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PubMed2004

Susac syndrome: report of four cases and review of the literature.

Susac syndrome is a rare disease of unknown pathogenesis. It is caused by a microangiopathy affecting the arterioles of the brain, retina, and cochlea, giving the classic clinical triad of subacute encephalopathy, visual loss secondary to retinal branch occlusions, and sensorineural hearing loss. The features of four cases of this syndrome are presented. MR imaging, retinal fluorescein angiography, and audiography findings enable diagnosis. Early therapy may reduce sequelae and improve recovery.

Adult↗
PubMed2004

[Neuro-ophthalmologic aspects in antiphospholipid syndrome].

Antiphospholipid syndrome is the one autoimmune noninflammatory syndrome affecting all blood vessels at any vascular segment, irrespective of the vascular size and topography. Defined in 1983 by Hughes, this syndrome represents the actual research theme in practically all medical fields, therefore the importance of antiphospholipid syndrome is recognized. The major aim of this article is to describe the neuro-ophthalmologic manifestations of the antiphospholipid syndrome, emphasizing the latest news about this syndrome and its impact on the retina and central nervous system.

Antibodies, Anticardiolipin↗
PubMed2005

A case of secondary frosted branch angiitis in Behçet's disease.

We report a case of Behcet's disease in a pregnant woman associated with secondary Frosted Branch Angiitis (FBA). FBA is a rare entity characterized by a florid translucent retinal perivascular sheathing of both arterioles and venules, with variable uveitis, retinal oedema and visual loss. To our knowledge, this is the first published report of FBA secondary to Behcet's disease in pregnancy.

Adult↗
PubMed2006

Unusual clinical cases that mimic MS.

Diseases which are associated with relapses and disability accumulation that mimic MS may be effectively treated if they are appropriately diagnosed. Unfortunately, however, it can be easy to assume that a patient is presenting with unusual symptoms of MS rather than establish unequivocally that there is no other cause. This article illustrates five short cases from a selection presented at the MS Forum Interactive Symposium at the LACTRIMS Congress, Brazil, 2004. These cases feature the differential diagnosis of MS and illustrate the importance of early and accurate diagnosis. Each case has clinical features suggestive of MS, together with diagnostic findings that are discordant with this disease. Clinical features that can easily be interpreted as unusual MS presentations, but which indicate the need to undertake additional investigation, are included.

Adult↗
PubMed2006

[Ocular changes in demyelinating diseases].

To synthetize the main ocular changes in the demyelinating diseases of CNS. The most important changes in this diseases are the neuro-ophthalmological ones: retrobulbar optic neuritis, papillitis, oculomotor nerves palsies. Also the demyelinating diseases can have an ophthalmological involvement represented by: retinal periphlebitis, pars planitis.

Demyelinating Diseases↗
PubMed1981

Severe retinal vascular lesions in systemic lupus erythematosus.

Retinal lesions in systemic lupus erythematosus (SLE) are usually closely related to the patient's general condition and occur frequently in severe states such as renal or central nervous system involvement. However, improved treatment of SLE has reduced retinal involvement, and it is rare today to observe severe retinal lesions in patients with SLE. We report two cases of severe retinal vascular lesions in SLE patients who developed otherwise unremarkable systemic manifestations and laboratory findings.

Adult↗
PubMed1994

[Clinical heterogeneity of non-Hodgkin's lymphoma of the eye with extraocular manifestations].

Primary, ocular Non-Hodgkin lymphoma is a multicentric disease. The prognosis is mostly determined by involvement of the central nervous system and/or visceral organs. Since ocular symptoms often precede accompanying manifestations and since the eye is easily accessible to diagnostic measures, the ophthalmological diagnosis is essential for early detection and treatment of this complex disease, which has a poor prognosis. To illustrate the extraordinary heterogeneity of the clinical symptoms, four case reports have been selected which demonstrate various ocular symptoms and extraocular localizations. The ophthalmological variability ranges from typical uveitis to retinitis and vasculitis with varying symptoms during the progression of the disease. Completely different extraocular features were induced by long-standing, local infiltrates within basal ganglia, a diffuse infiltration of the brain leading to an acute increase in intracranial pressure, a peripheral tumour within the spinal channel or massive infiltrates of liver, lung and kidneys. Lack of pathognomonic features, high clinical variability and the limited value of imaging techniques and histopathological measures often lead to serious delays in diagnosis.

Aged↗
PubMed1996

Acquired immunodeficiency syndrome--associated herpes simplex virus retinitis. Clinical description and use of a polymerase chain reaction--based assay as a diagnostic tool.

OBJECTIVES: To describe 2 patients with acquired immunodeficiency syndrome who experienced a rapidly progressive, bilateral retinitis due to herpes simplex virus (HSV) (1 case due to HSV type 1 [HSV-1] and 1 case due to HSV type 2 [HSV-2] and to present a novel diagnostic polymerase chain reaction (PCR)-based assay. METHODS: The presentation, clinical course, and diagnostic PCR-based assay used to make the diagnosis of HSV retinitis in 2 patients with acquired immunodeficiency syndrome are described. RESULTS: Both patients experienced a rapidly progressive, bilateral retinal necrosis associated with intraretinal hemorrhages and a diffuse vasculitis. The PCR-based assays demonstrated HSV DNA in the vitreous specimens from the 2 patients. Restriction analysis on the amplified DNA showed HSV-1 in 1 patient and HSV-2 in the second patient. The diagnosis was supported in both patients by the occurrence of a herpes simplex-like encephalitis, and in 1 patient by a positive vitreous culture. The HSV-1-associated vasculitis affected primarily the retinal arterioles, with marked capillary dropout and occlusion of larger arcade vessels. In contrast, the HSV-2-associated vasculitis affected the retinal veins more than the arterioles, and was associated with an exudative retinal detachment. CONCLUSIONS: To our knowledge, these are the first 2 patients with acquired immunodeficiency syndrome in whom HSV has been implicated as the sole cause of a rapidly progressing, necrotizing retinitis. Combined PCR and restriction analysis of vitreous samples from such patients is a useful and highly specific means of diagnosing HSV-1 and HSV-2 retinitis.

AIDS-Related Opportunistic Infections↗
PubMed2004

Branch retinal artery occlusions as the presenting feature of primary central nervous system vasculitis.

A 39-year-old woman presented with multiple branch retinal artery occlusions almost three years before developing a mass lesion containing calcium in the left frontal lobe. Brain biopsy revealed a small vessel vasculitis and ischemic necrosis of brain with dystrophic calcification. We believe this to be the first case of primary CNS vasculitis with branch retinal artery occlusions and brain calcification.

Adult↗
PubMedDate not supplied

[The nature of changes in the local and systemic fibrinolytic activity in patients with retinal angiitis of various genesis].

The mechanism responsible for the development of occlusive processes on the fundus of the eye in different forms of retinal angiitis remains unknown. So the purpose of this study was to investigate the local and systemic fibrinolytic activity in 10 patients with optic disk vasculitis (ODV), 16 patients with peripheral uveitis (PU), 21 patients with isolated retinal angiitis (IRA), 17 patients with systemic lupus erythematosus (SLE) versus 8 healthy donors (a control group). Fibrinolytic activity was determined by the area of lysis, which appeared on the fibrin plate around the 2x2-mm filter paper previously placed in tear or blood. Fibrinolytic activity was ascertained to be decreased if the area of lysis was less than 30 mm2. There was a significant reduction in local fibrinolytic activity in the eyes with occlusive retinal vasculitis (RV) compared with the eyes with RV without occlusions by PU (60 and 9.1%, respectively; p = 0.0048), IRA (61.5 and 12.5%, respectively; p = 0.0056), and as compared to the eyes without vasculitis by ODV (70 and 0%, respectively, p = 0.0031). In SLE, the decrease in local fibrinolytic activity was detected significantly less frequently (33.3 and 16.6% with and without retinal vascular occlusion (RVO), respectively). Blood fibrinolytic activity was lowered only in patients with SLE (with 100 and 16.7% with and without RVO, respectively; p = 0.0151) and remained unchanged in those with ODV, PU, and IRA (more than 50 mm2). Thus, RVO is associated with impaired local reactions in patients with PU, ODV, and IRA and with impaired systemic reactions in those with SLE.

Fibrinolysis↗
PubMed1992

[Segmental retinal arteritis and retinochorioiditis].

Acute ocular toxoplasmosis is characterized by a focal retinochoroidal infiltrate, often appearing in the immediate vicinity of an old retinochoroidal scar (satellite lesion). There are atypical forms of ocular toxoplasmosis, including retinal granulomas, intraretinal and subretinal neovascularization, preretinal gliosis and retinal detachment. In rare instances, associated immunological phenomena, i.e. concomitant vasculitis and perivasculitis, have been described. We report on a 28-year-old male patient presenting with signs of congenital toxoplasmosis with intracerebral calcifications and internal hydrocephalus (Whytt's disease). Ocular manifestations included an acute retinochoroidal infiltrate adjacent to a retinochoroidal scar and segmental retinal periarteritis. All arterioles were involved, but there was no inflammation of the venules. Fluorescein angiography revealed filling irregularities of the vessel segments involved. Like a tubercular patient with segmental periarteritis reported on as long ago as 1939, our patient had undergone treatment for active tuberculosis of the lung 6 years earlier. Considering the fact that segmental periarteritis without concomitant periphlebitis is very rarely seen, the coincidence of acute toxoplasmosis and previous tuberculosis suggests a complex immunological etiology of retinal vasculitis. This assumption may be supported by the fact that a combined systemic treatment with pyrimethamine, trimethoprim, sulfamethoxazole and prednisolone reduced the retinochoroidal inflammation and periarterial infiltrates.

Adult↗
PubMed2002

Systemic non-Hodgkin's lymphoma with optic nerve infiltration in a patient with AIDS.

PURPOSE: To report the clinicopathologic features of a patient with AIDS and clinically regressed systemic non-Hodgkin's lymphoma who subsequently developed lymphomatous infiltration of the optic nerve and occlusion of the central retinal vein in both eyes. METHODS: The eyes of this patient were examined ophthalmologically and by fluorescein angiography. The eyes, brain, and body were obtained after death and studied by light microscopy. RESULTS: Ophthalmic examination and fluorescein angiography revealed optic nerve swelling and central retinal vein occlusion first in the left eye and shortly thereafter in the right eye. Postmortem histopathologic examination showed dense infiltration of both optic nerves by lymphoma as well as necrotizing vasculitis involving the retinal vessels near the left optic nerve head. Examination of the brain revealed lymphomatous involvement in the paraventricular region with associated necrosis and inflammation. No bone marrow recurrence or other residual systemic lymphoma was present. CONCLUSION: This case demonstrates that infiltrative optic neuropathy may occur as the sole ocular manifestation of disease recurrence in a patient with systemic non-Hodgkin's lymphoma otherwise thought to be in clinical remission.

Biomarkers, Tumor↗
PubMedDate not supplied

Bilateral acute retinal necrosis--a case report.

A 42 year old man presented with acute bilateral uveitis and necrotizing retinitis. Systemic investigations including test for AIDS and CMV retinitis were negative. Despite oral Acyclovir, both eyes progressed rapidly to retinal detachment with loss of vision. Early recognition is necessary to diagnose the bilateral acute retinal necrosis syndrome and initiate treatment. Bilateral acute retinal necrosis (BARN) is a term first coined by Young and Bird in 1978 although the syndrome had been originally described by Urayama et al as an unilateral condition. This syndrome is characterized by the triad of acute confluent peripheral necrotizing retinitis, moderate to severe vasculitis and vitritis in an otherwise healthy individual. Rhegmatogenous retinal detachment occurs within two to three months of the onset of the disease and the second eye is involved in 36% of patients, usually within 6 weeks. We herein report a patient who presented with simultaneous BARN leading to retinal detachment in a matter of days. Also, to our knowledge this is the first report of this condition in India.

Adult↗
PubMed2004

[Bilateral acute retinal necrosis syndrome associated with meningoencephalitis caused by herpes simplex virus 2. A case report].

INTRODUCTION: Acute retinal necrosis syndrome (ARN syndrome) is a rare viral disease with a poor prognosis in most cases. It is characterized by substantial ocular inflammation with progressive retinal necrosis, occlusive vasculitis and sometimes extraocular features. CASE REPORT: We report the case of a 62-year-old woman who was referred for a suspicion of a stroke. Ophthalmological examination revealed a profound bilateral visual loss due to extensive retinal necrosis. The patient was immediately treated with antiherpetic drugs. ARN syndrome with meningoencephalitis caused by herpes simplex virus type 2 was confirmed by PCR studies performed on aqueous humor and cerebrospinal fluid. Herpes simplex virus 2 (IgG+ , IgM-) was probably reactivated after intrathecal injection of steroids because of pain associated with narrowing of the lumbar vertebral canal. The patient was treated with intravenous Acyclovir for 3 weeks. After 4 months, both retinas were detached. DISCUSSION AND CONCLUSION: ARN syndrome caused by herpes simplex virus 2 most often occurs after reactivation of the latent virus in patients with a neurological medical history or congenital infection. Antiviral treatment must begin early to decrease risks of bilateralization and complications.

Acyclovir↗
PubMed1999

Macrophages and dendritic cells in IRBP-induced experimental autoimmune uveoretinitis in B10RIII mice.

PURPOSE: To investigate the characteristics of the mononuclear cell infiltrate in murine experimental autoimmune uveoretinitis (EAU). METHODS: EAU was induced by immunization with bovine interphotoreceptor retinal binding protein (IRBP) in Freund's complete adjuvant (subcutaneous injection) and pertussis toxin (intraperitoneal injection) in B10RIII mouse. Then animals were killed on days 7, 9, 12, 15, 20, 26, and 39 after immunization. Eyes were processed for hematoxylin and eosin staining to characterize the disease and to assess the severity and extent of the EAU. Single and dual immunohistochemical staining in various combinations with monoclonal antibodies against CD45, CD4, CD8, major histocompatibility complex (MHC) class II, CD11c, NLDC-145, and a variety of macrophage markers was performed. RESULTS: The authors' results showed that vitritis, vasculitis and perivasculitis, retinal detachment, and granuloma formation in retina and choroid were the predominant features of IRBP-induced B10RIII mice EAU. Immunohistologic results showed that CD4+ T cells and macrophages were the main infiltrating cells in retina and choroid throughout the entire course of the disease. MHC class II negative macrophages expressing antigens reacting with MOMA-2, F4/80, sialoadhesin, and CD11b were prominent during the peak phase of tissue damage in the retina and choroid. Dendritic cells (DCs) characterized by dual positivity for MHC class II and CD11c and negative for sialoadhesin appeared at time of disease onset and continued to be recruited during the inflammatory process. DCs at the site of inflammation were NLDC-145 weak and CD8 negative, indicating that they were of the myeloid rather than the lymphoid lineage. CONCLUSIONS: The results suggest that EAU in B10RIII mice is initiated by local-infiltrating, dendritic antigen-presenting cells, whereas tissue damage is associated with sialoadhesin-positive, phagocytic nonantigen-presenting macrophages during the effector stage.

Animals↗