Acute spinal cord compression caused by disseminated Nocardia infection involving the conus medullaris.

Distinguishing between an infective and malignant process provides a diagnostic challenge for clinicians. This case highlights an example of an acute spinal cord compression that could fall into either of these two categories. The diagnosis in this case of disseminated Nocardiosis is an extremely rare cause of acute spinal cord compression and to our knowledge intrinsic conus medullaris infection from Nocardia has not previously been reported in the literature. Nocardia cyriacigeorgica is an emerging strain of Nocardia species recently identified which was previously categorised as Nocardia asteroides type VI infection. The challenge of eliciting the diagnosis and the need to have an index of suspicion of Nocardia as a possible aetiology agent is shown in the report. The case shows this is especially important in evaluation of a multi-system infection in an immunosuppressed individual. The case described highlights an interesting diagnostic case with the resultant causative organism an emerging strain of Nocardia species with no previous reported cases of conus medullaris involvement.

Multifocal VEP and OCT in optic neuritis: a topographical study of the structure-function relationship.

PURPOSE: To investigate topographical relationship between amplitude of multifocal visual evoked potentials (mfVEP) and retinal nerve fibre layer (RNFL) thickness following acute optic neuritis (ON). PATIENTS AND METHODS: Fifty patients with a clinical diagnosis of acute unilateral ON between 6 and 36 months prior to the study and 25 age-matched controls underwent mfVEP testing (Accumap V 2.1, ObjectiVision Pty Ltd, Sydney, Australia) and OCT imaging (fast RNFL protocol, Stratus, software version 3.0, Carl Zeiss Meditec, Inc., Dublin, CA). RNFL thickness and mfVEP amplitude were measured for upper, temporal and lower retinal sectors and corresponding areas of the visual field in affected eyes of ON patients and control eyes. Inter-eye asymmetry coefficients for both RNFL thickness and mfVEP amplitude were calculated for each zone, and corresponding coefficients were correlated between each other. RESULTS: There was highly significant reduction of RNFL thickness and mean mfVEP amplitude in all three retinal sectors of the affected eye. Largest reduction of RNFL thickness was noticed in temporal sector and of mfVEP amplitude in corresponding central part of the visual field. RNFL thickness correlated highly with amplitude of the mfVEP derived from corresponding areas of the visual field in all three zones. CONCLUSIONS: We demonstrated strong topographical associations between structural and functional measures of optic nerve integrity in patients with ON.

Effect of fenofibrate on the need for laser treatment for diabetic retinopathy (FIELD study): a randomised controlled trial.

BACKGROUND: Laser treatment for diabetic retinopathy is often associated with visual field reduction and other ocular side-effects. Our aim was to assess whether long-term lipid-lowering therapy with fenofibrate could reduce the progression of retinopathy and the need for laser treatment in patients with type 2 diabetes mellitus. METHODS: The Fenofibrate Intervention and Event Lowering in Diabetes (FIELD) study was a multinational randomised trial of 9795 patients aged 50-75 years with type 2 diabetes mellitus. Eligible patients were randomly assigned to receive fenofibrate 200 mg/day (n=4895) or matching placebo (n=4900). At each clinic visit, information concerning laser treatment for diabetic retinopathy-a prespecified tertiary endpoint of the main study-was gathered. Adjudication by ophthalmologists masked to treatment allocation defined instances of laser treatment for macular oedema, proliferative retinopathy, or other eye conditions. In a substudy of 1012 patients, standardised retinal photography was done and photographs graded with Early Treatment Diabetic Retinopathy Study (ETDRS) criteria to determine the cumulative incidence of diabetic retinopathy and its component lesions. Analyses were by intention to treat. This study is registered as an International Standard Randomised Controlled Trial, number ISRCTN64783481. FINDINGS: Laser treatment was needed more frequently in participants with poorer glycaemic or blood pressure control than in those with good control of these factors, and in those with a greater burden of clinical microvascular disease, but the need for such treatment was not affected by plasma lipid concentrations. The requirement for first laser treatment for all retinopathy was significantly lower in the fenofibrate group than in the placebo group (164 [3.4%] patients on fenofibrate vs 238 [4.9%] on placebo; hazard ratio [HR] 0.69, 95% CI 0.56-0.84; p=0.0002; absolute risk reduction 1.5% [0.7-2.3]). In the ophthalmology substudy, the primary endpoint of 2-step progression of retinopathy grade did not differ significantly between the two groups overall (46 [9.6%] patients on fenofibrate vs 57 [12.3%] on placebo; p=0.19) or in the subset of patients without pre-existing retinopathy (43 [11.4%] vs 43 [11.7%]; p=0.87). By contrast, in patients with pre-existing retinopathy, significantly fewer patients on fenofibrate had a 2-step progression than did those on placebo (three [3.1%] patients vs 14 [14.6%]; p=0.004). An exploratory composite endpoint of 2-step progression of retinopathy grade, macular oedema, or laser treatments was significantly lower in the fenofibrate group than in the placebo group (HR 0.66, 95% CI 0.47-0.94; p=0.022). INTERPRETATION: Treatment with fenofibrate in individuals with type 2 diabetes mellitus reduces the need for laser treatment for diabetic retinopathy, although the mechanism of this effect does not seem to be related to plasma concentrations of lipids.

Use of botulinum toxin in neuro-ophthalmology.

Botulinum toxin produces a temporary chemical denervation, which initially was used to reduce the diplopia associated with paralytic strabismus. Subsequently, the drug was found to be effective in the management of dystonias, including hemifacial spasm and blepharospasm. More recently, there is evidence that botulinum toxin may lessen the pain associated with some movement disorders, tension headache, and migraine. The effect of botulinum toxin in temporary, but the therapeutic benefit usually is maintained even after repeated injections. The form of botulinum toxin used in clinical practice is the A serotype. Other serotypes may have a therapeutic role in the future. The mode of action and the clinical experience of botulinum toxin in neuro-ophthalmology is discussed.

Melanoma-associated retinopathy: does autoimmunity prolong survival?

PURPOSE: To discuss the autoimmune basis of melanoma-associated retinopathy (MAR) and its implications for management and prognosis. METHODS: An unusual history of a woman with melanoma-associated retinopathy is presented in detail. A review of published reports and a summary of 19 reported cases of MAR provide a basis for discussion. RESULTS: This case report and other published reports highlight a number of points regarding MAR including the male predominance, prolonged survival in several patients and lack of response to immunosuppression. CONCLUSIONS: Melanoma-associated retinopathy may prolong the survival of patients with metastatic melanoma through the autoimmune response. Therefore, treatment of visual symptoms with immunosuppression needs to be considered carefully.

Steroid management in giant cell arteritis.

AIM: Ocular involvement in giant cell arteritis (GCA) is an ophthalmic emergency which, if untreated, can progress to permanent blindness. There is little evidence in the literature to support current protocols for the acute treatment of GCA with steroids. The authors sought to review the effects of intravenous and oral steroids in GCA. METHODS: This retrospective study reviewed the records of 100 consecutive patients with biopsy proved giant cell arteritis. 73 patients with visual loss who were treated at the Royal Victorian Eye and Ear Hospital (RVEEH) and St Vincent's Hospital were included in the final series. The authors studied the management of the patients in the first week after presentation, analysing types of treatment, dose, effect on visual acuity, and complications. RESULTS: All the patients except one had visual loss due to anterior ischaemic optic neuropathy (AION). 17 patients (23%) had bilateral eye involvement. Visual acuity improved in 21 of 73 patients (29%) by a mean of two Snellen chart lines after commencement of steroids. There was an increased likelihood of improved vision in the group who had intravenous steroids (40%) compared with those who received oral steroids (13%). In all except four patients (95%) vision remained stable at 1 month review. CONCLUSIONS: Prompt treatment of GCA with steroids leads to improvement of visual acuity in a significant number of cases. Intravenous steroids may offer a greater prospect of improvement compared with oral steroids. A prospective trial comparing intravenous with oral steroids is needed to validate these findings and would not expose elderly patients to unacceptable risks.

Acute multifocal placoid pigment epitheliopathy and central nervous system involvement: nine new cases and a review of the literature.

OBJECTIVE: The authors describe nine new cases of acute multifocal placoid pigment epitheliopathy (AMPPE) with associated central nervous system (CNS) involvement and permanent visual sequelae. The study includes a review of the literature and discussion of evaluation, management, and treatment options. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Nine patients were identified with AMPPE and CNS involvement in addition to 22 patients reviewed in the literature. MAIN OUTCOME MEASURES: A review of nine patients with AMPPE and CNS involvement was performed. Charts were reviewed for age, gender, preceding viral prodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment. RESULTS: Thirty-one patients (nine new patients) were diagnosed with AMPPE and various degrees of CNS involvement. Ages ranged from 8 to 54 years, with an average of 27 years. Twenty-one males (68%) and 10 females (32%) were identified. Eleven patients (35%) had antecedent viral illnesses. Visual acuity was variable and ranged from 20/20 to count fingers. The spectrum of CNS findings ranged from headaches to sagittal sinus thrombosis. CONCLUSIONS: Acute multifocal placoid pigment epitheliopathy can be associated with CNS abnormalities and permanent visual deficits. Neuroimaging, lumbar puncture, and cerebral angiography analysis provide useful diagnostic tools when CNS involvement is suspected. Intravenous corticosteroids and collaboration with neurovascular colleagues should be considered in these situations. In cases complicated by CNS arteritis, immunosuppressive agents can be a beneficial adjunct to corticosteroids.

Ocular adnexal lymphoproliferative disease: a series of 73 cases.

BACKGROUND: This study involved 73 patients with lymphoproliferative lesions of the ocular adnexa. The lesions were categorized using the Revised European American Lymphoma classification of lymphoid tissues and analysed to determine the frequency and prognostic impact of tumour type, location, stage and patient's age and sex. METHODS: The clinical, histopathological, immunohistochemical and phenotypic analysis by flow cytometry and follow-up data were studied. RESULTS: The ocular adnexal lymphoproliferative lesions included 70 lymphomas and six reactive lymphoid hyperplasia. Seventy-nine per cent had stage IE disease, 4% stage II, 1.5% stage III and 15.5% stage IV. Five patients (7%) had a past history of systemic lymphoma. Major histological types were extranodal marginal zone lymphoma (MZL) in 44 (63%), follicular (FL) in 12 (17%), diffuse large B-cell (DLBCL) in eight (11%), mantle cell (MCL) in two (3%), B-cell chronic lymphocytic leukaemia (CLL)/small lymphocytic lymphoma in two (3%), peripheral T-cell lymphoma (PTCL) one (1.5%) and natural killer cell lymphoma (NKCL) in one (1.5%). Longest survival was seen in those with low-grade lymphomas (MZL and FL) and worst in PTCL and NKCL. Lymphoma-related mortality was 2% for MZL, 33% for FL, 38% for DLBCL, and 100% for MCL, PTCL and NKCL. Systemic lymphoma was present prior to, at presentation or at subsequent follow up in 26/68 (39%) of all lymphoma patients, 17% for MZL, 38% for DLBCL, 83% for FL, and 100% for MCL, CLL, PTCL and NKCL. CONCLUSION: The majority of ocular adnexal lymphomas were low-grade B-cell lymphomas (MZL). Multivariate analysis showed that the only significant independent predictors of all causes of mortality were the histological type of lymphoma and the stage of disease at presentation.

Carotid dissection: a common cause of Horner's syndrome.

PURPOSE: To highlight that internal carotid artery dissection is a common cause of Horner's syndrome and that it is important to diagnose dissection as anticoagulation can prevent carotid thrombosis and embolism. METHODS: Five case reports are presented of patients with Horner's syndrome secondary to carotid dissection. One patient had carotid dissection secondary to trauma, two had spontaneous dissections and two had dissections in the settings of other illness. A literature search was performed on carotid dissection as a cause of Horner's syndrome and its diagnosis and management. RESULTS: The case reports and literature highlight that dissection is under-recognized as a cause of Horner's syndrome and can be missed. The investigation of choice is a magnetic resonance imaging and angiography scan of the head and neck. The treatment advocated is anticoagulation for 3-6 months. CONCLUSION: Carotid dissection should be suspected in patients with Horner's syndrome, particularly if head or neck pain is present.

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), Behçet'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.

Ocular-central nervous system lymphoma mimicking posterior scleritis with exudative retinal detachment.

OBJECTIVE: We describe an unusual ocular presentation of ocular-central nervous system lymphoma in a young patient. DESIGN: Interventional case report and literature review. METHODS: A previously well 24-year-old white woman presented with left eye pain and reduced vision. Episcleral injection, globe tenderness, an afferent pupil defect, and exudative retinal detachment were present. Computed tomographic scan of the head and orbits demonstrated scleral thickening, retinal detachment, and no other abnormality. A provisional diagnosis of posterior scleritis with exudative retinal detachment was made. Investigation for underlying connective tissue diseases was negative. There was an initial prompt response to corticosteroid therapy. The patient's symptoms and signs then recurred, and a left third cranial nerve palsy developed. Systemic investigations including lumbar puncture ultimately led to the diagnosis of primary T-cell central nervous system (CNS) lymphoma. Serologic tests for human immunodeficiency virus were negative. MAIN OUTCOME MEASURES AND RESULTS: The patient underwent orbital and cranial irradiation and intrathecal and systemic chemotherapy. Despite an initial response to treatment, she returned with a recurrence of the lymphoma in the anterior segment of the left eye. Her systemic disease progressed rapidly, and she died shortly thereafter. CONCLUSIONS: This patient's young age and initial presentation mimicking posterior scleritis with unilateral exudative retinal detachment, without evidence of vitreous involvement, are highly unusual for ocular involvement in primary CNS lymphoma. A review of the literature highlights the atypical nature of this presentation.

Leber's idiopathic stellate neuroretinitis: differential diagnosis and approach to management.

BACKGROUND: Leber's idiopathic stellate neuroretinitis (LISN) is a relatively uncommon clinical entity that is characterized by optic disc oedema and associated macular hard exudates. A broad range of causes can produce such a fundus appearance. METHODS: The clinical experience of the authors is combined with a review of the pertinent literature to describe the features of LISN and its differential diagnosis. CONCLUSION: A knowledge of the potential causes of neuroretinitis is important in order to formulate an effective management strategy.

Retinal arteriovenous malformation with fluctuating vision and ischemic central retinal vein occlusion and its sequelae: 25-year follow-up of a case.

A patient with a retinal arteriovenous malformation (AVM) had experienced episodic visual loss with spontaneous recovery for many years, followed by permanent visual loss secondary to central retinal vein occlusion. She subsequently progressed to development of retinal neovascularization extending onto the posterior vitreous face with recurrent vitreous hemorrhage requiring vitrectomy. A brief review of the literature is presented, and the cause of the fluctuations in vision and central retinal vein occlusion are discussed.

Cancer associated retinopathy and non-small cell lung cancer.

The syndrome of cancer associated retinopathy is a relatively recently described entity that has characteristic clinical and electrophysiological features. It is most closely associated with small cell carcinoma and a specific antibody against a retinal protein. We present a patient with cancer associated retinopathy and a non-small cell lung cancer. The diagnosis of the clinical syndrome led to the discovery of the tumour. The antibody was not present. Therapy with chemotherapy, corticosteroids and plasma exhange did not improve the retinopathy. Earlier recognition of the syndrome may be important in detecting malignancy and attempting treatment of the retinopathy before irreversible damage is done.