Pacheva Iliyana H, Ivanov Ivan S, Stefanova Krastina, Chepisheva Elena, Chochkova Lyubov, Grozeva Dafina, Stoyanova Angelina, Milenkov Stojan, Stefanova Penka, Petrova Anna
Department of Pediatrics and Medical Genetics, Medical University-Plovdiv, Plovdiv, Bulgaria.
Department of Pediatric Surgery, "St. George" University Hospital, Plovdiv, Bulgaria.
Case Rep Pediatr. 2017;2017:5483543. doi: 10.1155/2017/5483543. Epub 2017 Jan 21.
Central nervous system (CNS) involvement in Henoch-Schonlein purpura (HSP) is rare but poses diagnostic difficulties. The aim of the study was to establish the frequency of CNS involvement in HSP, to analyze its clinical characteristics and do a literature review. Medical files of patients with HSP admitted at the Department of Pediatrics, Plovdiv, were studied retrospectively for a five-year period (2009-2013). Diagnosis was based on the American College of Rheumatology criteria. Out of 112 children with HSP 1 case (0.9%) had CNS involvement presenting as Posterior Reversible Encephalopathy Syndrome (PRES), which may be a result of CNS vasculitis or arterial hypertension. It was an 8-year-old girl with atypical HSP which started with abdominal pain requiring surgery. On the third day after the operation a transient macular rash and arterial hypertension appeared, followed by visual disturbances, hemiconvulsive epileptic seizures, postictal hemiparesis, and confusion. Head CT showed occipital hypodense lesions and MRT-T2 hyperintense lesion in the left occipital lobe. The patient experienced a second similar episode after 2 weeks when palpable purpura had also appeared. Neurological symptoms and MRI resolved completely. HSP can be an etiological factor for PRES in childhood. Although PRES is a rare complication of HSP, clinicians must be aware of it and avoid diagnostic and therapeutic delays.
中枢神经系统（CNS）受累于过敏性紫癜（HSP）较为罕见，但诊断存在困难。本研究旨在确定HSP中枢神经系统受累的频率，分析其临床特征并进行文献综述。对普罗夫迪夫儿科收治的HSP患者的病历进行了为期五年（2009 - 2013年）的回顾性研究。诊断依据美国风湿病学会标准。在112例HSP患儿中，1例（0.9%）出现中枢神经系统受累，表现为后部可逆性脑病综合征（PRES），这可能是中枢神经系统血管炎或动脉高血压的结果。这是一名8岁女童，患有非典型HSP，起病时腹痛需手术治疗。术后第三天出现短暂性斑丘疹和动脉高血压，随后出现视觉障碍、半身惊厥性癫痫发作、发作后偏瘫和意识模糊。头部CT显示枕叶低密度病变，磁共振成像（MRT）-T2加权像显示左侧枕叶高信号病变。2周后患者再次出现类似发作，同时出现可触及的紫癜。神经症状和磁共振成像表现完全消失。HSP可能是儿童PRES的病因。虽然PRES是HSP的罕见并发症，但临床医生必须对此有所认识，避免诊断和治疗延误。
