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Acute ischemic stroke (AIS) is a known complication of Takayasu arteritis (TAK); however, it is infrequently the first symptom observed. There have been no reports of concomitant AIS and anterior ischemic optic neuropathy (AION) as an initial manifestation of TAK. We present a case of TAK causing stroke and painless vision loss in a young Nigerian woman. A 33-year-old female patient presented with abrupt onset weakness in the right extremities and painless vision loss in her left eye. Her medical history included joint pain, malaise, syncope, and dizziness, along with peripheral vascular collapse, requiring recurrent hospital admissions. She had an absent left radial pulse, reduced left carotid pulse, and unrecordable blood pressure in her left arm. There was a relative afferent pupillary defect (RAPD), with fundoscopy findings indicating optic disc pallor. She had expressive aphasia, right facial paresis, and right flaccid hemiparesis. Brain computed tomography (CT) showed an infarct in the left middle cerebral artery (MCA) territory. The brain CT angiography showed diffuse enhancing aortic arch wall thickening and multiple aortic arch branch obstructive disease. The diagnosis was TAK complicated by left hemispheric infarctive stroke and left AION. She began treatment with prednisolone, azathioprine, and secondary stroke preventive care. Her vision improved to the ability to count fingers, with good functional outcomes and reduced disease activity. This case highlights the challenging diagnostic trajectory of TAK in a Nigerian female patient, featuring a unique multi-vessel affectation. Clinicians should be aware of the protean clinical presentations of TAK to reduce adverse cardiovascular complications.

GCA is a granulomatous vasculitis of unknown origin and targets large vessels with predominance for the aortic arch and the cranial branches. The underlying etiology of GCA is complex and has been widely researched, yet is still not well understood. The average age of presentation is 72.5 years for women and 70.3 years old for men. Furthermore, women are affected between 2 and 6 times more often than men. With vision loss, ophthalmologists are on the front lines of diagnosing the disorder. In addition to vision loss, patients commonly note associated symptoms such as headache, jaw claudication, diplopia, myalgias, and constitutional symptoms. The most commonly feared sequel of GCA is permanent visual loss secondary to arteritic anterior ischemic optic neuropathy (AAION). Here we present a series of case reports of GCA with their various presentations.

Abstract Objectives Giant cell arteritis (GCA) may lead to vision loss. To what extent tocilizumab (TCZ) is able to prevent vision loss is unknown. The aim was to analyze the occurrence of vision loss in a large GCA cohort treated with TCZ. Methods In this observational monocentric study, GCA patients treated with TCZ between the years 2010 and 2018 were studied. Demographic, clinical, and laboratory data were analyzed. Results A total of 186 patients were included (62% female); 109 (59%) fulfilled the American College of Rheumatology (ACR) criteria, in 123 (66%) patients, large vessel vasculitis was diagnosed by magnetic resonance-angiography (MRA). Cumulative duration of TCZ treatment was 224 years, median treatment duration was 11.1 (IQR 5.6–17.9) months. Glucocorticoids (GC) were tapered over a median of 5.8 (IQR 3.0–8.5) months. At baseline, visual symptoms were present in 70 (38%) and vision loss in 21 (11%) patients. Patients with vision loss at baseline were older (p = 0.032), had a lower C-reactive protein (p = 0.002), and showed a negative association with MRA of the aorta (p = 0.006). Two patients (1.1%) developed vision loss, both at the initiation of TCZ treatment. Conclusion Our data show a very low incidence of vision loss in TCZ-treated patient. The two cases of AION occurred at the initiation of therapy, they support the hypothesis that advanced, and established structural changes of arteries are key factors for this accident. Whether a shorter duration of concomitant GC treatment is risky regarding vision loss needs to be studied.

PurposeTo evaluate demographic and clinical characteristics of a Chinese population with giant cell arteritis using multimodal imaging focusing on ophthalmic examinations.DesignRetrospective observational case series.Materials and MethodsIn the neuro-ophthalmology division of the Eye, Ear, Nose, and Throat Hospital, Shanghai, we evaluated the demographic and clinical characteristics of patients diagnosed with giant cell arteritis between January 2016 and June 2021. Results of routine ophthalmic examinations including fundus examination, optical coherence tomography, color duplex ultrasonography of ocular and superficial temporal arteries, orbital magnetic resonance imaging, and superficial temporal artery biopsy were evaluated.ResultsA total of 15 patients (22 eyes; ten male and five female) were evaluated with a mean age of 77.0 ± 8.5 years. Among them, seven had bilateral involvement that occurred simultaneously or sequentially. Twelve patients presented with arteritic anterior ischemic optic neuropathy, two with arteritic anterior ischemic optic neuropathy combined with cilioretinal artery occlusion, and one with cotton-wool spots. In acute stages of optic neuropathy and retinopathy, optical coherence tomography revealed optic disc edema, thickening of the inner retinal nerve fiber layer and ganglion cell layer, and loss of layer structure. In late stages, optical coherence tomography revealed diffuse atrophy of the inner retina. The “halo” sign was observed in 12 patients in the superficial temporal artery ultrasound, and seven out of eight patients who underwent biopsy demonstrated classic giant cell arteritis pathological changes. Most patients having poor visual acuity but ability to perceive light; 10/22 eyes had permanent vision loss.ConclusionAlthough rare in Asians, giant cell arteritis may be underdiagnosed among elderly Chinese patients presenting with anterior ischemic optic neuropathy. Non-invasive superficial temporal artery ultrasound detecting inflammatory thickening of the intima as the “halo” sign combined with routine elevated erythrocyte sedimentation rate and C-reactive protein may be helpful in diagnosing patients with a high probability of having giant cell arteritis.

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Temporal arteritis is a systemic granulomatous vasculitis affecting medium- and large-sized arteries with wideranging and potentially devastating effects, from malaise to catastrophic vision loss, and even death. It is often misdiagnosed when it fails to present in the most classic form of an anterior ischemic optic neuropathy (AION) in an elderly patient. Clinicians need to be aware of the various clinical manifestations of the disease in order to facilitate prompt diagnosis and treatment. Temporal arteritis remains one of the few true ocular emergencies with which every practitioner must be familiar in order to prevent its devastating effects. [ABSTRACT FROM AUTHOR]

Severe acute respiratory syndrome coronavirus-2 (SARS-Cov-2) may cause various neuro-ophthalmologic manifestations including optic perineuritis. Optic perineuritis is a rare form of orbital inflammatory disease in which optic nerve sheath is inflamed and nonspecific fibrotic thickening with classic radiological finding is a perineural enhancement of optic nerve sheath. A 45-year-old gentleman with known diabetes mellitus, hypertension and dyslipidemia was admitted with a critically ill COVID-19 infection. During the recovery period, the patient developed sudden onset of painless loss of vision. MRI head and orbit with gadolinium was suggestive of optic perineuritis. Other secondary causes of autoimmune or vasculitis myelin oligodendrocyte glycoprotein (MOG) antibody disease and other common central nervous system (CNS) infection were excluded. The patient had dramatic response with steroids. This is the first rare case report of COVID-19-related optic perineuritis in critically ill COVID-19 patients with seronegative MOG antibody. Optic perineuritis is a rare orbital inflammatory disease and underlying mechanisms may arise from systemic response to COVID-19 infection as well as direct effects of the virus via angiotensin-converting enzyme 2 (ACE-2) receptors on ocular tissues. Optic perineuritis is a rare disease with inflammation restricted to the optic nerve sheath. Neuroimaging of the brain and orbit is the most important modality of choice for visualizing optic nerve sheath and optic nerve. Delay in the diagnosis of COVID-19-related optic perineuritis, may result in permanent optic nerve injury and irreversible vision loss., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2021, Ali et al.)