Sanghvi Jyoti, Mehta Sudhir, Mulye Swati
Department of Pediatrics, Sri Aurobindo Institute of Medical Sciences, Indore, Madhya Pradesh, India.
J Pediatr Neurosci. 2014 Jan;9(1):39-41. doi: 10.4103/1817-1745.131483.
Sturge-Weber syndrome (SWS) is a rare, sporadically occurring neurocutaneous disorder with a frequency of approximately 1 per 50,000. The hallmark is an intracranial leptomeningeal vascular angioma in association with a port wine nevus, usually involving ophthalmic or maxillary distribution of trigeminal nerve. Other clinical findings associated with SWS are seizures, glaucoma, hemiparesis and mental retardation. The radiological hallmark is "Tram-line" or "Gyri-form" calcification. 25 to 56% of patients experience recurrent episodes of paroxysmal focal neurological deficits in form of transient hemiparesis, which may be due to vascular ischemia or postictal in origin. EEG helps to differentiate the exact etiology, as it is normal in former. Aspirin prophylaxis in those, due to ischemia decreases their recurrences and improves overall neurological prognosis. We report a 25-month-old child of SWS with recurrent episodes of transient hemiparesis and atypical midline location of facial vascular nevus.
斯特奇-韦伯综合征（SWS）是一种罕见的、散发的神经皮肤疾病，发病率约为五万分之一。其特征是颅内软脑膜血管性血管瘤伴葡萄酒色斑痣，通常累及三叉神经的眼支或上颌支分布区域。与SWS相关的其他临床症状包括癫痫、青光眼、偏瘫和智力迟钝。影像学特征是“轨道样”或“脑回样”钙化。25%至56%的患者会反复出现阵发性局灶性神经功能缺损，表现为短暂性偏瘫，其原因可能是血管缺血或发作后所致。脑电图有助于区分确切病因，因为前者脑电图正常。对于因缺血导致的患者，阿司匹林预防可减少其复发并改善整体神经预后。我们报告了一名25个月大的SWS患儿，其反复出现短暂性偏瘫，面部血管痣位于非典型中线位置。
