关键词: 硬皮病; 系统性; 脑; 血管炎; 中枢神经系统

Abstract: Objective To describe the clinical,neuroimaging and neuropathological findings in patients withlinear scleroderma “en coup de saber” (LScs) and to explore the role of cerebrovascular changesin the pathogenesis of neurological abnormalities.Methods Two cases of LScs with intracranial involvement were evaluated by clinical,neuroimaging and neuropathology.Results One 30-year-old woman developed Oculomotor nerve palsy, seizure and Hemiparesis.Radiographic studies demonstrated ipsilateral skull atrophy, cerebral atrophy, white matterlesions directly subjacent to the area of scleroderma. Another 27-year-old woman had progressivesevere headache, neuroimaging revealed cyst-like lesion with white matter demyelination inright tempo-occipital lobe and basal ganglia. Both of them had linear scleroderma “en coup desaber” involving the right side of the scalp. Brain biopsy showed obvious thickening and fibrinoiddegeneration of vessel walls, perivascular lymphocytic infiltration, patchy ischemic infarct andwhite matter demyelination.Conclusion The neurological abnormalities associated with linear scleroderma “en coup de saber”were caused by hemodynamic disturbances from intracranial vasculitis.

Key words: Scleroderma; limited; Brain; Vasculitis; central nervous system