Document Detail


Patients with moyamoya disease presenting with movement disorder.
MedLine Citation:
PMID:  21121731     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Moyamoya disease is a rare cerebrovascular disease characterized by idiopathic bilateral stenosis or occlusion of bilateral internal carotid arteries and the development of characteristic leptomeningeal collateral vessels at the base of the brain. Typical presentations include transient ischemic attacks or stroke, and hemorrhage. Presentation with movement disorders is extremely rare, especially in the pediatric population. The authors describe the cases of 4 children with moyamoya disease who presented with movement disorders. Among 446 patients (118 pediatric) with moyamoya disease surgically treated by the senior author, 4 pediatric patients had presented with movement disorders. The clinical records, imaging studies, surgical details, and postoperative clinical and imaging data were retrospectively reviewed. The initial presenting symptom was movement disorder in all 4 patients: chorea in 2, hemiballismus in 1, and involuntary limb shaking in 1. All the patients had watershed infarcts involving the frontal subcortical region on MR imaging. Additionally, 1 patient had a ganglionic infarct. Single-photon emission computed tomography studies showed frontoparietal cortical and subcortical hypoperfusion in all patients. Three patients had bilateral disease, whereas 1 had unilateral disease. All the patients underwent superficial temporal artery-middle cerebral artery bypass. Postoperatively, all 4 patients had complete improvement in their symptoms. The SPECT scans revealed normal perfusion in 3 patients and a small residual perfusion deficit in 1. Movement disorders are a rare presenting feature of moyamoya disease. Hypoperfusion of the frontal cortical and subcortical region was seen in all patients, and the symptomatology was attributed to ischemic dysfunction and imbalance in the cortical-subcortical-ganglionic-thalamic-cortical circuitry. Combined revascularization with superficial temporal artery-middle cerebral artery bypass and encephaloduroarteriosynangiosis leads to excellent results.
Authors:
Paritosh Pandey; Teresa Bell-Stephens; Gary K Steinberg
Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Journal of neurosurgery. Pediatrics     Volume:  6     ISSN:  1933-0715     ISO Abbreviation:  J Neurosurg Pediatr     Publication Date:  2010 Dec 
Date Detail:
Created Date:  2010-12-02     Completed Date:  2011-01-04     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101463759     Medline TA:  J Neurosurg Pediatr     Country:  United States    
Other Details:
Languages:  eng     Pagination:  559-66     Citation Subset:  IM    
Affiliation:
Department of Neurosurgery, Stanford Stroke Center and Stanford Institute for Neuro-Innovation and Translational Neurosciences, Stanford University School of Medicine, Stanford, California, USA.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Cerebral Angiography
Cerebral Revascularization*
Cerebrovascular Circulation
Child
Dyskinesias / diagnosis,  etiology,  surgery
Female
Follow-Up Studies
Humans
Magnetic Resonance Imaging
Male
Movement Disorders / diagnosis,  etiology*,  surgery*
Moyamoya Disease / complications*,  diagnosis,  surgery*
Tomography, Emission-Computed, Single-Photon
Treatment Outcome

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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