J Rheum Dis.  2016 Dec;23(6):386-391. 10.4078/jrd.2016.23.6.386.

Case of Moyamoya Disease Aggravated during the Treatment of Behçet’s Disease

• Park ES
• Oh YJ
• Yoo BW
• Ahn SS
• Park JS
• Yoon PH
• Lee CH

• KMID: 2364693
• DOI: http://doi.org/10.4078/jrd.2016.23.6.386

Abstract

Behçet's disease (BD) is a multi-organ involved inflammatory disorder described by recurrent oral ulcers and other systemic manifestations. Almost all the clinical manifestations of BD are believed to be due to vasculitis. On the other hand, the cerebral arteries are rarely involved. Moyamoya disease (MMD) is an unusual chronic cerebrovascular disorder that is described by bilateral progressive stenosis or occlusion of the internal carotid artery and an abnormal collateral vascular network. A 32-year-old woman with MMD was referred for fever, oral pain, and diarrhea, and was diagnosed with BD. Her MMD was aggravated during treatment with high dose steroids to control the intestinal BD and a reduction in the MMD medication due to gastrointestinal bleeding. This is the first reported case of intestinal BD in a patient previously diagnosed with MMD, who experienced aggravation of her MMD after the cessation of MMD medication due to aggravated intestinal BD.

Keyword

Behcet syndrom; Intestinal Behçet’s disease; Moyamoya disease; Aggravated

MeSH Terms

Adult
Carotid Artery, Internal
Cerebral Arteries
Cerebrovascular Disorders
Constriction, Pathologic
Diarrhea
Female
Fever
Hand
Hemorrhage
Humans
Moyamoya Disease*
Oral Ulcer
Steroids
Vasculitis
Steroids

Figure

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  Figure 1. (A) Initial brain magnetic resonance angiography shows stenosis of bilateral distal internal carotid arteries with well-developed basal and pial collaterals. (B) Initial left internal carotid angiography shows stenosis of the left distal internal carotid artery. Well-developed basal collaterals and pial collaterals to the left hemisphere through the left posterior cerebral artery are also seen. (C) Follow up brain computed tomography shows a newly appearing acute intracerebral hemorrhage in the left thalamus with extension to the ventricles (arrow). (D) On follow up cerebral angiography, aggravated stenosis of the left distal internal carotid artery is seen.

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  Figure 2. (A) Abdominal computed tomography shows mild wall thickening of the terminal ileum (arrow) suggesting terminal ileitis. (B) Colonoscopy shows a large ulcerative lesion at the terminal ileum (arrow).

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  Figure 3. Acute phase reactant level and medication according to the clinical course. ESR: erythrocyte sedimentation rate, CRP: C-reactive protein, MMD: Moyamoya disease, BD: Behçet's disease, Dx: diagnosed. 

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