Arteritic Ischemic Optic Neuropathy Research Articles

Sudden Visual Loss due to Arteritic Anterior Ischaemic Optic Neuropathy: A Rare Manifestation of Eosinophilic Granulomatosis with Polyangiitis.

eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem inflammatory disease characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of various organs. While the eye is uncommonly affected in patients with EGPA, multiple ophthalmic manifestations have been reported, which can result in serious visual impairment without timely treatment. we report the case of a 79-year-old woman with a history of asthma and nasal polyps who presented with low-grade fever, mild alteration of mental status, and fatigue. Chest X-ray revealed bilateral interstitial infiltrates. Lab tests showed elevated C-reactive protein level and eosinophilia (eosinophil count, 4.6 x109 cells/l); blood cultures and parasitological examination of stools tested negative. Four days after presentation, the patient reported sudden and severe blurring of vision in her left eye. Ophthalmological examination revealed bilateral swollen optic disc and visual field loss, more severe in the left eye. A diagnosis of EGPA complicated by arteritic anterior ischaemic optic neuropathy (A-AION) was proposed, while an alternative or concurrent diagnosis of giant cell arteritis was ruled out based on clinical picture. Immunosuppressive treatment with high-dose intravenous glucocorticoids was promptly started. The patient's visual defect did not improve; however, two months later, no worsening was registered on ophthalmic reassessment. A-AION is an infrequent but severe manifestation of EGPA, requiring prompt diagnosis and emergency-level glucocorticoid therapy to prevent any further vision loss. Disease awareness and a multidisciplinary approach are crucial to expedite diagnostic work-up and effective management of EGPA-related ocular complications. Arteritic ischaemic optical neuropathy is a potential cause of sudden and severe visual loss in eosinophilic granulomatosis with polyangiitis (EGPA) patients.Visual loss due to arteritic ischaemic optical neuropathy is rarely reversible; however, a timely glucocorticoid treatment may prevent further progression of visual impairment.Multidisciplinary approach is crucial to expedite diagnostic work-up and effective management of EGPA patients with ocular complications.

Can Optical Coherence Tomography Angiography be a Useful Adjunctive Diagnostic Tool in Patient with Non Arteritic Ischaemic Optic Neuropathy?

Abstract
 Introduction : Perfusion deficiency of the optic nerve head is presumed to have an immense role in the pathogenesis of non-arteritic ischaemic optic neuropathy (NAION). Optical coherence tomography angiography (OCTA) is a non-invasive method that can assess posterior pole vasculature at multiple levels. Thus, these case series want to compare the OCTA in the acute (before four weeks) and chronic phases (after six weeks) of NAION with their fellow unaffected eyes to give an overview of vasculature changes in NAION.
 Case Illustration : This case series consists of four patients with NAION. Half of them came with a sudden painless blurry vision, and the remaining presented with an abrupt visual field defect. All of them have diabetes mellitus and hypertension. None of them have superimposed vascular pathology. The first OCTA was taken within seven days of the first symptoms, and the second OCTA was taken more than six weeks after.
 Discussion : The high percentage of systemic vascular risk factors and the acute onset supported the vascular occlusive hypothesis in the pathogenesis of NAION. Meanwhile, the OCTA provides an actual overview of the vasculature changes. Microvasculature is more likely to expand in the acute phase, and a more significant expansion was found in greater optic disc edema. On the other hand, microvascular tend to attenuate in the chronic phase, representing the pruning of capillaries. A swept-source OCTA with deeper penetration might give a better peripapillary choriocapillaris image in the acute phase.
 Conclusion : OCTA is a promising adjunctive tool for diagnosing and monitoring progression in patients with NAION.

Diagnosis, Treatment, and Follow-Up of Giant-Cell Arteritis: A Retrospective Multicenter Study.

Giant-cell arteritis (GCA) is the most common type of vasculitis in the elderly and is associated with high risks of visual loss and recurrence. Owing to its rarity in Asian populations, the current clinical interventions for these patients are not well known. Here we aimed to characterize the current management status of patients with GCA using Korean multicenter data. This retrospective study analyzed medical records of patients with GCA at six Korean university hospitals from February 2009 to November 2022. GCA had originally been diagnosed based on the 1990 American College of Rheumatology (ACR) criteria, and cases were selected for inclusion in this study based on the 2022 ACR/European Alliance of Associations for Rheumatology criteria. We evaluated treatments, follow-up periods, and outcomes (relapse, remission, and adverse drug reactions) in patients with GCA with or without arteritic anterior ischemic optic neuropathy (AAION). This study analyzed 18 patients with a median age of 75.5 years that included 12 females (66.7%). Seven patients (38.8%) had AAION. All patients initially received prednisolone treatment, while four (22.2%) underwent adjuvant treatment with methotrexate and azathioprine during prednisolone tapering. During the median follow-up of 3.5 months (interquartile range: 2.0-23.2 months), 4 patients (22.2%) had prednisolone-related adverse reactions, 2 (11.1%) relapsed, and 13 (72.3%) dropped out. Nine patients (50.0%) experienced remission, with this being sustained in four (36.4%). This study observed high dropout rates and short follow-ups. Adverse effects of prednisolone were common, and relapses occurred in approximately one-tenth of Korean patients with GCA. Thus, optimizing GCA treatment necessitates regular monitoring and long-term follow-up.

A 66-Year-Old Woman Presented with Sudden Onset Eye Pain and Loss of Vision: Do Not Miss MOGAD

A 66 -year- old woman presented to ED with sudden onset eye pain and unilateral loss of vision. She had considerable vascular risk factors including age, hypertension, diabetes mellitus and hypercholesterolemia. She was referred to neurology service with possible arteritic anterior ischemic optic neuropathy (AION). Given her age, painful visual loss and raised ESR, she was treated with steroids for possible giant cell arteritis (GCA). Because of her psychiatric history and poorly controlled diabetes mellitus, steroids were weaned quickly. 6 months later, she had a similar presentation affecting the other eye. MRI head showed disease progression with positive myelin oligodendrocyte glycoprotein (MOG) antibody in serum. MOG associated disorder (MOG-AD) is a steroid responsive inflammatory demyelinating disease but needs long term immunosuppression plan and monitoring of serum antibody.

The Causes of Optic Disc Edema in Patients Presenting With Significantly Compromised Vision.

To evaluate the most common causes of optic disc edema (ODE) in patients with significantly compromised vision (initial best-corrected visual acuity [BCVA] of 20/400 or worse) at presentation. Retrospective chart review over a 5-year period of consecutive patients presenting to tertiary neuro-ophthalmology clinics at the University of Toronto. A total of 656 patients with ODE were included, and 49 patients (7.47%) had an initial BCVA of 20/400 or worse. There were 54 eyes included at baseline and 49 eyes at final follow-up. There were 29 female and 20 male patients. The mean age at first visit across patients was 55.9 years. Female patients (n = 29) were significantly older than male patients (n = 20) (P < 0.05). The causes of ODE were optic neuritis (ON) (n = 22; 40.7%), nonarteritic anterior ischemic optic neuropathy (NAION) (n = 22; 40.7%), arteritic anterior ischemic optic neuropathy (AAION) (n = 5; 9.26%), uveitis-related (n = 3; 5.56%), papilledema from idiopathic intracranial hypertension (IIH) (n = 1; 1.85%), and Vogt-Koyanagi-Harada disease (n = 1; 1.85%). Initial BCVA was not significantly different between ON and NAION groups (P = 0.52); however, final BCVA was significantly better in the ON group (P < 0.0001). The mean initial BCVA was worst in the AAION group (2.62 ± 0.54 logarithm of the minimum angle of resolution). The most common cause of ODE in patients <40 years old was ON (83.3%), whereas the 2 most common causes in patients >80 were NAION (60%) and AAION (40%). In patients between the ages of 60-80, NAION (100%) was the only cause. Patients with ODE and poor vision at presentation represent a minority of cases seen in neuro-ophthalmology clinics (<10%). Optic neuritis and NAION are the 2 most common causes of ODE with poor vision at presentation. These findings are limited by a small sample size and potential sampling bias.

[Nonarteritic anterior ischemic optic neuropathy (nAION)].

Nonarteritic anterior ischemic optic neuropathy (nAION) is the second most common degenerative disease of the optic nerve. The pathogenesis remains elusive. Atransient ischemia in the short posterior ciliary arteries not triggered by thromboembolic events is suspected. The typical history of asudden onset of scotoma without associated pain in conjunction with (sectorial) optic disc swelling, an afferent pupillary defect and avisual field defect are of decisive diagnostic importance. The most urgent diagnostic measure is the exclusion of arteritic AION. There are no proven treatment approaches. Frequently used but without clear study results, is the treatment with steroids and secondary prophylaxis with acetylsalicylic acid (ASA). Recurrence in the ipsilateral or contralateral eye is possible.

When Two is More Than Too Much: Carotid Stenosis and Giant Cell Arteritis (P1-9.002)

<h3>Objective:</h3> NA <h3>Background:</h3> NA <h3>Design/Methods:</h3> Case Report <h3>Results:</h3> A 72-year-old male was seen at the neuro-ophthalmology clinic for a right oculomotor palsy and right central retinal artery occlusion (CRAO) occurring within three days of each other. Stroke work up at an outside institution revealed the significant finding of a severe, right internal carotid stenosis for which a carotid endarterectomy was planned. The patient however developed a right CRAO before the endarterectomy could be performed. Though the patient reported experiencing headaches, unintentional weight loss, and jaw claudication as well as having elevated sedimentation rate, C-reactive protein, and platelet count at the time of initial presentation of the oculomotor palsy, the diagnosis of giant cell arteritis (GCA) was dismissed in favor of the carotid stenosis as the more likely etiology for the patient’s presentation. It was not until a couple of months after the patient’s initial diagnoses of right oculomotor palsy and CRAO that the diagnosis of GCA was made and appropriate treatment with steroids started. <h3>Conclusions:</h3> Though carotid stenosis has been reported as a cause of ischemic oculomotor palsies, this is much less common compared to GCA which has been known to cause diplopia from other extraocular cranial motor neuropathies as well as visual loss from arteritic ischemic optic neuropathy and CRAO. Though headaches and periocular pain could accompany ischemic extraocular motor neuropathies unrelated to GCA, clinicians should be more astute in recognizing other symptoms like jaw and tongue claudication, unintentional weight loss, anorexia, etc. that might indicate an alternative etiology–in this case, GCA–other than what is readily obvious, e.g., carotid stenosis. With both GCA and carotid stenosis having the potential to cause serious permanent disability, it is imperative to acknowledge the possibility of their coexistence and address each in the most appropriate and timely manner. <b>Disclosure:</b> An immediate family member of Dr. Santos has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Horizon Therapeutics. An immediate family member of Dr. Santos has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Cochrane. Dr. Santos has stock in Gilead Sciences. Dr. Santos has stock in Novocure. Dr. Santos has stock in Shockwave Medical. Dr. Santos has stock in Teva. An immediate family member of Dr. Santos has received research support from American Academy of Neurology. The institution of an immediate family member of Dr. Santos has received research support from Trinity Health Saint Mary’s Foundation. An immediate family member of Dr. Santos has a non-compensated relationship as a Delegate with AAN Neurology on the Hill that is relevant to AAN interests or activities. An immediate family member of Dr. Santos has a non-compensated relationship as a Participant with AAN Palatucci Advocacy Leadership Forum that is relevant to AAN interests or activities. An immediate family member of Dr. Santos has a non-compensated relationship as a Scholar with AAN Health Equity Symposium Scholarship that is relevant to AAN interests or activities. An immediate family member of Dr. Santos has a non-compensated relationship as a Participant with AAN Emerging Leaders Program that is relevant to AAN interests or activities.

Paracentral acute middle maculopathy progressing to central retinal artery occlusion following coronavirus disease vaccination: a multimodal imaging report

Background: Coronavirus disease (COVID-19) vaccines can cause adverse ocular effects, including vascular insults, acute macular neuroretinopathy, paracentral acute middle maculopathy (PAMM), ophthalmic vein thrombosis, Graves’ disease, arteritic anterior ischemic optic neuropathy (A-AION), and nonarteritic AION. Here, we report a case of unilateral PAMM progressing to central retinal artery occlusion (CRAO) after COVID-19 vaccination, identified using multimodal imaging.&#x0D; Case Presentation: A 24-year-old healthy man presented with unilateral progressive blurring of vision in the right eye. He had a recent history of fever without rashes 2 weeks after coronavirus disease vaccination. He was diagnosed with PAMM in the right eye at a local hospital and treated with a tapering dose of oral steroids. At presentation, he showed progressive blurring of vision in the right eye and the best-corrected distance visual acuity (BCDVA) was 20/60. The anterior segment was normal. Fundus examination revealed a pale optic disc with arteriolar attenuation and barrage laser scarring at the inferotemporal periphery. CRAO was diagnosed based on the right eye findings. The patient underwent multimodal imaging, including wide-field fundus photography using Optos® (Optos Carfornia®, Optos Inc., Dunfermline, United Kingdom), multicolor imaging with Spectralis™ (Heidelberg Retinal Angiograph; Heidelberg Engineering, Inc., Dossenheim, Germany), fundus fluorescence angiography (Heidelberg Retinal Angiograph; Heidelberg Engineering, Inc., Dossenheim, Germany), and optical coherence tomography angiography (ANGIOVUE, OPTOVUE, Inc., Fremont, CA, USA) using the split-spectrum amplitude-decorrelation angiography algorithm. The condition progressed from PAMM to CRAO during the oral steroid treatment course. At the 2-month follow-up, the right eye BCDVA had improved to 20/50, with fundus findings remaining the same as at the previous visit.&#x0D; Conclusions: This was the first report of a young patient with PAMM presenting with focal vascular occlusion that evolved to global occlusion in the form of CRAO in the absence of systemic vascular risk factors and with a normal coagulation profile. This case suggests that arterial occlusion may exert a temporary effect secondary to COVISHIELD™ vaccination. Randomized controlled trials and case – control studies on the role of vaccination in precipitating thromboembolic events in healthy individuals would provide insight into the causation.

A Rare Case of Posterior Ischemic Optic Neuropathy after Appendicectomy

Ischemic optic neuropathy is classified into anterior and posterior ischemic optic neuropathy depending upon the part of optic nerve involved. In anterior optic neuropathy, optic nerve head is involved and in posterior ischemic optic neuropathy(PION) retrobulbar portion is involved. There is sudden loss of vision in both the entities but there are optic disc changes in anterior optic neuropathy while in posterior ischemic optic neuropathy optic disc is normal initially. Etiologically, posterior ischemic optic neuropathy is divided into non arteritic non-surgical, arteritic and perioperative non arteritic posterior ischemic optic neuropathy.

Clinical Images: Increased bone metabolism in secondary hyperparathyroidism.

The patient, a 47-year-old man, was diagnosed as having severe hyperparathyroidism secondary to end-stage kidney disease (ESKD), the underlying cause being leukocytoclastic vasculitis with IgA nephritis and minimal-change glomerulonephritis, which was confirmed by kidney biopsy in 2008. The patient had been receiving hemodialysis since 2014 and had a history of multiple fractures. His treatment included azathioprine, low-dose prednisolone, calcium carbonate, vitamin D, sevelamer, cinacalcet, torasemide, carvedilol, amlodipine, moxonidine, simvastatin, low molecular weight heparin, and erythropoietin. After a sudden transitory episode of vision loss with increased C-reactive protein (18.1 mg/liter) and response to glucocorticoids, 18F–positron emission tomography/computed tomography (18F-PET/CT) evaluation was performed to rule out systemic vasculitis. Ophthalmologic examination was negative for arteritic ischemic optic neuropathy, ophthalmoparesis, loss of visual field, and diplopia. Imaging with 18F-PET/CT revealed enhanced fluorodeoxyglucose uptake in the skeleton (A and B) and thickening of cortical bone such as in the femurs (C). Bone loss as osteolytic lesions in the calvaria, increased uptake in the setting of diffuse skull thickening with loss of distinction between the inner and outer table (D), and increased uptake in mandible bones (E) could be seen. There was no evidence for vasculitis. ESKD is associated with a complex remodeling of bone, summarized as renal osteopathy (1). This might include bone resorption, periosteal reaction, brown tumors, osteoporosis, osteosclerosis, osteomalacia, and soft tissue/vascular calcifications. Imaging may reveal any number of these conditions (2, 3). In our patient, 18F-PET/CT revealed osteolytic lesions, which were considered brown tumors and interpreted as osteitis fibrosa cystica. The patient was hypocalcemic (adjusted serum calcium 1.90 mmoles/liter), with elevated alkaline phosphatase (758 IU/liter) and massively elevated parathyroid hormone levels (1,872 pmoles/liter), indicating severe secondary hyperparathyroidism. These 18F-PET/CT images reflect the widespread, strongly induced metabolic activity of bone in secondary hyperparathyroidism during ESKD. Disclosure Form Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

A Case of Subtype Giant Cell Arteritis Without Symptoms of Arteritic Anterior Ischemic Optic Neuropathy

Purpose: We report a case of papilledema without the typical symptoms of arteritic anterior ischemic optic neuropathy, ultimately diagnosed as a subtype of giant cell arteritis, which has not been reported previously in Korea.Case summary: A 72-year-old man visited the hospital with decreased visual acuity in his left eye of 0.02 that had occurred 1 week prior. Optic disc swelling and relative afferent pupillary disorder were also evident in the left eye. Magnetic resonance imaging revealed no specific findings, leading to a diagnosis of anterior ischemic optic neuropathy. Afterwards, there was no improvement in visual acuity, and optic nerve atrophy occurred. Six months later, the patient complained of decreased visual acuity in the contralateral eye; the same clinical findings as in the left eye were confirmed. Temporal artery biopsy was performed, ultimately revealing a subtype of giant cell arteritis.Conclusions: This case was finally diagnosed as a subtype of giant cell arteritis through temporal artery biopsy, despite the absence of typical clinical symptoms of this condition. Although giant cell arteritis is rare in Korea, it can cause permanent blindness. Therefore, the possibility of giant cell arteritis should always be considered with optic disc swelling in both eyes.

Two Cases in Which Arteritic Anterior Ischemic Optic Neuropathy Due to Giant Cell Arteritis Occurred Sequentially in Both Eyes

Purpose: To report two cases of arteritic anterior ischemic optic neuropathy (AAION) caused by giant cell arteritis (GCA) that occurred sequentially in both eyes.Case summary: An 86-year-old man presented with decreased visual acuity in the left eye that had commenced 3 days earlier. The left eye visual acuity was hand motion, and there was chalky-white optic disc swelling. AAION was diagnosed based on the myalgia, temporal headache, and temporal artery wall thickening. After steroid treatment, the visual acuity had not improved, while the visual acuity in the right eye was stable. A 94-year-old man presented with decreased visual acuity in the left eye that began 3 days earlier. The visual acuity in the left eye was light perception, and a subretinal hemorrhage was diagnosed. Two weeks later, he returned with decreased visual acuity in the right eye, and had chalky-white optic disc swelling in the right eye and decreased optic disc swelling in the left eye. AAION was considered based on the temporal headache and jaw claudication, and GCA was diagnosed after a temporal artery biopsy. Although systemic steroid was given, the visual acuity did not improve.Conclusions: The visual acuity of the eye that first developed AAION did not improve, but the prognosis of the later-onset eyes differed. Although GCA is rarely reported in Korea, delayed treatment can cause permanent visual loss. Therefore, in older adults with rapid visual loss and optic disc swelling, the possibility of GCA and a temporal artery biopsy should be considered.

Color Doppler Eye Ultrasonography in giant cell arteritis: differential diagnosis between arteritic and non-arteritic sudden blindness.

Temporal (TA) and axillary (AXA) arteries Color Doppler Ultrasonography (CDUS) is the most reliable diagnostic technique for the diagnosis of giant cell arteritis (GCA), displaying high sensitivity and specificity. Nevertheless, CDUS is still poorly performed in the common clinical practice, being employed only by rheumatologists with a relevant expertise in this field. Color Doppler Eye Ultrasound (CDEUS) is a procedure variously employed in ophthalmology and preliminary findings have displayed a possible role also in the diagnostic work-up of GCA. Aim of this study was to assess whether CDEUS may play a role in the differential diagnosis between arteritic and non-arteritic blindness. We prospectively included all patients evaluated since September 2021 to May 2022 by our Ophthalmology Unit for sudden blindness and referred to our Vasculitis Clinic in the suspicion of GCA. All patients underwent complete ophthalmological evaluation, routine blood tests, AxA and TA CDUS and CDEUS. According to the definite diagnosis, patients were divided in the following subgroups: (A) patients suffering from arteritic central retinal artery occlusion (CRAO), (B) patients suffering from non-arteritic CRAO, (C) patients suffering from arteritic anterior ischemic optic neuropathy (AION), (D) patients suffering from non-arteritic AION. During the observational period, we included a total of 25 patients suffering from sudden blindness and referred to Vasculitis Clinic for ruling out GCA. Patients belonging to group A showed no flow or reduced flow within the territory of central retinal artery (CRA), no "spot sign" and positive TA CDUS; on the other hand, patients from group B presented normal TA CDUS, no flow or reduced flow within the territory of CRA and the presence of "spot sign". Conversely, no relevant difference was evidenced at CDEUS in patients with and without arteritic AION. Our preliminary data displayed a good reliability of CDEUS in distinguishing between arteritic and non-arteritic CRAO, while no difference was assessed between arteritic and non-arteritic AION. Since AION represents the most common presentation of cranial GCA, CDEUS does not seem a reliable procedure in the diagnostic work-up of GCA and should be restricted only to the exclusion of thrombo-embolic occlusions within the territory of central retinal artery.

Optic neuropathies post-Covid 19 - review.

The Corona virus infection started at the end of 2019 in Wuhan - China and spread rapidly throughout the world, generating the Covid 19 pandemic. The manifestations of the Covid disease were extremely varied, from a simple flu, with fever, cough, weakness, headache, joint pain, up to severe pneumonia, with severe acute respiratory syndrome (SARS-Cov2) and even death. The symptomatology of the disease, the evolution and the complications that appeared varied, depending on the associated pathology - diabetes mellitus (DM), hypertension (HT), the age and the immune status of the patient. Aim: The ocular manifestations related to Covid 19 were mostly represented by conjunctivitis, but the neurotropic character of Corona virus could justify the appearance of certain neuro-ophthalmological manifestations, such as: optic neuritis (ON), cranial nerve palsies, visual field (VF) anomalies. The aim of this paper was to research the cases of optic neuropathy post-Covid 19, published in the specialty literature between 2020 and 2022. The following were evaluated: risk factors, distribution by age group and gender, evolution and complications, as well as the clinical forms of optic neuropathies. Materials and methods: We used Google Scholar and PubMed databases to find articles on optic neuropathies related to the Covid-19 infection. We followed the articles published during the pandemic and selected 21 cases, belonging to 17 authors, irrespective of their origin and the language in which they were written. Results: 21 patients affected by ON in the Covid-19 disease, 11 women and 10 men, were mentioned. The optic neuropathies described by the authors were: retrobulbar optic neuropathy, only one associated with myelin oligodendrocyte glycoprotein (MOG), papillitis, neuroretinitis, anterior ischemic optic neuropathy (AION), out of which one arteritic anterior ischemic optic neuropathy (AAION) and the others non-arteritic anterior ischemic optic neuropathy (NAAION), one being related to pronation in an oro-tracheal intubated (OTI) patient with acute respiratory distress syndrome (ARDS). Discussions: The neuro-ophthalmological complications associated with Covid 19 disease can be severe, so the patients should be monitored continuously. Many investigations (serological, immunological and imaging exams) are necessary to exclude other etiologies of ON. Conclusions: A complete ophthalmological exam is mandatory for each patient diagnosed with Covid 19 disease, even if they have ocular manifestations or not. Abbreviations: SARS-Cov2 = severe acute respiratory syndrome; DM = Diabetes mellitus; HT = Hypertension; ON = Optic neuritis; VF = Visual field ; NS = Nervous system; CRP = C-reactive Protein; CL = cytokines; IL = interleukins; TNFɑ = tumor necrosis factor; CNS = central nervous system; ACE = angiotensin-converting enzyme; CRVO = central retinal vein occlusion; MOG = myelin oligodendrocyte glycoprotein; MOG-AD = myelin oligodendrocyte glycoprotein antibody disease; BBB = blood-brain barrier; ARDS = acute respiratory distress syndrome; IOP = intraocular pressure; CVP = central venous pressure; MSOF = multiple systems organ failure; AAION = arteritic anterior ischemic optic neuropathy; NAION = non-arteritic anterior ischemic optic neuropathy; AION = anterior ischemic optic neuropathy; OCT = optical coherence tomography; CT = computer tomography; AFG = angiofluorography; MRI = magnetic resonance imaging; ESR = erythrocyte sedimentation rate; RF = rheumatoid factor; ANA = antinuclear antibodies; ANCA = antineutrophil cytoplasmic antibodies; AQP4 = anti aquaporin 4; NMO = neuromyelitis optica; CSF = cerebrospinal fluid; OTI = oro-tracheal intubated; VA = visual acuity; ONTT = optic neuritis treatment trial; RNFL = retinal nerve fiber layer; ICU = intensive care unit; LE = left eye; RE = right eye; MS = multiple sclerosis; ICH = intracranial hypertension; BCVA = best correction visual acuity; LP = light perception; APD = afferent pupillary defect; BM = biomicroscopy; PDN = prednisone; MTX = methotrexate; MTPN = methylprednisolone; NSAID = non-steroidal anti-inflammatory drugs; CGL = cells ganglion layer; VEP = visual evoked potential; CF = counting fingers.

Simultaneous Appearance of Arteritic Anterior Ischemic Optic Neuropathy and Central Retinal Artery Occlusion in Giant Cell Arteritis

Giant cell arteritis (GCA) is uncommon among Asian population. It is frequently associated with sight threatening complications. Simultaneous bilateral ocular involvement with different pathology is uncommon. We would like to highlight a rare case of GCA that was presented with transient visual loss over the right eye with simultaneous onset of central retinal artery occlusion as well as arteritic anterior ischemic optic neuropathy in both eyes. High dose intravenous methylprednisolone then subsequently maintenance dose of oral steroid and oral aspirin were given. His visual acuity remained the same after treatment. Early diagnosis and treatment of GCA is crucial. Visual outcome can be devastating if treatment is delayed.

Paracentral Acute Middle Maculopathy as a Specific Sign of Arteritic Anterior Ischemic Optic Neuropathy

The purpose of this study is to assess the diagnostic accuracy of paracentral acute middle maculopathy (PAMM) in the setting of anterior ischemic optic neuropathy (AION) to distinguish arteritic (A-AION) from nonarteritic (NA-AION) type. Retrospective cross-sectional diagnostic evaluation. PAMM was evaluated by 3 physicians blinded to diagnosis using macular spectral-domain optical coherence tomography. We studied 45 patients with AION. Of those, 28 had NA-AION and 17 had A-AION. The study was conducted in the Department of Ophthalmology at the Hospital of Marseille-Assistance Publique, France, from January 1, 2018, to March 31, 2022. PAMM were only found in the A-AION group (N=4) (P=.0143). As a distinctive sign of A-AION, we found a specificity of 100% (95% IC, 88.06%-100%) and a positive predictive value of 100%. In contrast, sensitivity and negative predictive value were lower, 19.1% (95% IC, 5.5-42.0) and 63.0% (95% CI, 58.1-67.7), respectively. The PAMM finding is highly specific for A-AION in the setting of AION. According to our results, macular spectral-domain optical coherence tomography looking for PAMM should be performed with any patient presenting with AION.

Optical Coherence Tomography of Peripapillary Vessels in Giant Cell Arteritis and Ischaemic Ocular Disease

ABSTRACT With normal retinal blood flow, cross-sectional optical coherence tomography (OCT) of retinal vessels shows a structured intravascular reflectivity profile, resembling a ‘figure-of-8’. Altered profiles have been reported in vascular occlusive and haematological diseases. Giant cell arteritis (GCA) can cause visual loss, usually due to anterior ischaemic optic neuropathy (AION) or retinal artery occlusion. Our aim was to extend the assessment of OCT vascular profiles to patients with suspected GCA and to determine if any abnormalities were related to GCA per se or to ischaemic ocular conditions. This nested retrospective study included 61 eyes of 31 patients (13 with GCA). Six eyes had arteritic and seven eyes non-arteritic AION, three eyes had non-arteritic retinal artery occlusion, 11 eyes had other ocular conditions and 34 were unaffected control eyes. For each eye the appearance of structured intravascular profiles on peripapillary OCT was graded as present, partial, absent or uncertain. Non-presence of structured intravascular profiles was more frequent in AION and retinal artery occlusion than in other ocular conditions or unaffected eyes (Fisher’s test, p = .0047). Based on follow-up of 25 eyes, reflectivity profiles normalised in three out of four eyes after 85 (35–245) days. Vessel profiles were not associated with GCA (p = .32) and were similar in arteritic and non-arteritic AION (p = .66). In conclusion, absence of structured intravascular reflectivity profiles may be a marker of acute ischaemia in the anterior optic nerve or inner retina. However, it did not seem specific for GCA. The prognostic value warrants further studies.

Diagnosis of giant cell arteritis using clinical, laboratory, and histopathological findings in patients undergoing temporal artery biopsy

ObjectiveTo identify the clinical, laboratory, and histopathological features that may predict the diagnosis of giant cell arteritis (GCA). MethodsA retrospective chart review was performed on patients who underwent temporal artery biopsy (TAB) between January 1, 2011 and March 31, 2019. Patient demographics, clinical characteristics, laboratory features, histopathological features, and biopsy results were collected. GCA status was determined by a neuro-ophthalmologist (OOA). Stepwise logistic regression analysis was performed to identify features that predict GCA status. ResultsOf 101 patients who underwent TAB, 31 (31%) were diagnosed with GCA. Age was found to be statistically significant for the diagnosis of GCA (P = 0.009), with an average age of 74.4 years ( ± 8.1) in those with GCA vs. 68.9 years ( ± 10.0) in those without. The incidence of transient vision loss was higher in GCA than non-GCA patients (P = 0.005). Anterior arteritic ischemic optic neuropathy (n = 3), ophthalmic artery occlusion (n = 2), and posterior ischemic optic neuropathy (n = 1) were seen only in the GCA group. Of the 31 GCA patients, 15 had active GCA (48%), 3 (10%) had healed temporal arteritis (HTA), 8 (26%) had suggested HTA, and 5 (16%) had false negative biopsies. Of the 70 non-GCA patients, 63 (90%) had negative biopsies, 2 (3%) had HTA, and 5 (7%) had suggested HTA. Histopathological analysis revealed that CD68 staining had a sensitivity of 69% and specificity of 86%. Both presence of multinucleated giant cells (MNGC) and transmural inflammation had 100% specificity; however, sensitivity was ≤ 50%. In patients with negative TABs and complete risk factor data available (n = 66), the odds of GCA increased 2.16-fold every 5 years of age, and 1.08-fold every mg/day of oral steroid use. A biopsy result of HTA had an odds ratio of 84.7 and suggested HTA of 49.2 against a negative TAB for diagnosis of GCA. ConclusionAge at time of biopsy, HTA, and suggested HTA are predictive for the diagnosis of GCA. Transient vision loss is more commonly seen in GCA, and anterior arteritic ischemic optic neuropathy, ophthalmic artery occlusion, and posterior ischemic optic neuropathy are important ophthalmic manifestations of GCA. CD68 staining is more sensitive but less specific for diagnosing GCA in comparison to other histopathologic findings such as presence of MNGC and transmural inflammation. Further work is recommended to investigate the importance of the specific histopathologic finding of CD68 staining in the diagnosis of GCA.

Management of ocular arterial ischemic diseases: a review.

To summarize the existing treatment options regarding central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO), arteritic anterior ischemic optic neuropathy (AAION), non-arteritic anterior ischemic optic neuropathy (NAION), and ocular ischemic syndrome (OIS), proposing an approach to manage and treat these patients. A systematic literature search of articles published since 1st January 2010 until 31st December 2020 was conducted using MEDLINE (PubMed), Scopus, and Web of Science. Exclusion criteria included case reports, non-English references, articles not conducted in humans, and articles not including diagnostic or therapeutic options. Further references were gathered through citation tracking, by hand search of the reference lists of included studies, as well as topic-related European society guidelines. Acute ocular ischemia, with consequent visual loss, has a variety of causes and clinical presentations, with prognosis depending on an accurate diagnosis and timely therapeutic implementation. Unfortunately, most of the addressed entities do not have a standardized management, especially regarding their treatment, which often lacks good quality evidence on whether it should or not be used to treat patients. Ophthalmologic signs and symptoms may be a warning sign of cardiovascular or cerebrovascular events, namely stroke. Most causes of acute ocular ischemia do not have a standardized management, especially regarding their treatment. Timely intervention is essential to improve the visual, and possibly vital, prognosis. Awareness must be raised among non-ophthalmologist clinicians that might encounter these patients. Further research should focus on assessing the benefit of the management strategies already being employed .

Case Report: Giant Cell Arteritis presenting with 6th Nerve Palsy without Ischemic Optic Neuropathy

Purpose: Giant cell arteritis (GCA) can be a difficult condition to identify in the early stages especially in the absence of the pathognomonic arteritic anterior ischemic optic neuropathy. Optometrists serve an important role in correctly triaging and initiating appropriate work-up and treatment for this emergent condition. This case report and review serves as a refresher of the systemic and ocular signs and symptoms of GCA. Background: GCA is a systemic autoimmune condition characterized by granulomatous inflammation of medium and large arteries in patients over 50. The most well-known constellation of signs and symptoms include new onset headache, jaw claudication, scalp tenderness, temporal artery abnormality with painless vision loss secondary to arteritic anterior ischemic optic neuropathy. However, it is important to be aware of alternate presenting signs and symptoms including pain anywhere in the distribution of the external carotid (occipital, neck, tongue, throat, ear) and signs of large vessel GCA (arm/limb claudication, chest/back pain, Raynaud’s phenomenon) and Polymyalgia Rheumatica (PMR) (bilateral hip/shoulder pain and morning stiffness). Case Report: An 86-year-old Caucasian female presents for a referred exam regarding new onset diplopia with concurrent occipital headache, neck pain and sore throat originally dismissed as symptoms of her other systemic health conditions. ESR, CRP and platelets were elevated on serology and subsequent temporal artery biopsy was positive for GCA. Oral steroids were initiated, and she was lost to follow up after her ocular symptoms resolved. Conclusion: GCA can present with a large range of manifestations, and many are nonspecific and easily attributable to other causes especially when it deviates from the classic constellation of new onset headache, jaw claudication, scalp tenderness and temporal artery abnormality with painless vision loss. Optometrists as primary eye care providers may be the first point of contact and need to be cognizant of the broader set of manifestations to minimize delays in diagnosis and treatment of this life and vision threatening condition. CE Notification: This article is available as a COPE accredited CE course. You may take this course for 1-hour credit. Read the article and take the qualifying test to earn your credit. Click here to Enroll (https://www.crojournal.com/case-report-giant-cell-arteritis-presenting-with-6th-nerve-palsy-without-ischemic-optic-neuropathy) Please check COPE course expiry date prior to enrollment. The COPE course test must be taken before the course expiry date.