Original Paper
Progression of Unilateral Moyamoya Disease: A Clinical SeriesKelly M.E.a · Bell-Stephens T.E.a · Marks M.P.a, b · Do H.M.a, b · Steinberg G.K.aDepartments of aNeurosurgery and bRadiology and Stanford Stroke Center, Stanford University School of Medicine, Stanford, Calif., USA
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Article / Publication Details
Received: November 08, 2005
Accepted: January 18, 2006
Published online: July 14, 2006
Issue release date: July 2006
Number of Print Pages: 7
Number of Figures: 2
Number of Tables: 3
ISSN: 1015-9770 (Print)
eISSN: 1421-9786 (Online)
For additional information: https://www.karger.com/CED
Abstract
Background: The natural history of unilateral moyamoya disease (MMD) in adult patients is not clearly described in the literature. We present a series of 18 patients with unilateral MMD and analyze the risk factors for progression to bilateral disease. Methods: A retrospective review of 157 MMD patients treated at Stanford University Medical Center from 1991 to 2005 identified 28 patients with unilateral MMD (defined as none, equivocal or mild involvement on the contralateral side). Results: Eigh teen patients (5 males and 13 females) were identified with unilateral MMD and angiographic follow-up of ≧5 months. Mean radiologic follow-up (± standard error of the mean) was 19.3 ± 3.4 months and mean clinical follow-up was 24.5 ± 3.7 months. Five patients had childhood onset MMD and 13 patients had adult onset disease. Angiographic progression from unilateral to bilateral disease was seen in 7 patients (38.9%) at a mean follow-up of 12.7 ± 2.4 months. Four of the 7 patients had significant clinical and radiologic progression requiring surgical intervention. Five of 7 patients that progressed had adult onset MMD. The presence of equivocal or mild stenotic changes of the contralateral anterior cerebral artery (ACA), middle cerebral artery (MCA) or internal carotid artery (ICA) was an important predictor of progression (p < 0.01); 6 of 8 patients (75%) with equivocal or mild contralateral disease progressed, whereas only 1 of 10 patients (10.0%) with no initial contralateral disease progressed to bilateral MMD. One patient had mild or equivocal MCA, ICA and ACA stenosis at the time of initial diagnosis and this patient progressed. Conclusions: Contralateral progression in the adult form occurs more commonly than previously reported. The presence of minor changes in the contralateral ACA, intracranial ICA and MCA is an important predictor of increased risk of progression. Patients with a completely normal angiogram on the contralateral side have a very low risk of progression.
© 2006 S. Karger AG, Basel
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Article / Publication Details
Received: November 08, 2005
Accepted: January 18, 2006
Published online: July 14, 2006
Issue release date: July 2006
Number of Print Pages: 7
Number of Figures: 2
Number of Tables: 3
ISSN: 1015-9770 (Print)
eISSN: 1421-9786 (Online)
For additional information: https://www.karger.com/CED
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Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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