Document Detail


Clinical characteristics and long-term outcomes of moyamoya syndrome associated with neurofibromatosis type 1.
MedLine Citation:
PMID:  25443089     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Moyamoya syndrome (MMS) associated with neurofibromatosis type 1 (NF1) has rarely been reported anywhere in the world, particularly in Asia. Because of the rarity of this disorder, its natural history, clinical symptoms, management, and follow-up findings remain unclear. The objective of this study was to evaluate the clinical presentation, neurological imaging, and long-term outcomes of patients with this disease by reviewing Chinese patients with MMS associated with NF1. A retrospective review was conducted from the moyamoya disease (MMD) and MMS patient database of our hospital. Six patients who were diagnosed with MMS associated with NF1 between January 2003 and October 2013 were identified. The clinical symptoms were transient ischemic attack (TIA, three patients), headache (one patient), intracerebral hemorrhage (one patient), and cerebral infarction (one patient). The mean age of diagnosis for NF1 and MMS was 2.7±2.1years (range, 1-6years) and 11.4±8.3years (range, 3.5-23years), respectively. Five of six patients (nine hemispheres) underwent revascularization surgery, and their clinical symptoms were stable during a 46.3±36.1month (range, 18-108month) follow-up. One non-surgical patient had a new infarct that resulted in visual field deficits during follow-up. Three patients had radiographic follow-up, and the postoperative angiograms showed successful revascularizations in the operated hemispheres. To conclude, the clinical and radiographic features for MMS-NF1 are similar to those of typical MMD. Routine vascular screening for NF1 patients is necessary for the early identification of MMS and other cerebral arteriopathies. Revascularization surgery may prevent the progression of clinical symptoms and reduce the risk of subsequent strokes.
Authors:
Cong Han; Wei-Zhong Yang; Hong-Tao Zhang; Ting Ye; Lian Duan
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-10-28
Journal Detail:
Title:  Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia     Volume:  -     ISSN:  1532-2653     ISO Abbreviation:  J Clin Neurosci     Publication Date:  2014 Oct 
Date Detail:
Created Date:  2014-12-2     Completed Date:  -     Revised Date:  2014-12-3    
Medline Journal Info:
Nlm Unique ID:  9433352     Medline TA:  J Clin Neurosci     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2014. Published by Elsevier Ltd.
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