Atypical presentation of giant cell arteritis in a patient with vertebrobasilar stroke: A case report

Abstract

Rationale: Giant cell arteritis (GCA) is known to present with typical manifestations like temporal headache and visual abnormalities. However, several cases with atypical manifestations were reported. Stroke occurs in 3% to 7% of patients with GCA.

Patient concerns: A 67-year-old male patient with known hypertension presented with somnolence, disorientation and mild bilateral limb ataxia. The magnetic resonance imaging showed multiple acute infarctions in the territory of the vertebrobasilar system with occlusion of the left vertebral artery.

Diagnosis: Ten months later, during a routine neurovascular follow-up, recanalization of the left vertebral artery was observed and a hypoechoic concentric "halo" sign around both vertebral arteries, mainly on the left side was evident. On further examination of the superficial temporal artery, a hypoechoic concentric "halo" sign was also found, which-along with increased inflammatory markers-raised suspicion about GCA. Classical GCA features like headache, temporal tenderness or amaurosis fugax were not present. Repeated in-depth diagnostic work-up including 48 hours Holter-ECG did not reveal another stroke etiology.

Interventions: Intravenous Methylprednisolone 250 mg/d was immediately started and after 6 days the dose was tapered to 80 mg/d. The patient was discharged on a tapering scheme with the recommendation to start azathioprine. Additionally, we placed the patient on acetylsalicylic acid 100 mg/d and clopidogrel 75 mg/d. However, the patient was not compliant to treatment; he stopped prednisolone early and did not start azathioprine.

Outcomes: The inflammatory markers were markedly reduced at the beginning of the treatment. After stopping the immunosuppressive medications, the inflammatory markers were once again increased. Three months later, the patient developed bilateral middle cerebral artery and right occipital lobe infarctions.

Lessons: In patients with cryptogenic vertebrobasilar strokes, GCA may be considered in the differential diagnosis, especially if the inflammatory markers are increased.

Figure 1

Fluid-attenuated inversion recovery (A) and Diffusion-weighted imaging (B) in 12/2015 showed multiple small acute infarctions in the vertebrobasilar territory. Duplex ultrasound of the left vertebral artery showed occlusion signal denoting distal occlusion (C), which was confirmed in the DSA (D). FLAIR in 10/2016 showed a lacunar cavity in the pons (E), which was not present in the initial MRI images. Duplex ultrasound in 10/2016 showed recanalization and “halo” sign (red arrows) around the left vertebral artery (F). Ultrasound of the left superficial temporal artery in 10/2016 showed “halo” sign (red arrows) supporting the diagnosis (G).