期刊
JOURNAL OF CHILD NEUROLOGY
卷 28, 期 2, 页码 213-218出版社
SAGE PUBLICATIONS INC
DOI: 10.1177/0883073812463607
关键词
Sturge-Weber syndrome; aspirin; neurocutaneous syndrome
资金
- Brain Vascular Malformation Consortium (BVMC), National Institutes of Health (NIH) Rare Disease Clinical Research Network (RDCRN) [U54NS065705]
- NIH Office of Rare Diseases Research (ORDR) at the National Center for Advancing Translational Science (NCATS)
- National Institute of Neurological Disorders and Stroke (NINDS)
- Hunter's Dream For A Cure
Sturge-Weber syndrome is a neurocutaneous disorder with skin, eye, and brain involvement. Prior series suggest about 50% of patients have seizures/neurodeterioration. Low-dose (3-5 mg/kg/d) aspirin use in this population is controversial. This study further addresses the side effects and outcomes of low-dose aspirin usage in Sturge-Weber syndrome. Fifty-eight subjects on aspirin with brain involvement were analyzed in a retrospective chart review. Charts were evaluated for brain involvement, age at first seizure, and side effects. Subjects' clinical stability was compared using neurologic scores. The majority of subjects had neurologic scores reflecting reasonable seizure control (91%), none or mild hemiparesis (57%), no vision impairment (71%), and none or mild cognitive impairment (80%). Forty-nine reported no significant side effects, and 9 reported either allergic reaction or minimal to significant bleeding on aspirin. This cohort's clinical experience adds significant support for low-dose aspirin use to optimize neurodevelopmental outcome in Sturge-Weber syndrome with minimal side effects.
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