Graf W D, Milstein J M, Sherry D D
Department of Pediatrics, Children's Hospital and Medical Center, University of Washington, Seattle 98105.
J Child Neurol. 1993 Jul;8(3):256-9. doi: 10.1177/088307389300800309.
We describe the clinical presentation and course of two girls with cerebrovascular disease and mixed connective tissue disease. One developed rapid onset hemiparesis and aphasia secondary to left internal carotid artery occlusion. She experienced a complete recovery after treatment with prednisone and cyclophosphamide. The other patient was diagnosed as having mixed connective tissue disease but had acute neurologic deterioration. She died due to an intracerebral hemorrhage. Autopsy demonstrated small-vessel fibrinoid necrosis. Although cerebrovascular disease secondary to central nervous system vasculitis is a manifestation of systemic lupus erythematosus, this is the first description of cerebrovascular disease as a primary sign in mixed connective tissue disease. These cases demonstrate the range of cerebrovascular disease observed in children with mixed connective tissue disease.
我们描述了两名患有脑血管疾病和混合性结缔组织病的女孩的临床表现及病程。其中一名女孩继发于左颈内动脉闭塞，迅速出现偏瘫和失语。经泼尼松和环磷酰胺治疗后完全康复。另一名患者被诊断为混合性结缔组织病，但出现急性神经功能恶化，因脑出血死亡。尸检显示小血管纤维蛋白样坏死。虽然继发于中枢神经系统血管炎的脑血管疾病是系统性红斑狼疮的一种表现，但这是首次将脑血管疾病作为混合性结缔组织病的主要体征进行描述。这些病例展示了混合性结缔组织病患儿中观察到的脑血管疾病范围。
