J Cerebrovasc Endovasc Neurosurg.  2016 Mar;18(1):32-37. 10.7461/jcen.2016.18.1.32.

Multiple Intracranial Aneurysms Associated with Behçet's Disease

• Ha S
• Kim J
• Kim CG
• Jang SJ

• KMID: 2161700
• DOI: http://doi.org/10.7461/jcen.2016.18.1.32

Abstract

Behçet's disease is an inflammatory disorder involving multiple organs. Its cause is still unknown, but vasculitis is the major pathologic characteristic. The common vascular lesions associated with Behçet's disease are aneurysm formation, arterial or venous occlusive diseases, and varices. Arterial aneurysms mostly occur in large arteries. Intracranial aneurysms hardly occur with Behçet's disease. We would like to present a 41-year-old female patient with Behçet's disease who showed symptoms of severe headache due to subarachnoid hemorrhage. Brain computed tomography revealed multiple aneurysms. We also present a literature review of intracranial arterial aneurysms associated with Behçet's disease.

Keyword

Behçet's disease; Aneurysm

MeSH Terms

Adult
Aneurysm
Arteries
Brain
Female
Headache
Humans
Intracranial Aneurysm*
Subarachnoid Hemorrhage
Varicose Veins
Vasculitis

Figure

• 

  Fig. 1 (A) A CT scan obtained on admission showing a diffuse SAH in the basal cistern. (B) A 3D reconstructed CTA showing multiple aneurysms at both MCAs and ACA (arrow). CT = computed tomography; CTA = CT angiography; SAH = subarachnoid hemorrhage; MCA = middle cerebral artery; ACA = anterior cerebral artery.

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  Fig. 2 (A) A CT obtained on the 2nd day postoperation showinga low-density lesion in Rt. MCA territory. (B) Diffusion-weighted imagerevealingrestricted diffusion in the same area. (C) A Rt. ICA angiogram showing obstruction of Rt. M1 branch (arrow). CT = computer tomography; MCA = middle cerebral artery; ICA = internal carotid artery.

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  Fig. 3 3. (A) A CTA on the 20th day after discharge showing the revascularization of the previously obstructed Rt. M1 branch and the persistent ACA aneurysm (arrow). (B) A CTA performed 3 years after the initial exam showing ACA aneurysm without any changes in size or shape despite continuous steroid therapy. CTA = CT angiography; ACA = anterior cerebral artery.

Reference

1. Aktaş EG, Kaplan M, Ozveren MF. Basilar artery aneurysm associated with Behcet's disesase : a case report. Turk Neurosurg. 2008; 1. 18(1):35–38. PMID: 18382975.

2. Al Dalaan AN, Al Balaa SR, El Ramhi K, Al Kawi Z, Bohlega S, Bohlega S, et al. Behcet disease in Saudi arabia. J Rheumatol. 1994; 4. 21(4):658–661. PMID: 8035390.

3. Benamour S, Zeroual B, Bennis R, Amraoui A, Bettal S. Behcet's disease : 316 cases. Presse Med. 1990; 10. 19(32):1485–1489. PMID: 2146650.

4. Houman MH, Neffati H, Braham A, Harzallah O, Khanfir M, Miled M, et al. Behcet's disease in tunisia. Demographic, clinical and genetic aspects in 260 patients. Clin Exp Rheumatol. 2007; Jul-Aug. 25(No.4 Suppl 45):58–64.

5. International Study Group for Behcet's disease. Criteria for diagnosis of Behcet's disease. Lancet. 1990; 5. 335(8967):1078–1080. PMID: 1970380.

6. Katoh K, Matsunaga K, Ishigatsubo Y, Chiba J, Tani K, Kitamura H, et al. Pathologically defined neuro-, vasculo-, entero-Behcet's disease. J Rheumatol. 1985; 12. 12(6):1186–1190. PMID: 4093928.

7. Kerr JS, Roac E, Sinal S, McWhorter JM. Intracranial arterial aneurysms complicating Behcet's disease. J Child Neurol. 1989; 4. 4(2):147–149. PMID: 2715612.

8. Kingston M, Ratcliffe JR, Altree M, Merendino KA. Aneurysms after arterial puncture in Behcet's disease. Br Med J. 1979; 6. 1(6180):1766–1767. PMID: 466217.

9. Kocak A, Cayli SR, Ates O, Sarac K. Middle cerebral artery aneurysm associated with Behcet's disease-case report. Neurol Med Chir (Tokyo). 2004; 7. 44(7):368–371. PMID: 15347214.

10. Kurdi M, Baeesa S, Bin-Mahfoodh M, Kurdi K. Onyx Embolization of Ruptured Intracranial Aneurysm Associated with Behçet's Disease. Case Rep Vasc Med. 2013; 9. [Epub of ahead print].

11. Nakasu S, Kaneko M, Matsuda M. Cerebral aneurysms associated with Behcet's disease: a case report. J Neurol Neurosurg Psychiatry. 2001; 5. 70(5):682–684. PMID: 11309468.

12. Park JH, Han MC, Bettmann MA. Arterial manifestations of Behcet's disease. AJR Am J Roentgenol. 1984; 10. 143(4):821–825. PMID: 6332492.

13. Rosenstingl S, Dupuy A, Alves O, George B, Tobelem G. Behcet's disease manifested as a intracranial aneurysm. Rev Med Interne. 2001; 2. 22(2):177–182. PMID: 11234676.

14. Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's disease. N Engl J Med. 1999; 10. 341(17):1284–1291. PMID: 10528040.

15. Shimizu T, Ehrlich GE, Inaba G, Hayashi K. Behcet disease (Behcet syndrome). Semin Arthritis Rheum. 1979; 5. 8(4):223–260. PMID: 382361.

16. Tsuji S, Suzuki Y, Tomii M, Matsuoka Y, Kishimoto H, Irimajiri S. Behcet's disease associated with multiple cer ebral aneurysm and downhill esophageal varices caused by superior vena cava obstruction: a case report. Ryumachi. 1990; 10. 30(5):375–381. PMID: 2084860.

17. Zelenski JD, Capraro JA, Holden D, Calabrese LH. Central nervous system vasculitis in Behcet's syndrome: angiographic improvement after therapy with cytotoxic agents. Arthritis Rheum. 1989; 2. 32(2):217–220. PMID: 2920056.