1/81. Progressive outer retinal necrosis syndrome as an early manifestation of human immunodeficiency virus infection.Progressive outer retinal necrosis syndrome is a recently recognized variant of necrotizing herpetic retinopathy, developing in patients with acquired immune deficiency syndrome (AIDS) or other conditions causing immune compromise. We report a case in which the diagnosis of retinal necrosis syndrome was made before the diagnosis of AIDS was confirmed. A 41-year-old man presented with a 1-month history of blurred vision in his left eye. Ophthalmologic examination revealed extensive retinal necrosis with total retinal detachment in his left eye and multifocal deep retinal lesions scattered in the posterior fundus as well as in the peripheral retina in his right eye. The serologic test for human immunodeficiency virus (hiv) was positive. Despite intravenous acyclovir treatment for 1 week, the lesions in the right eye showed rapid progression. High doses of intravitreal ganciclovir were then given in addition to intravenous acyclovir. After combined treatment for 1 month, the lesions became quiescent and the visual acuity improved to 20/30. Although the patient soon developed full-blown AIDS, the vision in his right eye remained undisturbed. physicians should suspect progressive outer retinal necrosis syndrome in any patient with rapidly progressive necrotizing retinopathy and test the patient for hiv infection. Aggressive combined antiviral agent therapy should be considered to save vision.- - - - - - - - - - ranking = 1keywords = vision (Clic here for more details about this article) |
2/81. Immune-mediated retinopathy in a patient with stiff-man syndrome.BACKGROUND: Stiff-man syndrome is a rare neurological disorder characterised by rigidity and violent spasms of the body musculature. In the majority of patients, presence of antibodies against glutamic acid decarboxylase (GAD), the enzyme synthesizing gamma-aminobutyric acid (GABA), suggests an autoimmune attack against GABA-ergic inhibitory neurons. We report a 32-year-old patient with stiff-man syndrome and anti-GAD antibodies who developed subacute progressive loss of vision in the right eye, and in the left eye 18 months thereafter. methods: Ophthalmological work-up included electro-retinogram (ERG), visual evoked potentials (VEP) and fluorescein angiography. Antiretinal antibodies were investigated using an indirect immunofluorescence technique on frozen sections of macaque retina with patients serum and FITC-conjugated goat antihuman immunoglobulin. Staining with monoclonal anti-GAD65 antibodies and with serum from three healthy normals served as controls. RESULTS: visual acuity of both eyes decreased to 0.16 within a span of 6 weeks. Perimetry revealed a central scotoma in the visual field of both eyes. VEP and flash ERG were progressively disturbed on the right eye. On the left eye, initially only pattern ERG and photopic responses were abnormal. Follow-up recordings revealed widespread pathology of photopic single and flicker responses. Immunofluorescence revealed strong reactivity of the inner plexiform layer and to a lesser extent staining of the outer plexiform layer at dilutions of 1:1000 with patients serum. The same retinal staining pattern was obtained with monoclonal anti-GAD65 antibodies. CONCLUSIONS: These findings suggest autoimmune retinopathy, mediated by anti-GAD65 autoantibodies as the underlying cause of visual loss.- - - - - - - - - - ranking = 0.33333333333333keywords = vision (Clic here for more details about this article) |
3/81. University of Miami Division of Clinical pharmacology Therapeutic Rounds: ischemic renal disease.Ischemic renal disease (IRD) is defined as a significant reduction in glomerular filtration rate and/or loss of renal parenchyma caused by hemodynamically significant renal artery stenosis. IRD is a common and often overlooked clinical entity that presents in the setting of extrarenal arteriosclerotic vascular disease in older individuals with azotemia. IRD is an important cause of chronic renal failure and end-stage renal disease (ESRD), and many patients with a presumed diagnosis of hypertensive nephrosclerosis may actually have undiagnosed ischemic nephropathy as the cause of their ESRD. The primary reason for establishing the diagnosis of IRD is the hope that correction of a renal artery stenosis will lead to improvement of renal function or a delay in progression to ESRD. There are six typical clinical settings in which the clinician could suspect IRD: acute renal failure caused by the treatment of hypertension, especially with angiotensin-converting enzyme inhibitors; progressive azotemia in a patient with known renovascular hypertension; acute pulmonary edema superimposed on poorly controlled hypertension and renal failure; progressive azotemia in an elderly patient with refractory or severe hypertension; progressive azotemia in an elderly patient with evidence of atherosclerotic disease; and unexplained progressive azotemia in an elderly patient. It is important for the clinician to identify IRD, because IRD represents a potentially reversible cause of chronic renal failure in a hypertensive patient.- - - - - - - - - - ranking = 1.3333333333333keywords = vision (Clic here for more details about this article) |
4/81. Sorsby's fundus dystrophy: a case report of 24 years follow-up with electrodiagnostic tests and indocyanine green angiography.PURPOSE: Five families with dominantly inherited macular dystrophy were originally described by Sorsby et al. in 1949. Key features include early bilateral central visual loss secondary to either choroidal neovascularisation or central geographical atrophy and late progressive chorioretinal atrophy. We report a member of one of the original families who has been studied with a series of investigations over a long time, providing important information on differences in the phenotype and natural history of a rare genetically determined macular dystrophy. methods: The patient has been followed up for the last 24 years, from asymptomatic to full manifestation of Sorsby's fundus dystrophy. Series of fundus photographs, colour vision, dark adaptation and electrodiagnostic tests were performed. The disease was also studied with fundus fluorescein angiography and indocyanine green angiography. RESULTS: Unlike her other family members, who were reported in other studies as all having rapid loss of vision secondary to disciform macular disease, our patient has a unique clinical course in that she has a progressive bilateral central and generalised chorioretinal atrophy with a well-preserved minute central island of fovea. Nyctalopia was her early and only symptom. There was evidence of central scotoma, tritanopia and mild abnormality in dark adaptation. Rod function was affected earlier and to a larger degree than cone function. CONCLUSIONS: The overall features suggest phenotypic variability within a family in this autosomal dominant macular dystrophy. The findings from indocyanine green angiography and a consecutive series of electrodiagnostic tests in this condition support the theory of partial choroidal hypoperfusion and an interesting progressive rod-cone dystrophy as part of the pathophysiology.- - - - - - - - - - ranking = 0.66666666666667keywords = vision (Clic here for more details about this article) |
5/81. Unilateral proptosis resulting from giant-cell arteritis.BACKGROUND: Giant-cell arteritis (GCA) is a systemic, inflammatory vasculopathy that affects small- to medium-sized arteries. Arterial wall inflammation results in reduction of blood flow and subsequent ischemia. Arteries of the head and neck are particularly susceptible, including the ophthalmic and posterior ciliary arteries. The eye care provider is in a position to assist with the ultimate diagnosis of GCA. CASE REPORT: A 79-year-old black man was referred to the eye clinic for evaluation of exophthalmos of the left eye. The patient reported increasing proptosis over the previous 6 months; a history of sudden, permanent vision loss of the affected eye (approximately 2 years earlier); and generalized malaise and chronic frontal headache. Examination did reveal an exophthalmic eye of approximately 8-mm difference by Hertel exophthalmometry. Fundus examination revealed optic nerve pallor O.S. CT scan revealed chronic inflammatory changes of orbital tissue, including the extraocular muscles. No compressive lesions were present. Laboratory testing indicated an elevated erythrocyte sedimentation rate. A tentative diagnosis of giant-cell arteritis was made, which was confirmed with temporal artery biopsy. CONCLUSIONS: patients with ocular complications secondary to GCA manifest several different ocular symptoms, including unilateral and bilateral intermittent blur, sudden complete vision loss, double vision, etc. This was an unusual case of GCA because the initially manifested ocular sign was unilateral proptosis. The patient probably had initial ocular complications of GCA 2 years previously, with sudden loss of vision in the left eye. The patient never sought medical attention at that time, and the unilateral exophthalmic eye resulted from chronic inflammatory orbital changes associated with GCA.- - - - - - - - - - ranking = 1.3333333333333keywords = vision (Clic here for more details about this article) |
6/81. Functional recovery after bilateral pallidotomy for the treatment of early-onset primary generalized dystonia.This report describes the successful treatment of dystonia musculorum deformans with bilateral stereotactic pallidotomy in a 14-year-old girl in whom the dystonia was diagnosed when she was 7 years old. The patient presented with dystonia of the right upper extremity that progressed to generalized dystonia. Preoperatively, she required maximal assistance with all activities of daily living and transfers. She was not a functional ambulator. Postoperatively, she had remarkable functional recovery. At discharge, she was at modified independence level for all basic activities of daily living and required supervision for household ambulation. No postoperative complications were noted. We propose that bilateral stereotactic lysis of globus pallidus interna may be an alternative treatment for dystonia musculorum deformans. The technique of bilateral pallidotomy and theories of its effectiveness are discussed.- - - - - - - - - - ranking = 0.33333333333333keywords = vision (Clic here for more details about this article) |
7/81. Not so slowly progressive visual loss.A 51-year-old man presented with bilateral progressive visual loss during a 2-month period. visual acuity was 20/60 in both eyes with bilateral constricted visual fields. Funduscopy revealed bilateral disk pallor and arteriolar attenuation. His vision declined rapidly during the next 2 weeks. Investigations showed a positive cerebrospinal fluid Venereal Disease research Laboratory test (1:8). A diagnosis of neurosyphilis was made, and treatment was started with high-dose intravenous and intramuscular penicillin.- - - - - - - - - - ranking = 0.33333333333333keywords = vision (Clic here for more details about this article) |
8/81. Bilateral diffuse uveal melanocytic proliferation and uterine cancer. A case report.PURPOSE: To report a case of bilateral diffuse uveal melanocytic proliferation (BDUMP), a rare paraneoplastic syndrome causing visual loss in patients with systemic carcinoma. RESULTS: A 70-year-old woman developed visual symptoms 13 months after surgery and local irradiation therapy for uterine cancer. Following bilateral external beam irradiation supplemented with subsequent drainage of subretinal fluid in the left eye, the visual acuity improved from 0.01 to 0.15 in this eye only. The visual acuity remained at this level until she died 4 1/4 years after the onset of eye symptoms. CONCLUSION: This is the fourth case that survived longer than 24 months after the onset of visual symptoms of the 22 previously reported cases with BDUMP. It demonstrates that radiotherapy may have a vision-preserving effect in this group of patients. The patient also developed two different paraneoplastic phenomena--a nephrotic syndrome before and BDUMP after treatment for uterine cancer.- - - - - - - - - - ranking = 0.33333333333333keywords = vision (Clic here for more details about this article) |
9/81. meningeal carcinomatosis manifested as bilateral progressive sensorineural hearing loss.OBJECTIVE: meningeal carcinomatosis is defined as the diffuse infiltration of the leptomeninges and subarachnoid space by malignant cells metastasizing from systemic cancer. The authors describe a rare case of meningeal carcinomatosis initially appearing as bilateral progressive sensorineural hearing loss. PATIENT: A 57-year-old man with lung cancer was referred to the authors' clinic because of progressive hearing loss, tinnitus, dizziness, and blurred vision for 1 month. RESULTS: magnetic resonance imaging revealed abnormal leptomeningeal enhancement. meningeal carcinomatosis was diagnosed by the detection of malignant cells in the cerebrospinal fluid after lumbar puncture. The patient died 1 year after diagnosis. CONCLUSIONS: meningeal carcinomatosis must be considered in the differential diagnosis in cancer patients with bilateral progressive sensorineural hearing loss. gadolinium-enhanced magnetic resonance imaging is a useful complementary diagnostic tool before lumbar puncture.- - - - - - - - - - ranking = 0.33333333333333keywords = vision (Clic here for more details about this article) |
10/81. optic neuritis in children.PURPOSE: To describe the clinical characteristics of optic neuritis in children, including final visual acuity and development of multiple sclerosis (MS). methods: charts were reviewed of all patients < 15 years of age who presented with optic neuritis to the Bascom Palmer eye Institute or the Miami Children's Hospital between 1986 and 1998. RESULTS: Fifteen patients were identified. There was a slight female predilection in the study group (60%), with a mean age of 9.8 years at presentation. A preceding febrile illness within 2 weeks of visual symptoms was reported in 66% of patients. Initial visual acuity ranged from 20/15 to no light perception. Involvement was bilateral in 66% of patients, and disc swelling was present in 64% of involved eyes. Of the patients who underwent magnetic resonance imaging, 33% had focal demyelinating lesions in the brain, and 63% of affected nerves were enlarged or enhanced with gadolinium. Eleven patients were treated with intravenous steroids. Final visual acuity was > or = 20/40 in 58.3% of eyes. Thirty percent of the patients had vision of finger counting or worse. Four (26%) patients developed MS. The mean age of patients with MS was 12 years, compared with 9 years in children who did not develop MS. patients with unilateral involvement had an excellent visual prognosis (100% > 20/40), but a higher rate of development of MS (75%). Two patients had positive serology for lyme disease. CONCLUSIONS: optic neuritis presents differently in children than in adults. Children typically have bilateral involvement with papillitis following an antecedent viral illness. Although visual prognosis is poorer in children than adults, the development of MS is less common in children. Children who present with unilateral involvement have a better visual prognosis; however, they also develop MS at a greater frequency than children with bilateral involvement. patients who developed MS were, on average, older at presentation with optic neuritis than those who did not develop MS.- - - - - - - - - - ranking = 0.33333333333333keywords = vision (Clic here for more details about this article) |
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