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Moyamoya disease associated with Behcet's disease
Sung Pil Joo1, Tae Sun Kim, Je Hyuk Lee
1Department of Neurosurgery, Chonnam National University Hospital and Medical School, 8 HakDong, Dong-Gu, Gwangju 510-757, Korea. nsjsp@netian.com
Summary
Behcet's disease can present with moyamoya disease, causing repeated transient ischemic attacks (TIAs). Surgical revascularization effectively treated these symptoms, highlighting the importance of considering moyamoya disease in Behcet's patients.
Area of Science:
- Neurology
- Vascular Surgery
- Rheumatology
Background:
- Behcet's disease is a multisystem inflammatory disorder.
- Cerebrovascular events are a known complication of Behcet's disease.
Observation:
- A 32-year-old woman with Behcet's disease experienced recurrent transient ischemic attacks (TIAs) with left hemiparesis.
- Cerebral angiography confirmed moyamoya disease, characterized by internal carotid artery occlusion and abnormal collateral vessels.
Findings:
- The patient underwent superficial temporal artery to middle cerebral artery (STA-MCA) anastomosis and encephalomyosynangiosis.
- Post-surgical intervention, acetazolamide single-photon emission computed tomography (SPECT) indicated improved cerebral reserve capacity.
- The patient's TIA symptoms resolved after surgery.
Implications:
- This case represents the first documented association between moyamoya disease and Behcet's disease.
- Moyamoya disease should be included in the differential diagnosis for cerebrovascular events in patients with Behcet's disease.
- Revascularization surgery is a recommended preventative strategy for ischemic insults in such cases.
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