Frequency of visual involvement in a 10-year interdisciplinary cohort of patients with giant cell arteritis.

BACKGROUND: We present the largest study of the frequency and nature of visual complications in a cohort of 350 patients consecutively diagnosed with giant cell arteritis (GCA). METHODS: All individuals were assessed using structured forms and diagnosed using imaging or biopsy. A binary logistic regression model was used to analyse data for predicting visual loss. RESULTS: Visual symptoms occurred in 101 (28.9%) patients, with visual loss in one or both eyes in 48 (13.7%) patients. Four patients had binocular visual loss. Anterior ischaemic optic neuropathy (N=31), retinal artery obstruction (N=8) and occipital stroke (N=2) were the main causes of visual loss. Of the 47 individuals who had repeat visual acuity testing at 7 days, three individuals had improvement to 6/9 or better. After introducing the fast-track pathway, the frequency of visual loss decreased from 18.7% to 11.5%. Age at diagnosis (odds ratio (OR) 1.12) and headache (OR 0.22) were significant determinants of visual loss in a multivariate model. Jaw claudication trended to significance (OR 1.96, p=0.054). CONCLUSIONS: We recorded a visual loss frequency of 13.7% in the largest cohort of patients with GCA examined from a single centre. Although improvement in vision was rare, a dedicated fast-track pathway reduced visual loss. Headache could result in earlier diagnosis and protect against visual loss.

Ischemic optic neuropathy as first presentation in patient with m.3243 A > G MELAS classic mutation.

BACKGROUND: Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a systemic disorder in which multi-organ dysfunction may occur from mitochondrial metabolism failure. Maternally inherited mutations in the MT-TL1 gene are the most frequent causes for this disorder. Clinical manifestations may include stroke-like episodes, epilepsy, dementia, headache and myopathy. Among these, acute visual failure, usually in association with cortical blindness, can occur because of stroke-like episodes affecting the occipital cortex or the visual pathways. Vision loss due to optic neuropathy is otherwise considered a typical manifestation of other mitochondrial diseases such as Leber hereditary optic neuropathy (LHON). CASE PRESENTATION: Here we describe a 55-year-old woman, sister of a previously described patient with MELAS harbouring the m.3243A > G (p.0, MT-TL1) mutation, with otherwise unremarkable medical history, that presented with subacute, painful visual impairment of one eye, accompanied by proximal muscular pain and headache. Over the next weeks, she developed severe and progressive vision loss limited to one eye. Ocular examination confirmed unilateral swelling of the optic nerve head; fluorescein angiography showed segmental perfusion delay in the optic disc and papillary leakage. Neuroimaging, blood and CSF examination and temporal artery biopsy ruled out neuroinflammatory disorders and giant cell arteritis (GCA). Mitochondrial sequencing analysis confirmed the m.3243A > G transition, and excluded the three most common LHON mutations, as well as the m.3376G > A LHON/MELAS overlap syndrome mutation. Based on the constellation of clinical symptoms and signs presented in our patient, including the muscular involvement, and the results of the investigations, the diagnosis of optic neuropathy as a stroke-like event affecting the optic disc was performed. L-arginine and ubidecarenone therapies were started with the aim to improve stroke-like episode symptoms and prevention. The visual defect remained stable with no further progression or outbreak of new symptoms. CONCLUSIONS: Atypical clinical presentations must be always considered in mitochondrial disorders, even in well-described phenotypes and when mutational load in peripheral tissue is low. Mitotic segregation of mitochondrial DNA (mtDNA) does not allow to know the exact degree of heteroplasmy existent within different tissue, such as retina and optic nerve. Important therapeutic implications arise from a correct diagnosis of atypical presentation of mitochondrial disorders.

Unilateral Optic Nerve Diffusion Restriction After Sinus Surgery Secondary to Central Retinal Artery Occlusion: Case Report and Literature Review.

INTRODUCTION: Permanent perioperative vision loss is caused by ischemic optic neuropathy (ION) or central retinal artery occlusion (CRAO). Whereas diffusion restriction of the optic nerve (ON) on brain magnetic resonance imaging has been previously reported in perioperative posterior ION (PION), there are no reports of ON diffusion restriction in patients diagnosed with acute perioperative CRAO. We present a case of perioperative CRAO to highlight this neuroimaging finding for neuroradiologists and neurologists. CASE REPORT: A 71-year-old male without vascular risk factors underwent maxillary bilateral antrostomy and septoplasty for chronic sinusitis. Twenty to thirty minutes upon awakening, he complained of painless left eye vision loss. Ophthalmoscopic examination showed retinal whitening, segmented arterioles, and hyperemic disc. Brain MR-diffusion weighted imaging/apparent diffusion coefficient revealed ON diffusion restriction in the proximal segment. Despite attempted reperfusion, left eye remained with no light perception at 6 months. Patients undergoing nonocular surgeries who develop perioperative vision loss related to PION may exhibit ON diffusion restriction but usually have normal ophthalmoscopic findings. CRAO shows retinal whitening, edema, segmentation of arterioles, and cherry red spot on ophthalmoscopy. A recent study reported that ON diffusion restriction in nonperioperative CRAO cases has a sensitivity and specificity of 55% and 70% to 100%. Here, PION was initially considered based on imaging. However, given the neuro-ophthalmic findings, a proximal embolus in the central retinal artery, obstructing its entrance into the proximal ON was deemed more likely. CONCLUSION: We highlight that proximal ON diffusion restriction on brain magnetic resonance imaging can be diagnostic of proximal thromboembolic CRAO. Future studies should evaluate the diagnostic utility and accuracy of MR-diffusion weighted imaging/apparent diffusion coefficient in perioperative visual loss.

Angioinvasive aspergillosis mimicking giant cell arteritis in an 81-year-old man with jaw pain and vision loss.

The present case report focuses on an immunocompromised 81-year-old patient initially diagnosed with Waldenström's disease. The patient experienced a gradual vision loss and jaw pain with high erythrocyte sedimentation rate. We first suspected giant cell arteritis, despite inconclusive assessment, including a negative temporal artery biopsy. We rapidly started a corticosteroid pulse therapy followed by high-dose corticosteroid therapy that was followed even after discharge from the hospital. The patient was readmitted 20 days later with severe left retro-orbital pain and progressive left vision loss. Clinical examination revealed complete left eyelid ptosis and unilateral blindness with fixed mydriasis and no eye movement. MRI showed signs of ischaemic optic neuropathy with lysis of the left ethmoid sinus wall; thus, indicating ischaemic optic neuropathy related to lymphoplasmacytic infiltration of Waldenström's disease (Bing-Neel syndrome). Oncological treatment of ibrutinib, a tyrosine kinase inhibitor, was then administered. Despite a favourable prognosis, no improvement was seen. An infectious aetiology was finally confirmed. The left sphenoid sinus biopsy highlighted an angioinvasive aspergillosis with rhino-orbital infiltration observed as ischaemic optic neuropathy. Oncologic treatment was discontinued and antifungal therapy with voriconazole was introduced, leading to a favourable radiological development and analgesic control, without ophtalmological improvement.

Paracentral Acute Middle Maculopathy Associated With Anterior Ischemic Optic Neuropathy and Cilioretinal Artery Occlusion in Giant Cell Arteritis.

ABSTRACT: Paracentral acute middle maculopathy (PAMM) is a relatively new optical coherence tomography finding, defined by hyperreflectivity in the inner nuclear layer. In this article, we present a case of a 73-year-old woman who presented with transient vision loss followed by the sudden onset of complete vision loss to counting fingers at 1 foot for one day in the left eye. Dilated examination showed a right cotton wool spot, left pallid optic disc edema, and retinal edema in the distribution of the cilioretinal artery. OCT demonstrated hyperreflective band at the level of the inner nuclear layer, compatible with PAMM. Clinical and laboratory findings were consistent with GCA, for which she was prescribed high-dose oral prednisone, with confirmation of GCA on a subsequent temporal artery biopsy. PAMM may be seen in the context of GCA, and OCT of the macula serves as an important adjunct to define the retinal manifestations of this condition.

Posterior ischaemic opticus neuropathy after radical cystectomy.

In this case report, a 58-year-old male with comorbidities of BMI 41 kg/m2, hypertension and diabetes type 2 underwent radical cystectomy. The operation was performed in 30-degree Trendelenburg and lasted > 7 hours with a total blood loss of 850 ml. The patient presented with painless bilateral vision loss upon awakening. MRI, CT and CT angiography of the cerebrum was performed and revealed arteriosclerosis and hypophysis adenoma. Neurological and ophthalmic consults were performed. Three weeks post-operatively, bilateral papillary atrophy was present, and posterior ischaemic optic neuropathy was confirmed.

Ocular manifestation of giant cell arteritis vs AL-amyloidosis: similar presentations but different approaches.

Light chain (AL) amyloidosis may present with the features of vasculitis, including giant cell arteritis (GCA). Similarities between GCA and AL-amyloidosis can potentially cause confusion in diagnosis, in which case, temporal artery biopsy (TAB) should be performed to make a definitive diagnosis. Herein we report a case of a bilateral anterior ischaemic optic neuropathy (AION), showing evidence of AL-amyloidosis on the temporal artery biopsy. A 75-year-old male with AL-amyloidosis secondary to monoclonal gammopathy of undetermined significance (MGUS) presented to our hospital for subacute painless progressive visual impairment. Based on his elevated inflammatory markers and his age, he was suspected to have giant cell arteritis. However, a temporal artery biopsy excluded GCA, and the Congo red staining was positive for amyloid deposition. This present case reveals that AL-amyloidosis may present with visual impairment, high inflammatory markers, and involvement of temporal arteries, concerning for GCA. TAB with Congo red staining is found to be crucial for making the correct diagnosis.

Vision Loss From Giant Cell Arteritis in Patients With Other Ocular Diagnoses.

ABSTRACT: Vision problems from giant cell arteritis (GCA) can be difficult to diagnose as patients may present with vision loss in the absence of systemic symptoms, have other comorbidities that affect inflammatory blood markers, or have other ocular diagnoses. We present 3 cases illustrating this point including a patient with advanced glaucoma with worsening vision from posterior ischemic optic neuropathy from GCA, a patient with arteritic anterior ischemic optic neuropathy (AAION) erroneously diagnosed as optic neuritis without elevated inflammatory blood markers due to corticosteroid use, and a patient with AAION and a history of nonarteritic anterior ischemic optic neuropathy in her fellow eye and untreated obstructive sleep apnea. GCA should be kept in the differential diagnosis for patients over 50 years of age even if they carry other ocular diagnoses. Temporal artery biopsy remains the gold standard for GCA diagnosis and is often required in equivocal cases.

Optic Disc Drusen Associated Anterior Ischemic Optic Neuropathy: Prevalence of Comorbidities and Vascular Risk Factors.

BACKGROUND: Nonarteritic anterior ischemic optic neuropathy (NA-AION) associated with optic disc drusen (ODD) is termed ODD-AION, where NA-AION with no evidence of ODD is simply termed NA-AION. Patients with ODD-AION have been found to be younger than those with NA-AION but with similar vascular risk factors. This study compares the known risk factors for NA-AION between a group with ODD-AION and a similarly aged group with NA-AION. METHODS: A case-control study of 13 patients with ODD-AION and 14 patients with NA-AION diagnosed in the period 2008-2017. All patients underwent an interview designed to evaluate history of vascular risk factors and comorbidities and re-examination including enhanced depth imaging optical coherence tomography to confirm the presence or absence of ODD. RESULTS: No significant differences were found in demographic or clinical characteristics between the ODD-AION and the NA-AION group. Significantly more ODD-AION patients than NA-AION patients had no vascular risk factors (smoking, arterial hypertension, diabetes mellitus, and dyslipidemia) present (P = 0.047). Significantly fewer patients in the ODD-AION group were diagnosed with arterial hypertension or dyslipidemia than in the NA-AION group. CONCLUSIONS: In this cross-sectional study, the ODD-AION patients more often had no vascular risk factors as compared to NA-AION patients, which supports the hypothesis that ODD are an independent risk factor for AION.

Rapidly Sequential Vision Loss From Posterior Ischemic Optic Neuropathy Due to Methicillin-Susceptible Staphylococcus Aureus Bacteremia.

A 63-year-old man with a history of high-grade bladder cancer was admitted to the intensive care unit (ICU) with renal failure and methicillin-susceptible Staphylococcus aureus bacteremia originating from his nephrostomy tube. While in the ICU, he had painless, severe loss of vision in the right eye followed by his left eye 12 hours later. Visual acuity was no light perception in each eye. He was anemic, and before each eye lost vision, there was a significant decrease in blood pressure. Dilated fundus examination was normal, and MRI showed hyperintense signal in the bilateral intracanalicular optic nerves on diffusion-weighted imaging and a corresponding low signal on apparent diffusion coefficient imaging. He was diagnosed with bilateral posterior ischemic optic neuropathies (PION), and despite transfusion and improvement in his systemic health, his vision did not recover. PION may be seen in the context of sepsis, and patients with unilateral vision loss have a window for optimization of risk factors if a prompt diagnosis is made.

Nonarteritic anterior ischemic optic neuropathy is associated with cerebral small vessel disease.

We investigated the presence of cerebral small vessel disease (SVD) in patients with nonarteritic anterior ischemic optic neuropathy (NAION) compared to control subjects without NAION to identify the association between NAION and cerebral SVD. We retrospectively reviewed the cases of 63 patients with NAION and 2749 control subjects without any neurologic and ocular diseases including NAION who underwent careful medical interviews, ophthalmic examinations, and magnetic resonance imaging (MRI) studies of the brain. We assessed and compared the degree of cerebral SVD on the MRIs. The patients with NAION presented with cerebral SVD more frequently than controls (68% versus 37%, respectively, p<0.001), which was also observed after adjusting for age, sex, comorbid conditions including hypertension, diabetes, and dyslipidemia, and smoking using the standardized mortality ratio (68% vs. 37%, p<0.001). A multivariate logistic regression analysis showed that the odds of cerebral SVD were 4.86 (95% CI, 2.10 to 11.24, p<0.001) times higher in patients with NAION than in the controls. We found that there was an association between cerebral SVD and NAION even after adjusting for age, sex, and medical histories. Clinicians should consider brain MRI scans in patients with NAION to prevent neurological impairment after cerebral SVD.

Risk of Stroke After Nonarteritic Anterior Ischemic Optic Neuropathy.

PURPOSE: To determine whether nonarteritic ischemic optic neuropathy (NAION) raises the risk of subsequent stroke in the general population. DESIGN: Population-based, retrospective cohort study. METHODS: Setting: Nationwide, population-based, retrospective cohort study. PATIENTS: Of 1 025 340 beneficiaries in the National Health Insurance Service-National Sample Cohort database (2002-2013), we included 400 952 eligible individuals in the analysis. OBSERVATIONS: To determine the effect of incident NAION on the occurrence of subsequent stroke, we used time-varying covariate Cox regression models. Model 1 included only incident NAION as a time-varying covariate. Model 2 included Model 1 and defined demographics. Model 3 included Model 2, comorbidity, co-medication, and Charlson index score. MAIN OUTCOME MEASURES: Effect (hazard ratio [HR]) of NAION on stroke development. RESULTS: Of 400 952 eligible individuals, 1125 patients developed NAION and 16 998 patients suffered from stroke. NAION was not associated with an increased risk of subsequent stroke in Model 1, with HR of 1.31 (95% confidence interval [CI], 0.89-1.92). This was consistent, after adjusting for demographics and/or confounding factors, in Model 2 (HR = 1.19, 95% CI, 0.81-1.75) and Model 3 (HR = 1.10, 95% CI, 0.75-1.62). CONCLUSIONS: Our results suggest that NAION per se is not associated with a subsequent risk of stroke in the general population.

Seeing the difference-Painless progressive vision loss in a vasculopath.

A 78-year-old Caucasian man with significant vascular disease reported sudden onset of worsened vision during a routine wound-care appointment for nonhealing necrotic leg ulcers. He described painless blurring of vision with grey scotomas in his right eye, his only well-seeing eye, after trauma to the left eye as a child. He presented with retinal ischemia, a cotton wool spot, and optic nerve swelling. Temporal artery biopsy showed myxoid degeneration and extensive calcium deposition, which also was present on imaging throughout his carotid and vertebral arterial system-the clinical picture of calciphylaxis.

Postoperative Visual Loss: A Report of One Patient With Unilateral Blindness After Orthognathic Surgery.

INTRODUCTION: Blindness after orthognathic surgery may be the result of the surgical procedure itself or the consequence of factors induced by general anesthesia. However, the exact mechanism between is not known. The purpose of this article is to present a case of a postoperative visual loss after orthognathic surgery under general anesthesia concluding with a brief literature review about this topic. REPORT OF CASE: We report the case of a patient who suffered unilateral blindness with homolateral frontal paresthesia after orthognathic procedure in 2 steps. He presented intraoperative bradycardia with a potential undiagnosed hypertension, associated with significant blood loss and volume resuscitation by colloids and cristalloids.Postoperative examination concluded to posterior ischemic optic neuropathy. DISCUSSION AND CONCLUSION: By a systematic literature review, we discuss about surgical and anesthesic causes of postoperative visual loss, and particularly pathophysiology mechanism of posterior ischemic optic neuropathy. Some predisposition and risk factors have been identified and need to be taken into account.

Simultaneous bilateral posterior ischemic optic neuropathy secondary to giant cell arteritis: a case presentation and review of the literature.

BACKGROUND: This report highlights a rare case of simultaneous bilateral blindness due to posterior ischemic optic neuropathy. Typically, ophthalmic involvement in giant cell arteritis is monocular or sequential ischemia of the anterior portion of the optic nerve, and less frequently simultaneous. CASE PRESENTATION: An 80-year-old Saudi male came with a history of simultaneous bilateral vision loss 5 days prior to presentation. The exam showed dilated non-reactive pupils, no light perception in both eyes, and normal fundus exam. C-reactive protein and erythrocyte sedimentation rate levels were high Magnetic resonance imaging and magnetic resonance angiography of the brain showed a right posterior optic nerve lesion and absence of flow in both ophthalmic arteries respectively. A left temporal artery biopsy confirmed giant cell arteritis. CONCLUSION: The presentation of GCA can be atypical and patients may present with simultaneous blindness. Bilateral simultaneous PION does not exclusively occur in a post surgical setting, emphasizing the importance of decreasing the threshold of suspicion of similar cases to avoid further neurological complications.

Diffusion-Weighted Imaging Changes in a Child With Posterior Ischemic Optic Neuropathy.

BACKGROUND: Posterior ischemic optic neuropathy results from ischemia of the retrobulbar aspect of the optic nerve. It presents as acute loss of vision without optic disc swelling. This is rare in children, with only seven cases reported to date. Neuroimaging is frequently used to aid in the diagnosis of acute visual complaints in children; however, none of the cases described to date delineate the neuroimaging findings of this entity in children. METHODS: We retrospectively reviewed the electronic medical record. RESULTS: We describe the MRI findings in a 10-month-old boy with posterior ischemic optic neuropathy after intraophthalmic artery injection of chemotherapy for retinoblastoma. CONCLUSIONS: As targeted therapies for retinoblastoma and other diseases amenable to intravascular treatment delivery are more frequently used, the risk of grave vision-related side effects increases. Posterior ischemic optic neuropathy should be considered in the differential diagnosis of any child presenting with acute loss of vision. Dedicated imaging of the orbits can elucidate specific findings that may aid in the diagnosis of this entity in children.

Etiologies of Optic Disc Edema in Tertiary Eye Care Centre in Nepal.

INTRODUCTION: Optic disc edema is a common clinical finding that can be caused by benign to vision and or life-threatening conditions. OBJECTIVE: To investigate the etiologies of optic disc edema among patients presenting to Tilganga Institute of Ophthalmology, Kathmandu, Nepal. MATERIALS AND METHODS: A retrospective chart review of patients with optic disc edema presenting to Neuro-ophthalmology department of Tilganga Institute of Ophthalmology from May 2012 to May 2014, was made. RESULTS: 98 patients were diagnosed with disc edema. Females (64%) were frequently affected. It was noted more in the 21 - 50 age groups. Papilledema was the most frequent cause (35.7%) of disc edema followed by papillitis (28.6%), pseudopapilledma (18.4%) and ischemia (17.3%), respectively. Brain tumors (13%) were the most common etiology for papilledema. CONCLUSION: Papilledema along with other causes, are common etiologies for disc edema. A detailed history and careful evaluation are necessary as the treatment strategy highly depends on it's underlying etiologies.

Unusual combination of bilateral ischaemic optic neuropathy following cardiac surgery.

Ischaemic optic neuropathy is a rare but serious complication post cardiopulmonary bypass in cardiac surgery patients. It presents with visual loss either unilaterally or bilaterally, and it can be anterior or posterior in type depending on the segment of the optic nerve involved. In non-ocular surgery patients, the most common type is called non-arteritic ischaemic optic neuropathy. We report a case of bilateral non-arteritic ischaemic optic neuropathy following coronary artery bypass grafting and mitral valve surgeries and review the published literature for the aetiology, management and prognosis of this rare complication.