 |
Case Report
Vertebral artery dissection associated with Behçet’s disease in elderly female
1 MD, Director, Department of Neurosurgery, Hokuto Hospital, Obihiro, Hokkaido, Japan
Address correspondence to:
Akira Tempaku
7-5, Inada-cho-kisen, Obihiro, Hokkaido 080-0833,
Japan
Message to Corresponding Author
Article ID: 100084Z09AT2026
Access full text article on other devices
Access PDF of article on other devices
How to cite this article
Tempaku A. Vertebral artery dissection associated with Behçet’s disease in elderly female. J Case Rep Images Med 2026;12(1):1–4.ABSTRACT
Introduction: Behçet’s disease is known as an autoimmune disorder that is characterized by systemic inflammation. The disorder predominantly affects middle-aged individuals and is traditionally considered to be more prevalent in men. However, several reports point that number and ration of female patient have increased in recent years. In addition, several patient cases with significant disability due to vascular complications, such as thrombosis and aneurysms with stroke, have also been documented.
Case Report: An 87-year-old female patient with a medical history of Behçet’s disease presented with headaches as her primary complaint. No abnormalities were identified in the central nervous system. Blood pressure was also in normal range. However, subsequent head magnetic resonance imaging studies revealed a right vertebral artery (VA) dissection, which was considered the underlying cause of the headache. The patient was subjected to conservative management, with a satisfactory recovery ensuing without complications, such as hemorrhagic or ischemic stroke.
Conclusion: Vertebral artery dissection is most prevalent among middle-aged individuals; however, this case is atypical in that it occurred at an older age than is typically observed for the onset of the condition. Moreover, as a vascular lesion associated with Behçet’s disease, it was a rare occurrence because it manifested in the VA. Intracranial VA dissection is uncommon, which had been previously documented in few cases among middle-aged males with Behçet’s disease.
Keywords: Behçet’s disease, Dissected aneurysm, Elderly female, Intracranial vertebral artery
Introduction
Behçet’s disease is categorized as a systemic inflammatory disorder, which is typified by the presence of oral ulcers, genital ulcers, skin rashes, and ocular symptoms [1],[2]. The disease predominantly affects individuals between the ages of 20 and 40, with males demonstrating a higher propensity to exhibit more severe manifestations [3]. While cases in men have historically been more prevalent, there has been an increasing recognition of cases in women in recent years [3]. The prevalence of the Behçet’s disease patient has been documented mainly in East Asia, Middle East Asia, and Mediterranean countries [3],[4]. Patients with Behçet’s usually exhibit either Human Leukocyte Antigen (HLA)-B51 or HLA-A26 antigens [5],[6],[7]. Furthermore, immune-related molecule coding genes, including the interleukin (IL)-23 receptor, IL-12 receptor β2 chain, IL-10, and endoplasmic reticulum aminopeptidase (ERAP)1, have been characterized as susceptibility genes for Behçet’s disease [8],[9],[10],[11]. These factors are considered to contribute to regional and ethnic disparities in disease prevalence.
Although not prevalent, cases accompanied by gastrointestinal ulcers [12] or neurological symptoms, such as meningitis and encephalitis [13],[14],[15], tend to manifest with severe symptoms. Furthermore, vascular lesions [16],[17] involving thrombosis in major arteries or veins, and aneurysm formation with rupture, are reported to be more prevalent in males. Contrary to common knowledge, vertebral artery dissection, causing to aneurysm formation, was observed in an elderly female patient outside the typical age range for this condition. While vascular Behçet’s disease has been predominantly reported in males, the present case involved a female patient. In addition, based on a comprehensive review of the extant literature, it is believed that this constitutes the first documented instance of intracranial VA dissected aneurysm in female.
Case Report
An 87-year-old woman presented with symptoms including persistent headache and posterior neck pain. No instances of consciousness impairment, hemiplegia, sensory disturbances, or ataxia were observed. Her general condition remained stable with blood pressure was 133/72 mmHg, and heart rate was 69/min. The patient had been undergoing medical treatment to Behçet’s disease. A detailed analysis of the head and neck region was conducted using magnetic resonance imaging (MRI), which revealed a right VA dissection of the V4 segment. No acute hemorrhagic or ischemic structural abnormalities, neoplastic lesions, or white matter lesions were identified. The MRI scan revealed an acute phase mural thrombus with high T1-weighted image signal and high Black Blood signal (Figure 1). As demonstrated by the patient’s symptoms, no substantial deterioration was observed at the time of the visit, which occurred on the fourth day following the onset of symptoms. Her ethnicity is Japanese. She had no family history of Behçet’s disease among her known relations. Consequently, the therapeutic approach was chiefly oriented toward the regulation of blood pressure, and the patient was observed as an outpatient. In the ensuing clinical period, no adverse events have been observed.
Discussion
Vertebral artery (VA) dissection predominantly affects middle-aged individuals [18],[19]. Causes of VA dissection include hypertension and excessive mechanical stress by trauma to the neck [20]. This case involved an elderly patient, representing a rare age group for onset. Furthermore, no precipitating factors such as hypertension or excessive neck stress were identified.
Vascular involvement disorder is reported in 7–29% of patients with Behçet’s disease. The common vascular lesions associated with Behçet’s disease are arterial or venous occlusive diseases and aneurysm formation [21]. Aneurysms mainly develop in the major vessels including the abdominal aorta, femoral and pulmonary arteries. Although extracranial aneurysm is well known, intracranial aneurysm formation with Behçet’s disease is rare. However, this case demonstrated dissection with aneurysm formation in the intracranial VA. The structural composition of the vascular wall in head and neck vessels is characterized by a tripartite layering. Within the intracranial area, the VA exhibits a three-layer structure: intima, media, and adventitia, with an internal elastic lamina separating the intima and media [22]. The pathogenic features of Behçet’s disease are the inflammation of the vascular endothelium [23],[24],[25]. The inflammatory reactions lead to the thrombosis due to hypercoagulability [24],[25] and the enlargement of dissection cavities within the vessel wall, resulting in aneurysm formation [21],[26].
Intracranial aneurysms in Behçet’s disease were reported in the several papers including case reports [26],[27],[28],[29]. The present studies found that all patients with an intracranial VA dissected aneurysm were male. Furthermore, all patients are under 70 years of age. Inflammatory angiitis-related VA aneurysm has only been reported in one case, in a 67-year-old female patient [30]. That particular case has been linked to a condition known as eosinophilic granulomatous with polyangiitis, which is a type of disease that affects the immune system. In that report, Hayashi described two cases of fusiform aneurysm in VA among female patients as part of a literature review. However, the other cases were in 30-year-olds [30]. The present case is distinctive in that it was observed in a patient over the age of 80. Moreover, the present study documents, for the first time, the occurrence of an intracranial VA aneurysm associated with Behçet’s disease in a female patient. To prevent the progression of vessel wall dissection and rupture of aneurysm, blood pressure was controlled under 140 mmHg in systolic phase with calcium channel blocker administration. Because of clinical guidelines against Behçet’s disease never mention to handle with the dissected intracranial vessels, the patient was treated following to the manner of dissected intracranial aneurysm by hypertension.
In this case, it was hypothesized that inflammation of the vascular endothelium due to Behçet’s disease contributed to the formation of a dissection cavity in the intima and media. This case of vertebral artery dissection was atypical in nature, necessitating an examination that considered underlying disorders such as vasculitis or collagen diseases, in addition to hypertension.
This case includes several rare clinical features. Based on the basic pathologies of Behçet’s disease, constructing the clinical treatment protocol would be needed. Furthermore, clinical evidence accumulation and characteristic analysis would remain required.
Conclusion
A case of VA dissection in an elderly woman without hypertension was observed in the vascular complications of Behçet’s disease. This finding indicates that VA dissection can also occur as a consequence of inflammatory diseases. Preventing the dissected aneurysm rupture, blood pressure control under 140 mmHg by calcium channel blocker might be useful. There are no obvious clinical guideline of Behçet’s disease with the dissected intracranial vessels. Further case studies would be needed to construct the treatment chart.
REFERENCES
1.
Criteria for diagnosis of Behçet’s disease. International Study Group for Behçet’s Disease. Lancet 1990;335(8697):1078–80.
[Pubmed]
2.
Lavalle S, Caruso S, Foti R, Gagliano C, Cocuzza S, La Via L, et al. Behçet’s disease, pathogenesis, clinical features, and treatment approaches: A comprehensive review. Medicina (Kaunas) 2024;60(4):562. [CrossRef]
[Pubmed]
3.
Al-Otaibi LM, Porter SR, Poate TW. Behçet’s disease: A review. J Dent Res 2005;84(3):209–22. [CrossRef]
[Pubmed]
4.
Verity DH, Marr JE, Ohno S, Wallace GR, Stanford MR. Behçet’s disease, the Silk Road and HLA-B51: Historical and geographical perspectives. Tissue Antigens 1999;54(3):213–20. [CrossRef]
[Pubmed]
5.
Bodis G, Toth V, Schwarting A. Role of human leukocyte antigens (HLA) in autoimmune diseases. Rheumatol Ther 2018;5(1):5–20. [CrossRef]
[Pubmed]
6.
Capittini C, Rebuffi C, Lenti MV, Di Sabatino A, Tinelli C, Martinetti M, et al. Global meta-analysis on the association between Behcet syndrome and polymorphisms from the HLA Class I (A, B, and C) and Class II (DRB1, DQB1, and DPB1) genes. Dis Markers 2021;2021:9348697. [CrossRef]
[Pubmed]
7.
Nakajima T, Tsuji H, Inaba R, Saito R, Nishimura K, Morinobu A. Significant association of HLA-A26 with uveitis and gastrointestinal involvement in patients with Behçet’s disease in a multicentre study. Mod Rheumatol 2024;34(6):1221–5. [CrossRef]
[Pubmed]
8.
Aridogan BC, Yildirim M, Baysal V, Inaloz HS, Baz K, Kaya S. Serum levels of IL-4, IL-10, IL-12, IL-13 and IFN-gamma in Behçet’s disease. J Dermatol 2003;30(8):602–7. [CrossRef]
[Pubmed]
9.
Takeuchi M, Ombrello MJ, Kirino Y, Erer B, Tugal-Tutkun I, Seyahi E, et al. A single endoplasmic reticulum aminopeptidase-1 protein allotype is a strong risk factor for Behçet’s disease in HLA-B*51 carriers. Ann Rheum Dis 2016;75(12):2208–11. [CrossRef]
[Pubmed]
10.
Guasp P, Barnea E, González-Escribano MF, Jiménez-Reinoso A, Regueiro JR, Admon A, et al. The Behçet’s disease-associated variant of the aminopeptidase ERAP1 shapes a low-affinity HLA-B*51 peptidome by differential subpeptidome processing. J Biol Chem 2017;292(23):9680–9. [CrossRef]
[Pubmed]
11.
Sonmez C, Yucel AA, Yesil TH, Kucuk H, Sezgin B, Mercan R, et al. Correlation between IL-17A/F, IL-23, IL-35 and IL-12/-23 (p40) levels in peripheral blood lymphocyte cultures and disease activity in Behcet’s patients. Clin Rheumatol 2018;37(10):2797–804. [CrossRef]
[Pubmed]
12.
Skef W, Hamilton MJ, Arayssi T. Gastrointestinal Behçet’s disease: A review. World J Gastroenterol 2015;21(13):3801–12. [CrossRef]
[Pubmed]
13.
Caruso P, Moretti R. Focus on neuro-Behçet’s disease: A review. Neurol India 2018;66(6):1619–28. [CrossRef]
[Pubmed]
14.
Gomez de la Torre R, Consuelo Charca-Benavente L, Caminal-Montero L. Clinical features of neuro-Behçet. Med Clin (Barc) 2021;157(1):45. [CrossRef]
[Pubmed]
15.
Zhan H, Cheng L, Li Y. Neuro-Behçet’s disease: An update of clinical diagnosis, biomarkers, and immunopathogenesis. Clin Exp Immunol 2025;219(1):uxae123. [CrossRef]
[Pubmed]
16.
Emmi G, Bettiol A, Silvestri E, Di Scala G, Becatti M, Fiorillo C, et al. Vascular Behçet’s syndrome: An update. Intern Emerg Med 2019;14(5):645–52. [CrossRef]
[Pubmed]
17.
Torgutalp M, Sahin Eroglu D, Sezer S, Yayla ME, Karatas G, Uslu Yurteri E, et al. Analysis of vascular involvement in 460 patients with Behçet’s syndrome: Clinical characteristics and associated factors. Joint Bone Spine 2022;89(2):105277. [CrossRef]
[Pubmed]
18.
Yoshimoto Y, Wakai S. Unruptured intracranial vertebral artery dissection. Clinical course and serial radiographic imagings. Stroke 1997;28(2):370–4. [CrossRef]
[Pubmed]
19.
Gottesman RF, Sharma P, Robinson KA, Arnan M, Tsui M, Ladha K, et al. Clinical characteristics of symptomatic vertebral artery dissection: A systematic review. Neurologist 2012;18(5):245–54. [CrossRef]
[Pubmed]
20.
Zhang X, Han J, Wang J, Yu S. A comparative study of the etiology of intracranial vertebral artery dissection and carotid artery dissection. Neurologist 2023;28(5):281–6. [CrossRef]
[Pubmed]
21.
Park JH, Han MC, Bettmann MA. Arterial manifestations of Behçet disease. AJR Am J Roentgenol 1984;143(4):821–5. [CrossRef]
[Pubmed]
22.
Amran MY, Hawari I, La’biran FJ, Gunadi SGA, Muslich LT. Vertebral artery dissection from etiopathogenesis to management therapy: A narrative review with neuroimaging’s case illustration. Egypt J Neurol Psychiatry Neurosurg 2024;60:118. [CrossRef]
23.
Carmeliet P, Collen D. Molecular genetics of the fibrinolytic and coagulation systems in haemostasis, thrombogenesis, restenosis and atherosclerosis. Curr Opin Lipidol 1997;8(2):118–25. [CrossRef]
[Pubmed]
24.
Fernández-Bello I, López-Longo FJ, Arias-Salgado EG, Jiménez-Yuste V, Butta NV. Behçet’s disease: New insight into the relationship between procoagulant state, endothelial activation/damage and disease activity. Orphanet J Rare Dis 2013;8:81. [CrossRef]
[Pubmed]
25.
Butta NV, Fernández-Bello I, López-Longo FJ, Jiménez-Yuste V. Endothelial dysfunction and altered coagulation as mediators of thromboembolism in Behçet disease. Semin Thromb Hemost 2015;41(6):621–8. [CrossRef]
[Pubmed]
26.
Ha S, Kim J, Kim CG, Jang SJ. Multiple intracranial aneurysms associated with Behçet’s disease. J Cerebrovasc Endovasc Neurosurg 2016;18(1):32–7. [CrossRef]
[Pubmed]
27.
Ishikawa H, Shindo A, Ii Y, Sakano S, Asahi M, Matsuura K, et al. Vertebral artery dissection associated with familial Mediterranean fever and Behçet’s disease. Ann Clin Transl Neurol 2019;6(5):974–8. [CrossRef]
[Pubmed]
28.
Jalali KS, Alhazzaa MA, Alqahtani S, Alattas MY. Pontine infarction and vertebral artery dissecting aneurysm as the first presentation of Behçet’s disease: A case report. Cureus 2022;14(9):e29204. [CrossRef]
[Pubmed]
29.
Karki M, Rajpal G. Endovascular treatment for thrombosed giant fusiform vertebral artery aneurysm: A rare case report. Asian J Neurosurg 2025;20(2):397–400. [CrossRef]
[Pubmed]
30.
Hayashi T, Sakata H, Ezura M, Saito A, Osada Y, Tominaga T. Flow diverter therapy for immunosuppressant-resistant vertebral artery fusiform aneurysm due to eosinophilic granulomatosis with polyangiitis: Illustrative case. J Neurosurg Case Lessons 2022;3(11):CASE21701. [CrossRef]
[Pubmed]
SUPPORTING INFORMATION
Acknowledgments
The author thanks medical staff of Hokuto Hospital to support clinical care for the patient.
Author ContributionsAkira Tempaku - Conception of the work, Design of the work, Acquisition of data, Analysis of data, Drafting the work, Revising the work critically for important intellectual content, Final approval of the version to be published, Agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.
Guaranter of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthor declares no conflict of interest.
Copyright© 2026 Akira Tempaku. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
