed with peripapillary staphyloma. Her physiological blind spot in the right eye was enlarged on Humphrey visual field testing. However, she had no abnormalities on color vision testing, fluorescein angiography, or magnetic resonance imaging (MRI). There were no abnormalities in the evaluation of the brain, carotid arteries, or heart. There was no evidence of vasculitis or hypercoagulability. The symptom did not change on pressing the eyeball, performing the Valsalva maneuver or carotid massage, applying cycloplegics, or shining a strong light in the other eye. The symptom did not change after taking a calcium channel blocker for 4 weeks.

Pérdida transitoria de la visión / Transient vision loss

La pérdida transitoria de la visión monocular (PTVM), es un síntoma alarmante relacionado frecuentemente con alteraciones vasculares retinales y puede tener consecuencias importantes desde el punto de vista ocular y vital. Es por ello, que se requiere de un manejo precoz y adecuado. El presente artículo tiene por objeto revisar las principales causas, su presentación, diagnóstico y manejo, como enfermedades oclusivas vasculares y alteraciones el nervio óptico entre otras

Transient monocular vision loss (TMVL) is an alarming symptom often in relation with retinal vascular anomalies that may have severe consequences for vision and life, so it should be evaluated urgently and a prompt approach is needed. This article will review the main causes, its presentation, diagnosis and management such as vascular occlusive diseases and optic nerve abnormalities among others

Valsalva Maneuver-induced Amaurosis Fugax

PURPOSE: To report the case of a patient with amaurosis fugax that occurred following a Valsalva maneuver. CASE SUMMARY: A 40-year-old man presented with amaurosis fugax of the right eye, which had occurred several times during the previous month. After coughing, the visual acuity of the right eye decreased temporarily during the first episode. Subsequently, any time a Valsalva maneuver, such as coughing, occurred, this symptom reappeared. Initially, this symptom persisted for five to ten minutes and occurred once or twice a day, but it gradually increased in frequency. The physical examination was normal, and his best corrected visual acuity was 20/20 bilaterally. Neither specific findings in the slit lamp examination nor abnormal findings in the fundus examination were detected. On fluorescein fundus angiography, no abnormal finding was observed before the symptom was triggered by a Valsalva maneuver, but after the symptom was triggered by coughing, the choroidal and retinal arterial phases were delayed. Hematological and neurological examinations, including magnetic resonance imaging, magnetic resonance angiography, and cerebral angiography, were all normal. Therefore, he was diagnosed with amaurosis fugax generated by a Valsalva maneuver. CONCLUSIONS: In any patient who complains of amaurosis fugax repeatedly, as seen in this case, one must consider the possibility that it results from a Valsalva maneuver, after eliminating occlusive vascular diseases, such as carotid stenosis or atherosclerotic disease.

Ocular Ischemic Syndrome Successfully Treated With Carotid Angioplasty and Stenting

PURPOSE: To report a case of ocular ischemic syndrome successfully treated with delayed carotid angioplasty and stenting (CAS). CASE SUMMARY: A 52-year-old male was admitted to our hospital because of amaurosis fugax-like symptoms in the right eye for several months. His visual acuity was 0.8 in the right eye and he did not have rubeosis iridis. Neovascularization of the disc, narrowing of the retinal artery and multiple retinal hemorrhages were diagnosed by fundus examination. Fluorescein angiography showed delayed choroidal filling, a delayed arm-to-retina time, prolongation of arteriovenous transit time, neovascularization of the disc, retinal capillary nonperfusion, and staining of the retinal vessels. MR angiography showed severe stenosis in the proximal portion of the right carotid artery. We diagnosed this case as ocular ischemic syndrome. The patient was recommended carotid angioplasty and stenting at the severely narrowed portion of the right carotid artery, but it was postponed about six months after diagnosis because of personal problems. At the final follow-up, 24 months after stenting, the amaurosis fugax symptoms had disappeared, the patient had an improved visual acuity of 1.0, and the new vessels on the disc changed to fibrous tissue. Fluorescein angiography showed resolution of the delayed arm-to-retina time and prolongation of the arteriovenous transit time, disappearances of the leakage around the disc and the retinal capillary nonperfusion.

Amaurosis Fugax Associated with Stenosis of the Intracranial Internal Carotid Artery: Successful Restoration of Ophthalmic Artery Flow by Stent Placement

Two patients presented with amaurosis fugax(AMF), despite treatment with antiplatelet drugs and anticoagulants due to previous transient ischemic attacks. Angiography demonstrated severe stenosis in the cavernous and petrous internal carotid artery(ICA) respectively, with reduced flow in the ophthalmic artery(OA). Endovascular stent placement in both patients resulted in normalization of the vessel lumen of the stenotic vessel segments. In addition, complete restoration of OA flow was noted immediately after stenting. Both patients showed no further episode of AMF over a follow-up period of 38 and 23 months respectively. Our clinical and angiographic findings suggest that hemodynamic insufficiency in retinal vasculature caused by a stenosis of the cavernous or petrous ICA can be treated successfully by endovascular stent.

A Case of Systemic Lupus Erythematosus Presenting with Amaurosis Fugax without Antiphospholipid Antibodies Syndrome / 영남의대학술지

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease that may affect many organ systems including the nervous system. The immune response in patients with SLE can cause inflammation and other damage that can cause significant injury to the arteries and tissues. A 48-year-old woman was admitted to the hospital because of transient monocular blindness. Magnetic resonance imaging and conventional angiography showed severe stenosis of the distal intracranial internal carotid artery. The patient was diagnosed as having SLE but the antiphospholipid antibodies were negative. Amaurosis fugax has not been previously reported as an initial manifestation of SLE in Korea. We report a patient with a retinal transient ischemic attack as the first manifestation of SLE.

Transient visual disturbance / 医学研究生学报

Transient visual disturbance(TVD) is caused by artery stenosis,or artery embolism and migraine,which lead to reduction of eye blood flow,retinal ischemia and hypoxia.Major clinical manifestations include amaurosis fugax and visual transient ischemic attacks(TIA).The present research situation and progression of TVD were reviewed.