Treatment of Moyamoya Syndrome Associated with Systemic Lupus Erythematosus and Hypothyroidism in an Adult by Encephaloduroarteriosynangiosis: A Case Report

A 54-year-old woman presented to our hospital with progressive motor weakness of the right arm. She had a medical history of systemic lupus erythematosus (SLE) and hypothyroidism. Magnetic resonance imaging indicated a watershed infarction of the left hemisphere. Cervical echogram indicated severe s...

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Bibliographic Details
Main Authors: Arata Tomiyama, Hitoshi Kimura, Haruo Nakayama, Hideaki Izukura, Jun-ichi Harashina, Keisuke Ito, Ken-ichiro Sato, Morito Hayashi, Norihiko Saito, Takatoshi Sakurai, Yoko Hirata, Kazuya Aoki, Satoshi Iwabuchi
Format: Article
Language:English
Published: Wiley 2012-01-01
Series:Case Reports in Medicine
Online Access:http://dx.doi.org/10.1155/2012/120867
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Summary:A 54-year-old woman presented to our hospital with progressive motor weakness of the right arm. She had a medical history of systemic lupus erythematosus (SLE) and hypothyroidism. Magnetic resonance imaging indicated a watershed infarction of the left hemisphere. Cervical echogram indicated severe stenosis of the internal carotid artery (ICA) without wall thickening. Cerebral angiography indicated left ICA occlusion, development of unilateral moyamoya vessels, and leptomeningeal anastomosis. Encephaloduroarteriosynangiosis (EDAS) was performed after cerebral 99mTechnetium-ethyl-cysteinate-dimer single-photon emission computed tomography indicated a decreased cerebral blood flow, diminished cerebrovascular perfusion reserve. Motor weakness finally disappeared 6 months after surgery. Moyamoya syndrome is a rare complication of both SLE and hypothyroidism, and the surgical indication remains controversial. By evaluating the decreased cerebral perfusion reserve capacity and the existence of leptomeningeal anastomosis, EDAS could be an efficient method for the treatment of moyamoya syndrome associated with SLE and hypothyroidism.
ISSN:1687-9627
1687-9635