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1 Aortitis from Giant Cell ArteritisGiant cell arteritis is a life-threatening inflammatory large vessel vasculitis, commonly associated with jaw claudication, temporal headaches, vision changes, and elevated ESR & CRP. This case highlights an additional common presenting feature of GCA at diagnosis - aortitis - and emphasizes the imp...Nithya Shanmugam; Valerie Biousse
2 Artifact from Incomplete Orbital Fat Suppression on Magnetic Resonance ImagingOrbital fat has short relaxation times that results in a hyperintense appearance on T1-weighted magnetic resonance imaging (MRI). Fat suppressed T1 MRI sequences are needed to remove the fat signal and better visualize the orbital anatomy, including the optic nerve. Contrast can be used with fat sup...Matthew Boyko, MD; Valérie Biousse, MD
3 Bilateral Optic Disc Edema from Hypertensive RetinopathyA 29 year-old woman was assessed for 2 weeks of headaches and 4 days of blurred vision in both eyes. Her blood pressure was 225/135. Her examination showed: best-corrected visual acuity: 20/25 OD, 20/30 OS; pupils equal and reactive without relative afferent pupillary defect; intraocular pressures 1...Benjamin I. Meyer, MD; Valérie Biousse, MD
4 Checkboard Visual Field Loss Due to Bilateral Homonymous HemianopsiasA 70-year-old man with vascular risk factors was seen for assessment of sudden visual field loss in both eyes. His examination showed: visual acuity: 20/20 OD and 20/25 OS; pupils: equal with no relative afferent pupillary defect; color vision: 14/14 plates correct OU; anterior segment exam: normal ...Rahul A. Sharma, MD, MPH; Valérie Biousse, MD
5 Choroidal Hypoperfusion Defect in Giant Cell ArteritisHere, we present a case of a 62 year-old male with vision loss in the right eye, headaches, and neck/shoulder/temporal pain, found to have choroidal hypoperfusion and diagnosed with giant cell arteritis (GCA). In combination with anterior ischemic optic neuropathy and cotton wool spots, choroidal hy...Nithya Shanmugam; Michael Dattilo; Valerie Biousse
6 Choroidal Infarction in Giant Cell ArteritisAn 80-year-old Caucasian woman presented with a 10-day history of headaches, intermittent binocular diplopia, and jaw pain. Temporal artery biopsy confirmed a diagnosis of giant cell arteritis. Examination showed characteristic large area of choroidal ischemia that is well-known to be associated wit...Wael A. Alsakran, MD; Andre Aung, MD; Valérie Biousse, MD
7 Choroidal Neovascular Membrane in Chronic PapilledemaA 21-year-old woman with papilledema from idiopathic intracranial hypertension developed a peripapillary choroidal neovascular membrane (PCNVM) complicating untreated chronic papilledema 10 years later.George Alencastro; Valerie Biousse
8 Classic Pathology Findings in Giant Cell ArteritisAn 80-year-old Caucasian woman presented with a 10 day history of headaches, intermittent binocular diplopia, and jaw pain. Temporal artery biopsy confirmed a diagnosis of giant cell arteritis. Pathology findings were classic for giant cell arteritis with numerous inflammatory cells in the tunica me...Andre Aung, MD; Corrina Azarcon, MD; Wael A. Alsakran, MD; Valérie Biousse, MD
9 Clinical Features of NeuroretinitisA 13-year-old girl was seen for assessment of blurred vision and optic disc edema in her right eye. Her examination showed: best-corrected visual acuity of hand motion OD and 20/25 OS; pupils: no relative afferent pupil defect; color vision: 0/14 plates OD and 14/14 plates OS; humphrey visual fields...Rahul A. Sharma, MD, MPH; Jason H. Peragallo, MD; Valérie Biousse, MD
10 Compressive Optic Neuropathy from Cavernous HemangiomaA 42-year-old man was seen for assessment of progressive blurring of vision in his left eye (OS) over several weeks. His examination showed a left optic neuropathy: visual acuity: 20/20 OD and 20/20-2 OS; pupils: 1+ left relative afferent pupillary defect; color vision: 14/14 plates correct OU. Ther...Rahul A. Sharma, MD, MPH; Aaron M. Yeung, MD; Valérie Biousse, MD
11 Cotton Wool Spots in Giant Cell ArteritisThis is a case of cotton wool spots in a patient with temporal artery-biopsy proven temporal arteritis.; ; A 66-year-old woman presents with isolated painless vision loss related to a left optic neuropathy in her left eye. She denies systemic symptoms to suggest giant cell arteritis.; Her examinatio...Rahul A. Sharma, MD, MPH; Valérie Biousse, MD
12 Dilated Episcleral Vessels from Carotid Cavernous Fistula51 year white woman with a 6 months history of chronic right eye redness, periorbital swelling and progressive proptosis. She was seen by multiple providers and treated for dry eye and conjunctivitis. Her examination showed normal visual acuity, color vision and pupils. There was an intraocular pres...Amani Alzayani, MD; Valérie Biousse, MD; Ling Chen Chien, MD
13 Direct Carotid-Cavernous Sinus FistulaA 40-year-old man presented with decreased vision and redness in his left eye. He had a significant trauma to the left side of his face about one year ago, but did not seek medical attention. External examination showed significant proptosis of the left eye (Figure 1) and conjunctival injection and ...Jonathan A. Micieli, MD; Valérie Biousse, MD
14 Fundus AutofluorescenceThe retinal pigment epithelium (RPE) has many important functions including phagocytosis of the photoreceptor outer segments. The metabolism of the photoreceptor outer segments leads to the formation of lipofuscin. Disease states and potentially increased oxidative damage can lead to the buildup of ...Jonathan A. Micieli, MD; Valérie Biousse, MD
15 Funduscopic Findings of Acute Central Retinal Artery OcclusionA 59-year-old man was referred for assessment acute vision loss in the right eye. His examination showed: best-corrected visual acuity: light perception OD, 20/20 OS; pupils: Relative afferent pupillary defect OD; color vision: unable to visualize control plate OD, 14/14 OS correct Ishihara plates. ...David B. Enfield, MD; Valérie Biousse, MD
16 Ganglion Cell Layer Analysis by Optical Coherence Tomography (OCT)A normal optical coherence tomography (OCT) of the macula is shown (Figure 1) and the various layers of the retina are labelled (Figure 2). The cell bodies of retinal ganglion cells (RGC) are located in the ganglion cell layer (GCL) of the retina and mostly synapse in the lateral geniculate nucleus ...Jonathan A. Micieli, MD; Valérie Biousse, MD
17 Giant Cell Arteritis: Temporal Artery Anatomy and HistologyGross anatomy and histology of the normal superficial temporal artery.; Histopathology of the superficial temporal artery involved by active and healed GCA; Summary of the main histopathologic findings in GCASamuel Bidot, MD; Valérie Biousse, MD
18 Homonymous Hemianopia Secondary to an Intracranial Bleed from an Arteriovenous MalformationThis case demonstrates a homonymous hemianopia resulting from hemorrhage secondary to a ruptured intracranial arteriovenous malformation (AVM), providing grounds for illustration and discussion of the correlations between localization of this lesion on cerebral imaging and resultant visual field and...Lauren Hudson, MD, PhD; Valérie Biousse, MD
19 Incipient Non-Arteritic Anterior Ischemic Optic Neuropathy (NAION)A 61-year old white man with hypertension, diabetes, and dyslipidema was seen in neuro-ophthalmology consultation for asymptomatic right optic disc edema. He had a small, crowded optic disc in the left eye known as a "disc-at-risk" (Figure 1). He had normal visual function including normal 24-2 SITA...Jonathan A. Micieli, MD; Valérie Biousse, MD
20 Incipient Non-Arteritic Anterior Ischemic Optic Neuropathy (NAION) Evolving to Symptomatic NAIONA 54-year old woman with hypertension was seen in neuro-ophthalmology consultation for asymptomatic left optic disc edema. She had a small, crowded optic disc in the right eye known as a "disc-at-risk" (Figure 1). Her visual function including 24-2 SITA-Fast Humphrey visual fields were normal in bot...Jonathan A. Micieli, MD; Valérie Biousse, MD
21 Internuclear Ophthalmoplegia (INO)A 67-year-old man with a known history of heart failure and atrial fibrillation developed binocular horizontal diplopia in right gaze after cardiac catheterization. His examination showed normal afferent visual function, full ocular movement of the right eye, and slow adducting saccades in the left ...Wael A. Alsakran, MD; Valérie Biousse, MD
22 Interpreting Ocular Fundus Photographs: a brief guideBrief guide for interpreting ocular fundus photographs.Gabriele Berman, MD; Sachin Kedar, MD; Nancy J. Newman, MD; Valérie Biousse, MD
23 Junctional Scotoma from a Sellar MassThis is a case of a 55-year-old woman presenting with gradual painless vision loss in both eyes. Although visual acuity was 20/20 in both eyes, there was a left relative afferent pupillary defect and diffuse pallor of both optic nerves (Figure 1). Visual fields (24-2 SITA-Fast) showed a temporal def...Jonathan A. Micieli, MD; Valérie Biousse, MD
24 Macular Star in the Setting of NeuroretinitisNeuroretinitis is often inflammatory or infectious in nature. Here, we present a case of Bartonella neuroretinitis and a typical finding of a unilateral "macular star" on color fundus photographs and OCT. The macular star usually appears 1-2 weeks after infection, and is secondary to exudates accumu...Nithya Shanmugam; Valerie Biousse
25 Malignant Hypertension With Bilateral Optic Nerve EdemaThis is a case of malignant hypertension and severe hypertensive retinopathy. A 30-year-old woman with headache and vision loss in the left eye was found to have a markedly elevated blood pressure of 205/100. CT head without contrast showed acute hemorrhage in the right temporal-occipital junction a...Rahul A. Sharma, MD, MPH; Michael Dattilo, MD, PhD; Valérie Biousse, MD
26 MRI Findings in Giant Cell ArteritisCase 1. An 80-year-old Caucasian woman presented with a 10-day history of headaches, intermittent binocular diplopia, and jaw pain. Temporal artery biopsy confirmed a diagnosis of giant cell arteritis. MRI with contrast showed enhancement of bilateral optic nerve sheaths in addition to enhancement o...Wael A. Alsakran, MD; Andre Aung, MD; Valérie Biousse, MD
27 Multimodal Imaging in Ocular SyphilisA 60 year-old-man with a 3-month history of painless blurred vision was found to have bilateral disc edema. Visual function was unremarkable OU except for enlargement of the blind spot OS. Brain and Orbits MRI showed mild enhancement at the left optic disc insertion and optic disc edema OS. Multimod...George Alencastro; Valerie Biousse; Étienne Bénard-Séguin
28 Myelin Oligodendrocyte Glycoprotein (MOG) - Antibody Optic NeuritisThis is an illustrated guide to the clinical diagnosis of myelin oligodendrocyte glycoprotein (MOG)-antibody optic neuritis. Myelin oligodendrocyte glycoprotein (MOG) is a glycoprotein on the surface of myelin and is found exclusively in the central nervous system (CNS). MOG likely mediates a comple...Bryce Buchowicz, MD; Valérie Biousse, MD
29 NeurosarcoidosisThis is an illustrated guide to the clinical diagnosis of neurosarcoidosis. Sarcoidosis is a chronic systemic inflammatory disorder characterized by non-caseating granulomas. It can affect multiple organ systems including the lungs, skin, orbit, and brain. When there is central nervous system (CNS) ...Bryce Buchowicz, MD; Valérie Biousse, MD
30 Non-Arteritic Anterior Ischemic Optic Neuropathy (NAION) With Segmental Optic Disc EdemaA 75-year old white woman with hypertension and diabetes presented with a 1 week history of vision loss in the right eye. Dilated fundus examination revealed superior segmental optic disc edema in the right eye and a small, crowded optic disc in the left eye known as a "disc-at-risk" (Figure 1). Int...Jonathan A. Micieli, MD; Valérie Biousse, MD
31 Optic Disc Edema and PseudoedemaA presentation covering how to approach optic disc edema, including clinical characteristics and the distinction of pseudoedema.Rahul A. Sharma, MD, MPH; Valérie Biousse, MD
32 Optic Disc MelanocytomaA 26-year-old woman was seen for assessment of an asymptomatic optic nerve abnormality in her left eye. Her examination showed normal afferent visual function: visual acuity: 20/30 OD and 20/25 OS; PH 20/20 OU; pupils: no relative afferent pupil defect; color vision: 14/14 plates correct OU. Humphre...Rahul A. Sharma, MD, MPH; Valérie Biousse, MD
33 Optic Disc Pseudoedema Due to Leber Hereditary Optic NeuropathyA 23-year-old woman developed sequential painless central vision loss in both eyes (right eye 5 months ago and left eye 2 months ago). Her examination showed bilateral optic neuropathies: visual acuity: 20/300 eccentrically OU (no improvement with pinhole); pupils: equal and reactive with no relativ...Rahul A. Sharma, MD, MPH; Valérie Biousse, MD
34 Optic Nerve Head Granuloma from SarcoidosisWe present a case of optic neuropathy with optic nerve head granuloma as the presenting sign of neurosarcoidosis. Initial patient presentation was notable for a chronic cough and worsening of vision in the left eye. Subsequent imaging revealed multiple brain lesions and the presence of a subcarinal ...Nithya Shanmugam; Valerie Biousse
35 Optic Nerve HypoplasiaThis is an illustrated guide to the clinical diagnosis of optic nerve hypoplasia. Optic nerve hypoplasia (ONH) is the most common congenital optic nerve anomaly, with an estimated incidence of 1 in 2287 live births. It may present unilaterally or bilaterally. It is seen in isolation or in associati...Rahul A. Sharma, MD, MPH; Valérie Biousse, MD
36 Optic Nerve Sheath MeningiomaThis is a case of an optic nerve sheath meningioma (ONSM) in a 56-year-old woman who presented with gradual, painless vision loss in her left eye. Optic disc photos at presentation showed temporal pallor of the left optic nerve (Figure 1) and Cirrus optical coherence tomography (OCT) of the retinal ...Jonathan A. Micieli, MD; Valérie Biousse, MD
37 Optical Coherence Tomography of the Retinal Nerve Fiber LayerA normal optical coherence tomography (OCT) of the macula is shown highlighting the position of a single retinal ganglion cell and its axon in the retinal nerve fiber layer (Figure 1). The topographical relationship of retinal ganglion cells in the retina to the visual field and position in the ante...Jonathan A. Micieli, MD; Valérie Biousse, MD
38 Pineal Gland Mass Causing Severe PapilledemaThe pineal gland is a neuroendocrine gland that resides in the epithalamus, at the root of the third ventricle. Due to its close proximity to the ventricles, masses in the pineal region can lead to obstruction of the flow of cerebrospinal fluid and subsequent raised intracranial pressure. Here, we p...Nithya Shanmugam; Valerie Biousse
39 Previous Branch Retinal Artery OcclusionThis is a typical case of an old branch retinal artery occlusion in a 64 year old woman presenting with persistent monocular vision loss. She had sudden onset of painless vision loss in the inferior field of her left eye approximately one year prior. Her past medical history was significant for atri...Benson S. Chen, MBChB FRACP; Valérie Biousse, MD
40 Retrograde Trans-Synaptic Degeneration from a Longstanding Occipital Lobe TumorThis is an illustrated guide that (1) discusses the localization of paracentral homonymous hemianopic scotomatous visual field defects and (2) discusses the concept of trans-synaptic retrograde degeneration. A 43-year-old woman was assessed for longstanding blurred vision in both eyes. Her examinati...Rahul A. Sharma, MD, MPH; Valérie Biousse, MD
41 Right Lateral Mediullary Syndrome (Wallenberg Syndrome) With Lateropulsion and Ocular Tilt ReactionA 55-year old man presented with acute onset right-sided facial numbness, left-sided body numbness, vertigo, right ptosis, and binocular vertical diplopia. External examination showed right ptosis and miosis indicating a right Horner syndrome (Figure 1). He had gaze-evoked nystagmus only on right g...Jonathan A. Micieli, MD; Valérie Biousse, MD
42 Right Occipital Arteriovenous Malformation presenting as a Migraineous Visual AuraMigraine with visual aura is a distinct entity from a migraineous visual aura. A migraine with visual aura is characterized by a visual aura that does not prefer either visual field and accompanies a headache. The mechanism of the visual aura is via cortical spreading depression. A migraineous visua...Nithya Shanmugam; Fernando Labella; Valerie Biousse
43 Second Order Horner Syndrome Revealing Metastatic Squamous Cell CarcinomaHorner Syndrome is secondary to a lesion of the ipsilateral sympathetic pathway and is associated with ptosis, miosis, and anhidrosis. Here, we present a unique presentation of metastatic squamous cell carcinoma in a patient with a right-sided Horner Syndrome (second order). We also highlight the di...Nithya Shanmugam; Michael Dattilo; Valerie Biousse
44 Second Order Horner Syndrome Revealing Metastatic Squamous Cell Carcinoma (video)Horner Syndrome is secondary to a lesion of the ipsilateral sympathetic pathway and is associated with ptosis, miosis, and anhidrosis. Here, we present a unique presentation of metastatic squamous cell carcinoma in a patient with a right-sided Horner Syndrome (second order). We also highlight the di...Nithya Shanmugam; Michael Dattilo; Valerie Biousse
45 Sequential Non-Arteritic Anterior Ischemic Optic Neuropathy (NAION)A 68-year old woman with hypertension, obstructive sleep apnea and obesity was seen in neuro-ophthalmology consultation for vision loss in the right eye. She had right optic disc edema with a small optic disc hemorrhage a small, crowded optic disc in the left eye known as a "disc-at-risk" (Figure 1)...Jonathan A. Micieli, MD; Valérie Biousse, MD
46 Severe Bilateral Optic Disc Edema in Hypertensive RetinopathyHypertensive retinopathy occurs when acute or chronically high blood pressure damages the retina. Here, we present a patient with progressive blurring of vision and a blood pressure of 270/160. Fundoscopic examination revealed arteriovenous nicking, copper wiring, cotton wool spots, and flame-shaped...Nithya Shanmugam; Valerie Biousse
47 Superior Segmental Optic Nerve HypoplasiaThis is a case of superior segmental optic nerve hypoplasia in a woman with a history of maternal diabetes. A 25 year-old woman noticed a visual field defect in her right eye. Her examination showed: visual acuity: 20/20 OD, 20/20 OS; pupils: trace relative afferent pupillary defect OD; color visi...Naa Naamuah M. Tagoe, MBChB, FWACS, FGCS; Rahul A. Sharma, MD, MPH; Valérie Biousse, MD; Nancy J. Newman, MD
48 Toxoplasmic Chorioretinitis with Unilateral Disc EdemaA 53-year-old man had a history of high myopia and a seronegative spondyloarthropathy treated with immunosuppressive agents. He presented with mild, painless vision loss in his right eye. His examination showed findings of a right anterior optic neuropathy: visual acuity: 20/20 OD (right eye), 20/20...Rahul A. Sharma, MD, MPH; Nancy J. Newman, MD; Valérie Biousse, MD
49 Typical Idiopathic Intracranial Hypertension: Optic Nerve Appearance and Brain MRI FindingsA 24-year old African American woman was referred for bilateral optic disc edema that was incidentally noted on a routine eye examination. She had excellent visual function and dilated examination showed bilateral optic disc edema with peripapillary wrinkles in the right eye and pseudodrusen in the ...Jonathan A. Micieli, MD; Valérie Biousse, MD
50 Typical Idiopathic Optic NeuritisThis is a case of a typical optic neuritis in a 41-year-old woman presenting with vision loss and pain with eye movements in the right eye. Optic disc photos at presentation showed subtle hyperemia in the right eye (Figure 1) and optical coherence tomography (OCT) of the retinal nerve fiber layer (R...Jonathan A. Micieli, MD; Valérie Biousse, MD
51 Vitreomacular Traction Syndrome (VMTS)Vitreomacular traction (VMT) syndrome is characterized by an abnormal vitreous adherence to the macula due to incomplete posterior vitreous detachment (PVD). The resulting traction produces morphological changes of the macula. Patients may complain of decreased visual acuity, photopsia, micropsia, o...Kirstyn Taylor; Sachin Kedar, MD
52 Vitreopapillary TractionA 64-year-old woman was referred for bilateral optic disc edema. Examination of her optic nerves showed indistinct margins at the nasal aspect of both eyes (Figure 1). Humphrey 24-2 SITA-Fast visual fields showed non-specific depressed points in both eyes (Figure 2). Optical coherence tomography (...Jonathan A. Micieli, MD; Valérie Biousse, MD
53 Vitreopapillary Traction Syndrome (VPT)Vitreopapillary traction (VPT) is characterized by abnormal vitreous adherence to the optic disc due to a fibrocellular membrane or incomplete posterior vitreous detachment (PVD). Traction on the optic disc can result in elevation of the disc, blurred margins, and rarely peripapillary hemorrhage. Pa...Kirstyn Taylor; Sachin Kedar, MD
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