儿童过敏性紫癜合并可逆性后部脑白质病临床分析  

Clinical Analysis of Posterior Reversible Encephalopathy Syndrome in Childhood with Henoch-Sch?nlein Purpura

作　　者：何庭艳[1] 黄瑛[1] 杨军[1] HE Ting-yan HUANG Ying YANG Jun(Department of Rheumatology and Immunology, Shenzhen Children＇s Hospital, Shenzhen 518038, Guangdong Province, China)

机构地区：[1]深圳市儿童医院风湿免疫科,广东深圳518038

出　　处：《罕少疾病杂志》2017年第5期21-23,34,共4页Journal of Rare and Uncommon Diseases

摘　　要：目的探讨儿童过敏性紫癜(HSP)合并可逆性后部脑白质病(RPLS)的临床及影像学特点,提高儿科临床医师对儿童过敏性紫癜合并RPLS的认识。方法回顾性系统分析2例本院及国内外文献报道15例HSP合并RPLS患儿的临床、影像学资料、治疗及预后。结果 17例HSP合并RPLS患儿,女患儿10例,男患儿7例。RPLS发病前12例患儿接受糖皮质激素治疗,2例患儿合并V型紫癜性肾炎,1例患儿合并局灶性坏死性新月体肾小球肾炎,1例患儿出现急性肾功能不全,12例患儿伴有高血压。17例HSP合并RPLS患儿均有癫痫发作,5例出现头晕头痛,9例出现视觉异常,6例出现意识障碍。17例患儿均完善头颅影像学检查,其中MRI主要表现为以大脑后部为主的长T1、长T2信号,大部分呈对称性。17例RPLS患儿经控制惊厥、降颅压、积极控制原发疾病等治疗后,神经系统临床症状及影像学表现均在短期内好转。结论 HSP患儿出现癫痫发作、头痛、视觉障碍或意识障碍提示并发RPLS可能。及时完善头颅MRI检查有助于诊断HSP合并RPLS。早期诊断及积极治疗HSP合并RPLS,大部分患儿预后良好。To investigate clinical and imaging features of reversible posterior leukoencephalopathy syndrome（RPLS） in childhood with Henoch-Sch6nlein purpura and to improve recognition of RPLS secondary to HSP among pediatric clinicians. Methods The clinical, imaging, treatment and prognosis data of 17 cases with RPLS secondary to HSP were retrospectively analyzed. 2 cases were diagnosed in our hospital, and the others were reported by previous literature. Results There were 10 female and 7 male cases in total. 12 cases were accepting therapy of immunosuppressive agents. The main clinical characteristics of RPLS were epileptic attack （17cases）, headache（Bcases）,visual disorder （9cases）, conscious disturbance（6cases）. Most results of magnetic resonance imaging（MRI） showed symmetric long T1 and T2 signal in the occipital lobe. 17 cases were all improved soon after treatment of anti-convulsion, reducing intracranial pressure and controlling primary disease. Conclusion If cases with HSP have any clinical manifestations of seizures, headache, visual disturbances, unconsciousness, RPLS should be considered. Cranial MRI is the most important diagnostic examination for RPLS. Early recognition and recognition treatment of RPLS secondary to HSP may facilitate the prognosis.

关 键 词：过敏性紫癜 可逆性后部白质脑病综合征 儿童 风湿免疫 

分 类 号：R742[医药卫生—神经病学与精神病学]