Comi Anne M
Neurology and Pediatrics, Kennedy Krieger Institute and Johns Hopkins Medicine, 707 N. Broadway, Baltimore, MD 21205, USA.
Curr Opin Neurol. 2006 Apr;19(2):124-8. doi: 10.1097/01.wco.0000218226.27937.57.
Recent neuroimaging, clinical and molecular neuropathologic studies have provided new insights into the neurologic aspects of Sturge-Weber syndrome and are summarized here.
Molecular studies suggest that abnormal brain blood vessel vasoactive and extracellular matrix molecule expression, as well as aberrant brain vascular innervation, contribute to the vascular malformation and its consequences. New magnetic resonance sequences may be useful for the early diagnosis of Sturge-Weber syndrome and perfusion magnetic resonance imaging, single photon emission computed tomography imaging, and positron emission tomography imaging studies are suggesting that decreased brain blood flow combined with altered hemodynamics during prolonged seizures may contribute to the neurologic declines in Sturge-Weber syndrome.
Recent advances in our understanding of the neurologic issues offer promise for preventing brain injury in Sturge-Weber syndrome. More research is needed to translate advances in molecular research and neuroimaging advances into new treatment strategies for the disease.
近期的神经影像学、临床及分子神经病理学研究为斯特奇-韦伯综合征的神经学方面提供了新见解，本文对此进行总结。
分子研究表明，脑血管理化活性和细胞外基质分子表达异常，以及脑血管神经支配异常，均导致了血管畸形及其后果。新的磁共振序列可能有助于斯特奇-韦伯综合征的早期诊断，灌注磁共振成像、单光子发射计算机断层扫描成像和正电子发射断层扫描成像研究表明，长时间癫痫发作期间脑血流量减少与血流动力学改变可能导致斯特奇-韦伯综合征的神经功能衰退。
我们对神经学问题认识的最新进展为预防斯特奇-韦伯综合征的脑损伤带来了希望。需要更多研究将分子研究和神经影像学进展转化为该疾病的新治疗策略。
