A Case of Giant Cell Arteritis Presenting As Catastrophic Posterior Circulation Stroke: A Diagnostic Dilemma

Wong, Joshua; Chan, Siang

doi:10.7759/cureus.27961

Cited by 4 publications

(4 citation statements)

References 15 publications

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“…It is noteworthy that only one case of stroke ( 196 ) reported in the literature was considered eligible for thrombectomy, suggesting that the real percentage of GCA-related stroke at onset could be underestimated in the presence of obvious risk factors like atrial fibrillation or atheromatosis. Moreover, a GCA-related general inflammatory state may increase the risk of an embolic event, and a mural thrombus due to cardiac stroke may occur ( 263 ).…”

Section: Resultsmentioning

confidence: 99%

Giant-cell arteritis related strokes: scoping review of mechanisms and rethinking treatment strategy?

Bonnan,

Debeugny

2023

Front. Neurol.

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Stroke is a rare and severe complication of giant cell arteritis (GCA). Although early diagnosis and treatment initiation are essential, the mechanism of stroke is often related to vasculitis complicated by arterial stenosis and occlusion. Its recurrence is often attributed to early steroid resistance or late GCA relapse, so immunosuppressive treatment is often reinforced. However, many questions concerning the mechanisms of stroke remain elusive, and no review to date has examined the whole data set concerning GCA-related stroke. We therefore undertook this scoping review. GCA-related stroke does not necessarily display general signs and inflammatory parameters are sometimes normal, so clinicians should observe caution. Ischemic lesions often show patterns predating watershed areas and are associated with stenosis or thrombosis of the respective arteries, which are often bilateral. Lesions predominate in the siphon in the internal carotid arteries, whereas all the vertebral arteries may be involved with a predominance in the V3-V4 segments. Ultrasonography of the cervical arteries may reveal edema of the intima (halo sign), which is highly sensitive and specific of GCA, and precedes stenosis. The brain arteries are spared although very proximal arteritis may rarely occur, if the patient has microstructural anatomical variants. Temporal artery biopsy reveals the combination of mechanisms leading to slit-like stenosis, which involves granulomatous inflammation and intimal hyperplasia. The lumen is sometimes occluded by thrombi (<15%), suggesting that embolic lesions may also occur, although imaging studies have not provided strong evidence for this. Moreover, persistence of intimal hyperplasia might explain persisting arterial stenosis, which may account for delayed stroke occurring in watershed areas. Other possible mechanisms of stroke are also discussed. Overall, GCA-related stroke mainly involves hemodynamic mechanisms. Besides early diagnosis and treatment initiation, future studies could seek to establish specific preventive or curative treatments using angioplasty or targeting intimal proliferation.

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Section: Resultsmentioning

confidence: 99%

Giant-cell arteritis related strokes: scoping review of mechanisms and rethinking treatment strategy?

Bonnan,

Debeugny

2023

Front. Neurol.

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“…The recent increasing use of high-sensitivity MR vessel-wall imaging also showed up to 40% intracranial arterial involvement [7] . While temporal artery biopsy has traditionally represented the gold standard diagnostic tool for GCA, the recognised complications and high false negative rate have resulted in increased utilisation of imaging investigations in GCA diagnosis [8] . Recently, vascular ultrasound has gained popularity, facilitating a quick, cost-effective and noninvasive GCA diagnosis, being also useful in disease monitoring, as in most patients wall thickening improves throughout treatment [1,3] .…”

Section: Discussionmentioning

confidence: 99%

“…Recently, vascular ultrasound has gained popularity, facilitating a quick, cost-effective and noninvasive GCA diagnosis, being also useful in disease monitoring, as in most patients wall thickening improves throughout treatment [1,3] . MR angiography can also be performed for GCA diagnosis and, together with FDG-PET and CT angiography, plays a key role in detecting inflammation and damage in extracranial vessels [1,8] . Ischaemic stroke is a rare but well-described complication in GCA, occurring in 2% -7% of patients, commonly resulting from vertebrobasilar system involvement [1,[9][10][11][12] .…”

Section: Discussionmentioning

confidence: 99%

Giant cell arteritis presenting as multiple ischaemic strokes: a successful case of endovascular treatment

Órfão,

Saca,

Alexandre

et al. 2024

European Journal of Case Reports in Internal Medicine

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Giant cell arteritis (GCA) may manifest with aggressive intracranial stenosis resistant to medical therapy, and patients may develop refractory neurologic deficits and cerebral infarcts, making GCA a life-threatening condition. We report the case of a 68-year-old woman recently diagnosed with GCA, medicated with prednisolone 60 mg daily. Two weeks later, the patient was admitted to our Stroke Unit after a sudden episode of global aphasia. Magnetic resonance angiography showed two recent ischaemic lesions, besides an erythrocyte sedimentation rate of 17 mm/hour. A cerebral angiography revealed bilateral stenosis and dilation in the petrous, cavernous and supraclinoid segments of internal carotid arteries (ICA). The patient was started on intravenous methylprednisolone pulses (250 mg daily for five days). Computed tomography (CT) angiography and Doppler ultrasound showed severe vascular disease affecting multiple territories, without significant intracranial involvement. The hypothesis of GCA with extracranial vasculitic involvement was considered as the aetiology of ischaemic cerebral infarctions in multiple territories and, given the severity of the disease, it was decided to add tocilizumab. Despite this, the patient evolved with significant worsening neurological deficits and a CT scan confirmed the presence of new vascular events. Endovascular treatment (EVT) with balloon angioplasty was conducted on both ICAs, with improved calibre and downstream filling. After that, the patient presented sustained clinical improvement, without recurrence of any ischaemic events at the one-year follow-up. This clinical case stands out for the importance of EVT as an effective therapy in patients with medically refractory GCA with symptomatic intracranial stenosis, improving their prognosis.

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“…It is the most common form of systemic vasculitis in adults, being more frequent in patients over the age of 50. The pathogenesis of GCA is T-cell dependent and antigen-driven, and clinical subsets of GCA appear to result from variable cytokine expression [1,2].…”

Section: Introductionmentioning

confidence: 99%

From Temporal Cell Arteritis to Giant Cell Aortitis Presenting as a Constitutional Syndrome: A Case Report

Lopes¹,

Rocha²,

Fonseca³

et al. 2023

Cureus

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Giant cell arteritis (GCA) is the most common form of systemic vasculitis in adults, especially in patients over the age of 50. It manifests most commonly with an intense headache and visual symptoms. Although constitutional symptoms are also frequent in GCA, these can be dominant in 15% of patients at first presentation and 20% of patients when relapsing. Treatment with high-dose steroids should be initiated as soon as possible to rapidly control the inflammatory symptoms and prevent ischemic complications, the most feared being blindness from anterior ischemic optic neuropathy.We present a case of a 72-year-old man who presented to the emergency department with a right temporal headache with retroocular radiation associated with scalp hyperesthesia, without any visual symptoms. The patient also reported low-grade fever, night sweats, anorexia, and weight loss over the last two months. The physical exam revealed a tortuous and indurated right superficial temporal artery, which was tender to palpation. The ophthalmological examination was normal. The erythrocyte sedimentation rate (ESR) and Creactive protein (CRP) were elevated, and he also had inflammatory anemia with a hemoglobin of 11.7 g/L. Due to this clinical presentation as well as the elevation of inflammatory markers, the diagnosis of temporal arteritis was suspected, and the patient was started on prednisolone (1 mg/kg). A right temporal artery biopsy was performed on the first week after the initiation of corticotherapy and was negative. After treatment initiation, there was a remission of symptoms accompanied by a decrease and normalization of inflammatory markers. However, after steroid tapering, there was a reappearance of constitutional symptoms but without any other organ-specific symptoms, such as headache, visual loss, arthralgia, or other. The corticosteroid dose was increased to the initial dosage, but there was no improvement in the symptoms this time. After the exclusion of other causes of the constitutional syndrome, a positron-emission tomography (PET) scan was performed, which showed a grade 2 aortitis. The diagnosis of giant cell aortitis was assumed, and given the lack of clinical response to corticotherapy, tocilizumab was initiated with a resolution of constitutional symptoms as well as a normalization of inflammatory markers.In conclusion, we report a case of temporal cell arteritis that further progressed to aortitis manifesting solely with constitutional symptoms. Furthermore, there was no optimal response to corticotherapy and no improvement with tocilizumab, therefore making this a case with a unique and infrequent clinical course. GCA is characterized by a wide variety of symptoms and organ involvement, and although it most frequently affects temporal arteries, it can be associated with aortic involvement that can cause life-threatening structural complications, highlighting the need for a high suspicion index for this condition.

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A Case of Giant Cell Arteritis Presenting As Catastrophic Posterior Circulation Stroke: A Diagnostic Dilemma

Cited by 4 publications

References 15 publications

Giant-cell arteritis related strokes: scoping review of mechanisms and rethinking treatment strategy?

Giant-cell arteritis related strokes: scoping review of mechanisms and rethinking treatment strategy?

Giant cell arteritis presenting as multiple ischaemic strokes: a successful case of endovascular treatment

From Temporal Cell Arteritis to Giant Cell Aortitis Presenting as a Constitutional Syndrome: A Case Report

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