Chung M H, Sum J, Morrell M J, Horoupian D S
Division of Neuropathology, Stanford Comprehensive Epilepsy Center, Stanford University Medical Center, CA 94305-5324, USA.
Ann Neurol. 1995 May;37(5):679-81. doi: 10.1002/ana.410370519.
Linear scleroderma en coup de sabre (LScs) is a rare disorder not infrequently associated with neurologic symptoms, notably epilepsy. However, histopathologic documentation of intracerebral lesions in LScs is very limited and the etiology of the central nervous system symptoms has therefore never been convincingly established. We describe a 27-year-old woman with LScs and a longstanding history of epilepsy. Radiographic studies demonstrated a focal, intraparenchymal lesion in the left frontal lobe directly subjacent to the area of scleroderma on the forehead and scalp. The resected cerebral lesion revealed localized band-like sclerosis of the leptomeninges and associated vessels, as well as intraparenchymal calcifications and anomalous, ectatic vessels. These findings suggest that LScs may represent a neurocutaneous syndrome of vascular dysplasia similar to the Sturge-Weber syndrome, rather than a localized form of collagen vascular disease, as suggested by some.
线性硬皮病（线状硬皮病）是一种罕见疾病，常伴有神经症状，尤其是癫痫。然而，线性硬皮病脑内病变的组织病理学记录非常有限，因此中枢神经系统症状的病因从未得到令人信服的确立。我们描述了一名患有线性硬皮病且有长期癫痫病史的27岁女性。影像学研究显示左额叶有一个局灶性脑实质内病变，直接位于前额和头皮硬皮病区域下方。切除的脑病变显示软脑膜和相关血管有局部带状硬化，以及脑实质内钙化和异常扩张的血管。这些发现表明，线性硬皮病可能代表一种类似于斯-韦综合征的血管发育异常的神经皮肤综合征，而不是如一些人所认为的局限性胶原血管病。
