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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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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author 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
author_facet 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
author_sort Arata Tomiyama
collection DOAJ
description 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.
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spelling doaj-art-ca9904d5eb894de7bf7485109afd52d22025-08-20T03:24:06ZengWileyCase Reports in Medicine1687-96271687-96352012-01-01201210.1155/2012/120867120867Treatment of Moyamoya Syndrome Associated with Systemic Lupus Erythematosus and Hypothyroidism in an Adult by Encephaloduroarteriosynangiosis: A Case ReportArata Tomiyama0Hitoshi Kimura1Haruo Nakayama2Hideaki Izukura3Jun-ichi Harashina4Keisuke Ito5Ken-ichiro Sato6Morito Hayashi7Norihiko Saito8Takatoshi Sakurai9Yoko Hirata10Kazuya Aoki11Satoshi Iwabuchi122nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, Japan2nd Department of Neurosurgery, Toho University Ohashi Medical Center, 2-17-6 Ohashi, Meguro-ku, Tokyo 104-0045, JapanA 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.http://dx.doi.org/10.1155/2012/120867
spellingShingle 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
Treatment of Moyamoya Syndrome Associated with Systemic Lupus Erythematosus and Hypothyroidism in an Adult by Encephaloduroarteriosynangiosis: A Case Report
Case Reports in Medicine
title Treatment of Moyamoya Syndrome Associated with Systemic Lupus Erythematosus and Hypothyroidism in an Adult by Encephaloduroarteriosynangiosis: A Case Report
title_full Treatment of Moyamoya Syndrome Associated with Systemic Lupus Erythematosus and Hypothyroidism in an Adult by Encephaloduroarteriosynangiosis: A Case Report
title_fullStr Treatment of Moyamoya Syndrome Associated with Systemic Lupus Erythematosus and Hypothyroidism in an Adult by Encephaloduroarteriosynangiosis: A Case Report
title_full_unstemmed Treatment of Moyamoya Syndrome Associated with Systemic Lupus Erythematosus and Hypothyroidism in an Adult by Encephaloduroarteriosynangiosis: A Case Report
title_short Treatment of Moyamoya Syndrome Associated with Systemic Lupus Erythematosus and Hypothyroidism in an Adult by Encephaloduroarteriosynangiosis: A Case Report
title_sort treatment of moyamoya syndrome associated with systemic lupus erythematosus and hypothyroidism in an adult by encephaloduroarteriosynangiosis a case report
url http://dx.doi.org/10.1155/2012/120867
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