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Published on: March 26, 2019
Complex vasculitic overlap: temporal arteritis complicating suspected neuro-behçet disease with recurrent ischemic
Sanobar Shariff1,2, Samar Iltaf Mairajuddin2, Syed Habibullah Kamran2
1Yerevan State Medical University, Yerevan, Armenia.
Insights
Diagnosing giant cell arteritis (GCA) can be challenging due to overlapping symptoms with other vasculitic disorders. This case demonstrates successful GCA management through integrated clinical, imaging, and treatment response evaluation.
Area of Science:
- Rheumatology
- Neurology
- Vascular Medicine
Background:
- Vasculitic disorders often present with overlapping clinical and radiologic features, complicating diagnosis and management.
- Distinguishing Behçet disease-associated vasculitis from giant cell arteritis (GCA) is particularly challenging but crucial for effective treatment.
Purpose of the Study:
- To present a case highlighting the diagnostic challenges in differentiating between vasculitic syndromes.
- To emphasize the importance of integrating various diagnostic modalities for managing suspected GCA.
Main Methods:
- A patient with a history of thrombosis and suspected Behçet disease presented with neurological deficits and temporal headache.
- Neuroimaging revealed ischemic infarct and internal carotid artery occlusion; vascular imaging showed superficial temporal artery inflammation.
- Diagnosis of GCA was considered based on clinical presentation, inflammatory markers, and imaging findings, after excluding other causes.
Main Results:
- High-dose systemic corticosteroid therapy was initiated for suspected GCA.
- The patient experienced significant clinical improvement, with headache resolution and neurological stabilization.
Conclusions:
- This case underscores the diagnostic complexities of overlapping vasculitic syndromes.
- Successful management of suspected GCA relies on a comprehensive approach integrating clinical, imaging, and treatment response data.
Background:
Vasculitic disorders can present with overlapping clinical and radiologic features, complicating diagnosis and management. Differentiating between Behçet disease-associated vasculitis and giant cell arteritis (GCA) is particularly challenging but essential for appropriate treatment.
Case Presentation:
A 49-year-old woman with a history of recurrent venous and arterial thrombosis and suspected Behçet disease presented with severe temporal headache and acute focal neurological deficits.
Investigations:
Neuroimaging revealed a chronic ischemic infarct with occlusion of the right internal carotid artery and collateral circulation, without evidence of active intracranial vasculitis. Laboratory studies demonstrated elevated inflammatory markers. Vascular imaging showed inflammatory changes involving the superficial temporal artery.
Diagnosis:
Based on the presence of temporal headache, raised inflammatory markers, imaging evidence of superficial temporal artery inflammation, and exclusion of alternative causes, a diagnosis of giant cell arteritis was considered most likely in the setting of a complex vasculitic background.
Management:
High-dose systemic corticosteroid therapy was initiated.
Outcome And Follow-Up:
The patient showed marked clinical improvement following treatment, with resolution of headache and stabilization of neurological deficits. She continues under multidisciplinary follow-up for monitoring of disease activity and thrombotic risk.
Conclusion:
This case highlights the diagnostic challenges of overlapping vasculitic syndromes and emphasizes the importance of integrating clinical findings, imaging results, and therapeutic response in guiding management of suspected giant cell arteritis.
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