Safaa Mourabit*^,1, Safaa Mhaber¹, Achraf Elkabli¹, Leila Barakat², Khadija Echchilali², Mina Moudatir² and Hassan El Kabli²

¹Resident Physician, Department of Internal Medicine, Ibn Rochd University Hospital, Casablanca, Morocco
²Professor in the Department of Internal Medicine at the Ibn Rochd University Hospital in Casablanca, Morocco

*Corresponding author: Safaa Mourabit, Resident Physician, Department of Internal Medicine, Ibn Rochd University Hospital, Casablanca, Morocco

Abstract

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Behçet's disease, characterized by systemic manifestations primarily affecting the vascular system, presents unique challenges in diagnosis and management, particularly when arterial involvement occurs. This case report discusses a 50-year-old patient with Behçet's disease, highlighting the importance of recognizing and addressing arterial complications. The patient, diagnosed with bipolar aphthosis at 49, developed deep venous thrombosis and bilateral panuveitis, showing improvement with corticosteroids, azathioprine, and anticoagulation. A subsequent presentation a year later included deep vein thrombosis, loss of consciousness, and paraparesis, alongside mouth ulcers. Cerebral-medullary MRI revealed inflammatory arterial stenoses and tuberculous spondylodiscitis. A coincidental discovery of pulmonary embolism complicated therapeutic options, with satisfactory evolution following treatment. Discussion emphasizes the intricate nature of Behçet's disease, known for systemic and vascular involvement, especially in arteries. The rarity of cerebral arterial manifestations underscores the need for heightened awareness to diverse morphological presentations.
Managing arterial complications requires a tailored therapeutic strategy, balancing immunosuppression and potential risks. Collaborative efforts among specialists are crucial for refining treatment approaches and improving outcomes.
In conclusion, this case report underscores the importance of recognizing and addressing arterial complications in Behçet's disease for effective management. Continued research and multidisciplinary collaboration are essential to enhance our understanding and refine therapeutic interventions for this complex systemic disorder.

Introduction

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Behçet's disease is a systemic disorder with a wide array of morphological manifestations, primarily impacting the vascular system. While the venous system is commonly affected, arterial involvement exacerbates the severity and morbidity of Behçet's disease. Vasculitis, characterized by occlusion, aneurysms, or a combination of both, presents significant challenges in diagnosis, treatment, and achieving remission. This report details a case of Behçet's disease with a focus on its vascular manifestations.

Case Report

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A 50-year-old patient with Behçet's disease inaugurated at the age of 49 by bipolar aphthosis and revealed by vascular involvement consisting of deep venous thrombosis of the left lower limb and ocular involvement consisting of bilateral panuveitis, for which he received high-dose corticosteroid therapy, azathioprine and oral anticoagulation with good improvement. One year later, the patient presented with deep vein thrombosis of both lower limbs, with loss of consciousness and paraparesis, concomitant with an attack of mouth ulcers. Cerebral-medullary MRI revealed multiple segmental arterial stenoses of inflammatory appearance in the cerebrum, and spondylodiscitis of probably tuberculous origin centred on D12 with vertebral compression in the medulla. in addition to the vascular work-up, a fortuitous discovery of bilateral proximal pulmonary embolism with no impact on the cardiac chambers, no focus of pulmonary infarction, no image of an aneurysm or focus of pleuropulmonary tuberculosis. Therapeutic options were limited by the presence of tuberculous spondylodiscitis. The evolution was very satisfactory, with improvement in motor deficit, loss of consciousness, deep vein thrombosis and pulmonary embolism. The follow-up brain MRI came back without any particularities.

Discussion

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Behçet's disease, characterized by its systemic nature and vascular manifestations, poses intricate challenges in diagnosis and management, particularly when involving the arterial system. The presented case emphasizes the significance of recognizing arterial complications, which intensify the severity and pose unique therapeutic challenges.

Vascular involvement in Behçet's disease often targets the venous system, leading to complications such as cerebral venous thrombosis and dural sinus thrombosis. However, the rarity of cerebral arterial involvement complicates the diagnostic landscape. In this case, we observed manifestations of arterial involvement, including stenosis, intracranial aneurysm, and arterial dissection, highlighting the need for heightened awareness of diverse morphological presentations.

The literature reports sporadic cases of cerebral arterial involvement in Behçet's disease, typically under specific conditions. This emphasizes the importance of considering arterial complications in the diagnostic approach, especially when the disease course deviates from the more commonly observed venous involvement.

The challenges extend beyond diagnosis, as managing arterial complications requires a tailored therapeutic strategy. Treatment decisions should balance the need for immunosuppression to control inflammation with the potential risks, considering the complex nature of arterial involvement in Behçet's disease.

Moreover, the rarity of cerebral arterial manifestations in Behçet's disease calls for more extensive research to elucidate underlying mechanisms, risk factors, and optimal therapeutic approaches. Collaborative efforts between internists, neurologists, and vascular specialists are essential to refine treatment strategies and improve outcomes in cases of Behçet's disease with arterial involvement.

In conclusion, this case report underscores the importance of vigilance in recognizing arterial complications in Behçet's disease. Continued research and multidisciplinary collaboration are imperative to enhance our understanding and refine therapeutic interventions for this complex systemic disorder.

Conclusion

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Vascular involvement in neuro Behçet predominantly manifests as cerebral venous thrombosis and dural sinus thrombosis. Although cerebral arterial involvement is rare, it may present as stenosis, intracranial aneurysm, and arterial dissection. The literature reports isolated cases of cerebral arterial involvement under specific conditions. This case underscores the importance of recognizing and addressing arterial complications in Behçet's disease for effective management.