Engel D G, Gospe S M, Tracy K A, Ellis W G, Lie J T
Department of Neurological Surgery, Northwestern University, Evanston, Ill., USA.
Stroke. 1995 Apr;26(4):699-701. doi: 10.1161/01.str.26.4.699.
Infantile polyarteritis nodosa usually presents in children under 2 years of age as a multiorgan system disease with signs of congestive heart failure or renal failure. This disease and Kawasaki disease may share certain clinical and pathological features.
We describe a child who first presented at 8 months of age with a febrile illness followed by a delay in motor and language development and a mild right hemiparesis. Five years later he died after developing oculomotor dysfunction, hypertension, and intracranial hemorrhage. Autopsy revealed focal segmental necrotizing vasculitis of cerebral arteries, without involvement of coronary or renal vessels.
Although this child was evaluated on several occasions during this time period, the diagnosis was not made antemortem. The predominant central nervous system features, both clinical and pathological, together with the prolonged course are the two unique features of this child's disease that need to be emphasized.
婴儿结节性多动脉炎通常发生于2岁以下儿童，表现为多器官系统疾病，伴有充血性心力衰竭或肾衰竭的体征。这种疾病与川崎病可能具有某些临床和病理特征。
我们描述了一名8个月大时首次因发热性疾病就诊的儿童，随后出现运动和语言发育迟缓以及轻度右侧偏瘫。5年后，他在出现动眼神经功能障碍、高血压和颅内出血后死亡。尸检显示脑动脉局灶性节段性坏死性血管炎，冠状动脉或肾血管未受累。
尽管在此期间对该患儿进行了多次评估，但生前未做出诊断。临床和病理上主要的中枢神经系统特征以及病程延长是该患儿疾病需要强调的两个独特特征。
