Eun Seong Park; Yoon-Jeong Oh; Byung-Woo Yoo; Sung Soo Ahn; Jin Su Park; Pyeong Ho Yoon; Chan Hee Lee

doi:10.4078/jrd.2016.23.6.386

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Park, Oh, Yoo, Ahn, Park, Yoon, and Lee: Case of Moyamoya Disease Aggravated during the Treatment of Behçet's Disease

Case Report

J Rheum Dis 2016;23(6):386-391.

Published online: 31 October 2016

DOI: https://doi.org/10.4078/jrd.2016.23.6.386

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

Eun Seong Park¹, Yoon-Jeong Oh¹, Byung-Woo Yoo¹, Sung Soo Ahn¹, Jin Su Park², Pyeong Ho Yoon³, Chan Hee Lee²

¹Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea

²Department of Internal Medicine, National Health Insurance Service Ilsan Hospital, Goyang, Korea

³Department of Radiology, National Health Insurance Service Ilsan Hospital, Goyang, Korea

Corresponding to: Chan Hee Lee, Division of Rheumatology, Department of Internal Medicine, National Health Insurance Service Ilsan Hospital, 100, Ilsan-ro, Ilsandong-gu, Goyang 10444, Korea. E-mail: chanheell@daum.net

Received 6 July 2016 Revised 25 July 2016 Revised 27 July 2016 Accepted 29 July 2016

This is a Free Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Keywords: Behcet syndrom, Intestinal Behçet's disease, Moyamoya disease, Aggravated

REFERENCES

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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.

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).

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.

Table 1.

Clinical characteristics of Moyamoya disease (MMD)/Behçet's disease (BD) coexistence cases

Variable	Park et al. [4]	Joo et al. [5]	Degirmenci et al. [6]	This patient
Country	Korea	Korea	Turkey	Korea
Sex	Female	Female	Female	Female
Dx age of BD/MMD (yr)	27/32	32/36	28/28	32/27
HLA-B51	Positive	Not mentioned	Positive	Negative
Other medical history	None	Tuberculosis	none	None
Clinical manifestation of BD	OU, GU, EN	OU, GU, EN, arthritis, uveitis	OU, GU, EN	OU, GU, uveitis, GI
Onset symptom of MMD	Rt. hemiparesis, dizziness	TIA, dizziness	Involuntary movement of Rt. arm, leg	Rt. arm weakness, Lt. hemiparesis, TIA
ICA involvement	Bilateral	Bilateral	Bilateral	Bilateral
Treatment of MMD	Surgery	Surgery	Medical treatment	Medical treatment
Prognosis	Well controlled MMD and BD	Well controlled MMD and BD	Well controlled MMD and BD	Aggravated MMS after BD Dx

Dx: diagnosis, HLA: human leukocyte antigen, ICA: internal carotid artery, OU: oral ulcer, GU: genital ulcer, EN: erythema nodosum, GI: gastrointestinal, Rt.: right, TIA: transient ischemic attack, Lt.: left.

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