9. Ruptured Cerebral Aneurysm as a Rare Complication of Behçet's Disease

Abstract

Background: Behcet's disease (BD) is a systemic inflammatory disease of unknown cause. The clinical triad involves apthous stomatitis, genital ulceration and uveitis. Incidence is higher in Mediterranean and Far Eastern countries. Vascular involvement affects 7-29% of patients with BD, which is typically in the venous system in 95% of cases. In comparison, arterial involvement with aneurysm or occlusion is much rarer. Aneurysmal formation usually occurs in large vessels such as the abdominal aorta and pulmonary artery. It rarely occurs in cerebral vessels. Examining the literature shows the paucity of cerebral aneurysms in the context of BD. To date there have been only eighteen reported cases with the majority of these being discovered in the context of Subarachnoid Haemorrhage (SAH). We describe a patient with BD who presented with a ruptured cerebral aneurysm, which responded completely to treatment with steroids. Methods: A 50 year old man was admitted to the neurosurgical unit following sudden onset of severe headache. CT scanning confirmed SAH. He revealed a 20 year history of recurrent oral ulceration, a 6 month history of genital ulceration, recurrent large joint arthritis of the knees and ankles, intermittent abdominal symptoms and severe headaches. A CT angiogram (CTA) revealed two fusiform aneurysms; one arising from the left peri-callosal artery and a second one from a more distant right peri-callosal artery. The appearance of the aneurysms alluded to an inflammatory cause and the diagnosis of Behcet's was suspected. Due to the position of the aneurysms endovascular and surgical treatment was considered too risky. He was treated medically with 3 days of high dose methylprednisolone followed by prednisolone 40mg daily. Results: He was readmitted 1 week later with left arm weakness and was found to have two new cortical infarcts in the territories close to the aneurysms. A CTA at this time indicated shrinking of the aneurysms. A further CTA 3 months later confirmed the disappearance of the aneurysms with no new aneurysms detected. Additional investigations to confirm the diagnosis included a CT TAP, which showed thickening of the aorta. A PET scan showed no evidence of active vasculitis. HLA B51 was negative. He is currently being treated with MTX as a steroid-sparing agent following the advice of rheumatology colleagues at the Hammersmith. Additionally, he required renal artery stenting for left renal artery stenosis. Conclusion: This case highlights the rare complication of cerebral aneurysms in BD and the effectiveness of steroid immunosuppression for the treatment of small, unruptured aneurysms. There have been only two reported cases where aneurysms associated with BD have been treated solely with steroids resulting in resolution. In case of rupture surgical or endovascular treatment may be the first choice.