Posner-Schlossman Syndrome European Study Group: study protocol and baseline patients characteristics of a multicentre study.

BACKGROUND: The aim of the Posner-Schlossman Syndrome European Study Group (PSS-ESG) is to acquire a comprehensive dataset of European patients with PSS. Here, we present the first report on the study protocol and the clinical findings of the patients at baseline. METHODS: The PSS-ESG is a retrospective, multicentre study designed to evaluate patients with PSS. The study, designed and driven by a European Expert Committee includes three datasets: (1) the baseline, (2) the follow-up and (3) the intraocular pressure (IOP)/glaucoma dataset. RESULTS: A total of 11 centres adhered to the PSS-ESG and 107 patients were included (68 males, 39 females) mostly Caucasian (93.4%). At uveitis onset, the patient's age ranged between 11 and 76 years, (mean age: 42±15 years).Best-corrected visual acuity was >0.5 in 80.3% of the eyes, IOP was >40 mm Hg in 44% of the eyes. Keratic precipitates were found in 78.5% of the eyes. No flare or cells in anterior chamber were detected in 56% and 53% of the cases, respectively. PCR analysis on aqueous sample was positive for cytomegalovirus-DNA in 50.6% out of the 81 tested patients. CONCLUSIONS: The PSS-ESG is the first multicentre study aimed to collect a comprehensive dataset of patients with PSS in non-Asian countries. A middlde-aged Caucasian male with a low-grade anterior chamber inflammation, keratic precipitates, preserved visual acuity and marked increased in IOP seemed to be the standard PSS patient across the 11 uveitis and glaucoma centres participating in the PSS-ESG.

Acute-Onset Retinal Conditions Mimicking Acute Optic Neuritis: Overview and Differential Diagnosis.

Acute optic neuritis (AON) is a common cause of sudden visual loss in young patients. Because of the risk of demyelinating disease, patients affected by unilateral or bilateral optic neuritis should be evaluated and treated accordingly. Despite advancements in imaging of the brain and retina, misdiagnosis of AON is not uncommon. Indeed, some acute disorders of the retina have the potential to mimic AON and their prompt diagnosis may avoid unnecessary neurologic investigation, psychological stress to the patient, and delays in treatment. This review describes uncommon retinal disorders presenting with sudden-onset visual loss and absent or subtle funduscopic manifestation that can mimic AON. Multimodal retinal imaging is essential in detecting these conditions and in their differential diagnosis. It behooves neurologists and general ophthalmologists to be aware of these entities and be familiar with multimodal imaging of the retina.

Hellp syndrome-related hypertensive chorioretinopathy: A multimodal imaging and optical coherence tomography angiography (OCTA) study.

INTRODUCTION: Pregnancy may be associated to unique retinal disorders and is associated to worsening of retinal disease that also occurs in non-pregnant females. We report a case of chorioretinopathy caused by pre-eclampsia associated to HELLP (Hemolysis-Elevated-Liver enzymes and Low Platelet count) syndrome. METHODS: Multimodal Imaging features of HELLP syndrome-related hypertensive chorioretinopathy including retinography, Spectral Domain-Optical Coherence Tomography (SD-OCT), Fluorescein angiography (FA), Indocyanine-green angiography (ICG) along with OCT-angiography (OCTA) are presented and discussed. RESULTS: Multimodal imaging and OCTA show both retinal and choroidal involvement by HELLP syndrome, resolved after hypertension treatment. CONCLUSIONS: Multimodal imaging is useful to study HELLP syndrome-related hypertensive chorioretinopathy. Moreover, OCTA is a new technology able to study and follow the circulatory status of the choriocapillaris during the disease.

PREDICTIVE ROLE OF SWEPT SOURCE OCT ANGIOGRAPHY IN RELAPSING VOGT-KOYANAGI-HARADA DISEASE.

PURPOSE: The purpose of this study was to describe the optical coherence tomography (OCT) angiography findings as a predictive role in the chronic relapsing stage of Vogt-Koyanagi-Harada disease and its comparison with other imaging modalities such as fluorescein angiography, indocyanine-green angiography, and spectral domain OCT. METHODS: A 37-year-old woman from Bangladesh was diagnosed with Vogt-Koyanagi-Harada disease. She was evaluated 8 months before for a routine examination when she was in clinical remission. Full ophthalmic evaluation with multimodal imaging and OCT angiography was performed. RESULTS: Ophthalmic evaluation was unremarkable. Spectral domain OCT disclosed increased choroidal thickness in both eyes while swept source OCT angiography imaging showed choroidal flow voids well-matching hypofluorescent round lesions found by indocyanine-green angiography. A week later the disease reactivated. CONCLUSION: Optical coherence tomography angiography may provide novel insights into inflammatory activity of the choroid and potentially have a predictive role in relapsing Vogt-Koyanagi-Harada disease.

Clinical Reasoning: Wilbrand's Knee, Scotoma of Traquair, and Normal Tension Glaucoma.

An otherwise healthy 63-year-old woman was given a diagnosis of normal tension glaucoma (NTG) in the right eye (OD) 2 months before presentation. Standard computerized perimetry showed a unilateral right hemianoptic temporal field defect. On examination visual acuity was preserved, intraocular pressure was normal, there was a right relative afferent pupillary defect (RAPD) with an asymmetric cupping of the disc, but no pallor. Brain magnetic resonance imaging (MRI) showed a meningioma compressing the right optic nerve at its junction with the chiasm. Compressive disorders on the anterior chiasm, albeit rarely, may cause cupping of the disc and unilateral temporal visual field defect (junctional scotoma of Traquair) with normal visual acuity that should be considered in the differential diagnosis of NTG.

Optical Coherence Tomography Angiography in Chiasmitis.

A 29-year-old girl presented complaining of acute bilateral visual loss associated with mild headache and retrobulbar pain. She was diagnosed with chiasmal optic neuritis caused by multiple sclerosis. Her visual acuity and visual field defect promptly improved after steroid therapy. However optical coherence tomography angiography showed a progressive reduction of superficial capillary plexus density of the retina and optic nerve consistent with the progressive impairment of the retinal ganglionar cell layer. Contrary to chiasmal compression, in chiasmal optic neuritis, the superficial capillary plexus density reduction is diffuse and does not reflect the peculiar anatomy of the chiasm.

Spontaneous chronic subdural hematoma as the cause of oculomotor cranial nerve palsy: A narrative review.

Acute complete third nerve palsy with pupillary involvement is usually caused by a posterior communicating artery aneurysm (i.e. "the rule of the pupil"). The pupillary fibers run peripherally in the third nerve and are thus susceptible to the external compression. Headache is usually present, and urgent diagnosis and treatment are warranted. Rarely, however, neuroimaging shows other causes of third nerve palsy. In this study, we perform a literature review of spontaneous chronic subdural hematoma that, although rarely, may cause an acute pupil-involving third nerve palsy as a false localizing sign. We review the localizing, nonlocalizing, and false localizing nature of ocular motor cranial nerve palsy in this setting.

Topical Nerve Growth Factor for the Treatment of Neurotrophic Keratopathy Caused by Wallenberg Syndrome.

PURPOSE: The aim of this study was to report a case of central neurotrophic keratopathy (NK) in Wallenberg syndrome (WS) and its successful management with topical recombinant nerve growth factor (rNGF). METHODS: A 47-year-old man with WS caused by a stroke in the territory of the left vertebrobasilar artery complained of progressive visual loss in his left eye (OS). Examination showed corneal anesthesia associated with a corneal epithelial ulceration consistent with a diagnosis of NK grade 3 of central origin. Topical treatment with rNGF, 1 drop 6 times daily, was started for 8 weeks, and the patient was followed up for 1 year. RESULTS: Topical treatment with rNGF was successful in promoting complete epithelial corneal healing. No recurrence was seen at 1-year follow-up. CONCLUSIONS: Clinicians should be aware that visual loss can also occur from NK of central origin. To the best of our knowledge, this is the first case report of NK caused by WS successfully treated with rNGF reported in the literature.

Intraocular Tuberculosis: A Challenging Case Mimicking Wet Age-Related Macular Degeneration.

An otherwise healthy 72-year-old Chinese patient diagnosed with exudative age-related macular degeneration and decreased vision in left eye was fully investigated. The retrospective analysis of past multimodal imaging revealed bilateral severe choroidal neovascularization and choroiditis associated with a positive tuberculin skin testing and interferon-gamma release assay (QuantiFERON-TB Gold - Cellestis®, Chadstone, VIC, Australia) suggestive of latent ocular tuberculosis. The variable presentation and tests' results interpretation represent the greatest limitations in understanding and treating intraocular TB (IOTB). This may present without any other systemic symptoms, the intraocular tissues are of limited access to biopsies and other tests, including imaging and immunological tests, are of relative value. This case highlights how variable may be the presentation of IOTB, which can be easily misdiagnosed leading to a delayed treatment and worse prognosis.

Idiopathic binasal hemianopia: case report and literature review.

A 56-year-old lady was referred for complete binasal hemianopia noticed during routine glaucoma screening. On examination the patient was asymptomatic, there were no ophthalmic causes explaining her visual field defect and further neurologic investigation was normal. Binasal hemianopia is an uncommon finding that is usually associated to intraocular conditions, but may rarely be caused by neurologic diseases. The Authors also review the current ophthalmic literature about binasal hemianopia in patients with otherwise complete neurologic and ophthalmic investigation (idiopathic binasal hemianopia).

Complete bilateral ophthalmoplegia in malignant intracranial hypertension in a child.

PURPOSE: To describe a case of fulminant idiopathic intracranial hypertension (IIH) in a child with "malignant" presentation. CASE REPORT: A 16-year-old, previously healthy, girl presented with bilateral visual loss and bilateral global limitation of eye movements in the absence of headache. Extensive laboratory evaluation for infectious, inflammatory, autoimmune, and neoplastic conditions was negative. Magnetic resonance imaging (MRI) of the brain and lumbar puncture findings were consistent with a diagnosis of IIH. Extraocular motility improved in the next few days as well as optic disc edema but visual acuity remained poor. CONCLUSION: The authors believe that the acute, severe, and fulminant ("malignant") presentation with markedly elevated intracranial pressure may produce the unique presentation of severe vision loss and bilateral complete ophthalmoplegia. Interestingly, there was no headache. To our knowledge this is the first such case to be reported in the English language ophthalmic literature.

Optical Coherence Tomography Angiography Findings in Optic Nerve Sheath Meningioma.

A 46-year-old female with a history of 5 months of progressive painless visual loss in the left eye was found to have an optic nerve sheath meningioma. Optical coherence tomography angiography showed a reduction in the superficial capillary plexus density consistent with her visual field defect and peripapillary retinal nerve fiber layer thinning. Moreover, abnormalities in the choriocapillaris were found in the affected eye compared to the fellow eye. Possible explanations for these findings are discussed. Further studies and a consistent number of cases are needed to correctly assess the impairment of ocular blood flow in optic nerve sheath meningioma.

Retrogeniculate Lesion of the Visual Pathways: Retinal Optical Coherence Tomography Angiography Shows Evidence of Transsynaptic Retrograde Degeneration.

Transsynaptic retrograde degeneration (TSRD) of the visual pathways is the loss of retinal ganglion cells occurring upstream from a posterior visual pathway lesion. We present a case of a 54-year-old woman with an epidermoid cyst at the right temporo-occipital junction, in whom retinal optical coherence tomography angiography showed a reduction of the superficial retinal capillary density consistent with TSRD. While this reduction has been described in chronic optic neuropathies and pregeniculate lesions, to our knowledge, this is the first case report showing how a reduction in the superficial retinal capillary density occurs also for a postgeniculate lesion.

Corneal Involvement in Wallenberg Syndrome: Case Report and Literature Review.

A 47-year-old man with Wallenberg syndrome after a stroke in the territory of the left vertebrobasilar artery and posterior inferior cerebellar artery, presented a week later complaining of visual loss in the left eye. Examination showed corneal anaesthesia associated with a neurotrophic corneal epithelial defect. Corneal involvement secondary to trigeminal neuropathy in Wallenberg syndrome is not frequently reported and may lead to epithelial erosion and neurotrophic keratopathy. Clinicians should be aware that visual loss can occur from a neurogenic basis (e.g., Wallenberg syndrome) due to neurotrophic keratopathy. The use of cocaine, a potential cause of neurotrophic keratopathy, should also be suspected in the appropriate clinical scenario.

Cilioretinal artery hypoperfusion and its association with paracentral acute middle maculopathy.

BACKGROUND/AIMS: To study the multimodal imaging findings of a large series of eyes with cilioretinal artery obstruction (CILRAO) and describe the systemic associations. METHODS: Multicentre, retrospective chart review from 12 different retina clinics worldwide of eyes with CILRAO, defined as acute retinal whitening in the distribution of the cilioretinal artery, were identified. The clinical, systemic information and multimodal retinal imaging findings were collected and analysed. RESULTS: A total of 53 eyes of 53 patients with CILRAO were included in the study. In 100% of eyes, fundus photography illustrated deep retinal whitening corresponding to the course of the cilioretinal artery. Twenty-eight patients (52.8%) presented with isolated CILRAO (baseline best-corrected visual acuity (BCVA) 20/50, final BCVA 20/25) associated with nocturnal hypotension, 23 patients (43.4%) with CILRAO secondary to central retinal vein occlusion (CRVO) (baseline BCVA 20/40, final BCVA 20/20) and two patients with CILRAO due to biopsy-proven giant cell arteritis (GCA) (baseline BCVA 20/175, final BCVA 20/75). With spectral domain optical coherence tomography (SD-OCT), a hyper-reflective band involving the inner nuclear layer (ie, paracentral acute middle maculopathy or PAMM) was noted in 51 eyes (28/28 eyes with isolated CILRAO and 23/23 eyes with CILRAO+CRVO) corresponding to the retinal whitening. In the two eyes with CILRAO+GCA, SD-OCT illustrated hyper-reflective ischaemia of both the middle and inner retina. CONCLUSIONS: Isolated CILRAO and CILRAO secondary to CRVO are the result of hypoperfusion or insufficiency, rather than occlusion, of the cilioretinal artery and are associated with PAMM or selective infarction of the the inner nuclear layer. With GCA, there is complete occlusion of the cilioretinal artery producing ischaemia involving both the middle and inner retina associated with worse visual outcomes.

Vogt-Koyanagi-Harada Disease Presenting as Unilateral Neuroretinits.

A 42-year-old female presented with unilateral visual loss associated with systemic symptoms of fever and headache. Although initial ophthalmic examination revealed a unilateral neuroretinitis, investigation for infectious and non-infectious causes of neuroretinitis were negative. At our examination, retinal imaging (suggestive of bilateral involvement) along with the results of lumbar puncture (pleocytosis) and clinical findings was consistent with a diagnosis of Vogt-Koyanagi-Harada disease. The patient was treated with intravenous steroids with prompt resolution of her symptoms. Vogt-Koyanagi-Harada disease may present atypically and should be considered in the differential diagnosis of neuroretinitis.