Photophobia as the Presenting Symptom of Internal Carotid Artery Dissection.

A 53-year-old man developed right eye photophobia after a hypertensive crisis, followed by right-sided headache and retro-orbital pain. On examination, a right Horner syndrome was evident and an urgent brain and neck Computed Tomography confirmed the suspicion of an internal carotid artery (ICA) dissection. While photophobia may occur in many neurological disorders, to our knowledge, it has never been described when the ICA is involved. Possible hypotheses of photophobia caused by carotid artery dissection are discussed.

Optical Coherence Tomography Angiography Findings in Deficiency Optic Neuropathy.

A 44-year-old man was evaluated for bilateral progressive visual loss and diagnosed with vitamin B12 deficiency optic neuropathy. Optical Coherence Tomography Angiography features of optic nerve and macula showed a decrease in peripapillary and macular vessel density that correlated well with the areas of retinal nerve fibre layer thinning seen on OCT. Further studies are needed to evaluate the role of this new technology in the evaluation of toxic and metabolic optic neuropathy.

Neuro-Ophthalmic Sarcoidosis.

Background: In the absence of confirmatory biopsy, the criteria for diagnosis of neuro-ophthalmic sarcoidosis are not well established. Diagnostic criteria for both intraocular sarcoidosis and neurosarcoidosis have been proposed, but the diagnosis of neuro-ophthalmic sarcoidosis remains challenging. It is our intention to augment what is currently known about the diagnosis of neuro-ophthalmic sarcoidosis by providing a series of biopsy-proven cases that contribute to the continued development of diagnostic criteria for this enigmatic condition. Methods: Case series of four Caucasian women with biopsy-proven neuro-ophthalmic sarcoidosis. Results: The first patient was initially diagnosed with traumatic optic neuropathy following a fall. Years later, the presence of pathologic submandibular lymphadenopathy was identified and biopsied, revealing non-caseating granulomas. The second and third cases involved sarcoidosis of the extraocular muscles without clear or common systemic features of sarcoidosis. In the fourth and final case, the patient presented with a Horner syndrome attributed to sarcoid infiltration of the ipsilateral sympathetic chain. Bronchoscopy with biopsy showed non-caseating granulomas consistent with sarcoidosis. Conclusions: We describe four cases of neuro-ophthalmic sarcoidosis and propose possible neuro-orbital and neuro-ophthalmic criteria both with and without diagnostic biopsy.

From A to Z(OOR): The Clinical Spectrum of Acute Zonal Occult Outer Retinopathy.

Acute idiopathic blind spot enlargement (AIBSE) is often caused by Acute Zonal Occult Outer Retinopathy (AZOOR), an outer retinal disease. We report two illustrative cases of AZOOR. The first one was a 21-year-old white female who presented with a scotoma and "shimmering lights" in her left eye. In the second case, a 73-year-old white female was referred for evaluation of a "bitemporal hemianopsia" that started years prior, with no clinical significant photopsias. To our knowledge, case two is the longest documented duration of bilateral, progressive, and chronic, idiopathic, enlargement of the blind spot (CIBSE) documented in the English language ophthalmic literature.

Serous macular detachment in ocular toxoplasmosis: A review.

Ocular toxoplasmosis, a disease of the eye caused by the protozoan parasite Toxoplasma gondii, represents a common cause of posterior uveitis. The Authors review the current Literature regarding the uncommon presentation of ocular toxoplasmosis as macular serous retinal detachment (SRD). It is imperative to keep in mind that inflammatory SRD is a possible presentation of toxoplasmic retinochoroiditis. Underestimation of this clinical scenario and treatment with steroids alone without appropriate antiparasitic drugs, could lead to devastating consequences.

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.

A Case of Spontaneous Bilateral Direct Carotid-Cavernous Fistula.

A 92-year-old female with poorly controlled systemic hypertension presented with bilateral eye redness, lid fullness, conjunctival chemosis, ophthalmoplegia, and ptosis for two days. A neuro-ophthalmic evaluation revealed bilateral proptosis, severe conjunctival chemosis and congestion, and an almost complete bilateral ophthalmoplegia with a complete right superior eyelid ptosis. Computed tomography (CT) scans demonstrated bilateral dilation of the superior ophthalmic veins, and a CT angiography (CTA) showed a direct high-flow carotid-cavernous fistula (CCF) with secondary extraocular muscle enlargement. Clinicians should be aware that a typical direct high-flow CCF, although usually occurs after trauma and unilaterally, can present spontaneously without trauma and bilaterally.

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.

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).

Acute zonal occult outer retinopathy complex disease: Lessons learned about choroid, photoreceptors, and retinal function.

PURPOSE: Retinal photoreceptors layer integrity is considered essential to visual function. We report a case of acute zonal occult outer retinopathy (AZOOR) complex disease (namely AIBSE: acute idiopathic blind spot enlargement) in which apparently a full anatomic regeneration is not needed for a complete functional recovery. METHODS: Case report with multimodal imaging. REPORTS: Visual field recovery in the presence of photoreceptors layer disruption studied by means of Optical Coherence Tomography. Choroid and photoreceptors layer thickness thinned progressively during recovery. CONCLUSION: This case suggests that anatomical retinal integrity as shown by OCT does not always correspond to visual function. Our case highlights that a complete visual recovery can occur even when structural abnormalities are still observable.

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.

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.

Tempo(ral) was the heart of the matter.

A 71-year-old woman was admitted with fever, headache, and weight loss associated with elevated inflammatory markers. She developed acute bilateral ophthalmoplegia and asymmetrical ptosis, rapidly followed by anterior ischemic optic neuropathy. Although the first temporal artery biopsy was negative, contralateral temporal artery biopsy revealed features consistent with giant cell arteritis. Even while under steroid therapy, she died a few days later from myocardial infarction. Acute bilateral complete ophthalmoplegia is a rare presentation of a limited number of possible diseases. Among these, giant cell arteritis should be suspected in the appropriate clinical scenario.

Uveitis and internuclear ophthalmoplegia as ocular manifestations of sarcoidosis.

Sarcoidosis is a multisystemic granulomatous chronic disease of unknown etiology with a wide range of clinical presentations. Diagnosis of sarcoidosis in patients with ocular manifestations can be challenging. We first describe a case of sarcoidosis presented with pulmonary involvement and both uveitis and internuclear ophthalmoplegia as ocular manifestations. A 55-year-old caucasian woman with non-productive cough and weakness presented with bilateral granulomatous anterior uveitis. Few days later, the patient presented again complaining of horizontal diplopia due to internuclear ophthalmoplegia. The diagnosis of sarcoidosis was made as a result of clinical examination and systemic investigations. Particularly, high-resolution computed tomography scanning of the chest was able to identify bilateral hilar lymphadenopathy not previously detected by chest X-ray. Biopsy confirmed diagnosis showing classic non-caseating granulomas.

The unfaithful neuritis.

A 55-year-old man with a history of mantle cell lymphoma reported acute blurred vision in the right eye. Although initially diagnosed with acute retrobulbar optic neuritis, 3 weeks later retinal infiltrates and vitritis developed, but vitrectomy resulted negative for vitreoretinal lymphoma. Further investigation revealed a serology positive for syphilis. This case highlights the role of the ophthalmologist in the diagnosis of syphilis, which is able to mimick multiple eye disorders, optic neuritis, and vitreoretinal lymphoma among others.

G/R 241 polymorphism of intercellular adhesion molecule 1 (ICAM-1) is associated with Fuchs uveitis.

PURPOSE: To investigate potential associations of the ICAM-1 gene polymorphisms and Fuchs uveitis in a cohort of Italian patients. METHODS: Seventy-one consecutive Italian patients affected by Fuchs uveitis were observed at the Ocular Immunology Unit, Arcispedale S. Maria Nuova (Reggio Emilia, Italy) from 2002 to 2008. Two hundred twenty-six healthy Italian blood donors from the same geographic area were selected as the control group. All Fuchs uveitis patients and control subjects were genotyped by polymerase chain reaction (PCR) and allele-specific oligonucleotide techniques for ICAM-1 polymorphisms at codon 241 (exon 4). RESULTS: The frequency of the ICAM-1 G/R 241 polymorphism was significantly higher in Fuchs uveitis than in the control subjects (16.9% vs. 5.8%; P=0.006, Pcorr=0.012; odds ratio, 3.3; 95% confidence interval, 1.4-7.7). No significant association between clinical features and ICAM-1 polymorphisms was found. CONCLUSIONS: This study demonstrates for the first time that the ICAM-1 G/R 241 polymorphism may represent a candidate gene for Fuchs uveitis susceptibility.