Stroke and Multi-Infarct Dementia as Presenting Symptoms of Giant Cell Arteritis

Abstract

Cerebrovascular accidents (CVAs) and multi-infarct dementia have rarely been reported as presenting symptoms of giant cell arteritis (GCA), although 3%-4% of patients with GCA may present with CVAs during the course of the disease. We describe 7 patients with biopsy-proven GCA who presented with stroke or multi-infarct dementia. Most of them had other symptoms of GCA when the disease began that were misdiagnosed or not noticed. The internal carotid arteries were involved in 4 patients and the vertebrobasilar arteries in 3, with bilateral vertebral artery occlusion in 1. Small cerebral infarction foci on cranial computed tomography (CT) scan and magnetic resonance imaging (MRI) were found in 5 cases, and cerebellar infarction, in 2. MR angiography showed intracranial arteritis in 4 cases. Treatment with glucocorticoids and adjunctive antiplatelet or anticoagulant therapy was given in all cases, with neurologic improvement in 5. Two patients died. Necropsy demonstrated generalized GCA involving the medium and small cerebral vessels in 1 case. Central nervous system involvement is a rare complication in GCA but is important to recognize, as it can be reversible if diagnosed and treated promptly. Suspicion should arise in elderly patients suffering from strokes with a quickly progressing stepwise course and associated headache, fever, or inflammatory syndrome. In these cases, temporal artery biopsy should be performed without delay. Early diagnosis of GCA and immediate initiation of corticosteroid treatment may prevent progressive deterioration and death. Additional antiplatelet or anticoagulant therapy should be evaluated according to the individual risk and benefit to the patient under care.

Lethal giant cell arteritis with multiple ischemic strokes despite aggressive immunosuppressive therapy

“…In this case, the constellation of slow cognitive decline and profound bradykinesia led to a delay in the diagnosis of GCA. Notably, both patients seemed to suffer from a step-wise cognitive decline, which we postulate was the result of a vascular subcortical dementia, as described previously [5].…”

“…Several long-term studies have shown that treatment has an overall preventative effect on vascular complications [9,10]. Our two patients and others reported in the literature were characterized by a steroidresistant form of the disease with poor outcomes [2,3,5]. Glucocorticoid therapy may represent a double edged sword in the management of systemic vasculitis, with progression of vascular occlusion even after control of inflammation [11].…”

Immune Mediated Diseases and Immune Modulation in the Neurocritical Care Unit

“…Granulomatous involvement of the intracranial vessels is most often seen in the extradural space, and the inflammation can be severe enough to cause profound vessel narrowing and strokes. Most GCA-related strokes occur within the vertebral-basilar system [134,135].…”

Diagnosis and Treatment of Giant Cell Arteritis (Temporal Arteritis) – Past, Current and Future Aspects

“…Some rare neurological manifestations are summarized in Table 1. According to most reports, cerebrovascular ischemic events are found in 3-15% (65,97,98) of GCA, but are due to involvement of the extradural segment of the vertebral and carotid arteries. In one study, eight of 287 patients (2.8%) had strokes between the onset of GCA and 4 weeks after the onset of CS (97).…”