儿童急性播散性脑脊髓炎临床特征及复发因素分析

Clinical features of children with acute disseminated encephalomyelitis and related recurrence factors

摘要:

目的 探讨儿童急性播散性脑脊髓炎（ADEM）的临床特征及复发因素。方法 回顾性分析2011年11月至2017年1月住院的73例ADEM患儿的临床资料及转归情况。结果 73例患儿中，发病前41例（56%）有感染病史，7例（10%）有疫苗接种史。所有患儿均具有脑病表现，其中意识改变47例（64%），精神行为异常54例（74%）；余以发热（53例，73%）、运动障碍（47例，64%）、头痛（47例，64%）、呕吐（40例，55%）等多见。头颅MRI检查以皮层下白质（83%，54/65）、深部核团（60%，39/65）、脑干（58%，38/65）、小脑（42%，27/65）受累多见。47%（20/43）患儿脊髓受累。随访发现共15例患儿复发。与未复发患儿比较，复发患儿更多出现深部核团受累（P < 0.05），≥ 3个脊髓节段损伤者更多见（P < 0.01），丙种球蛋白和/或激素治疗时间距起病>2周的比例更高（P < 0.05）。结论 ADEM临床表现多样，少数患儿可复发。MRI深部核团受累、≥ 3个节段的长节段脊髓病变、丙种球蛋白和/或激素治疗时间较晚（>2周）可能与ADEM复发相关。

Abstract:

Objective To study the clinical features of children with acute disseminated encephalomyelitis (ADEM) and related recurrence factors. Methods A retrospective analysis was performed for the clinical data and prognosis of 73 children with ADEM who were hospitalized from November 2011 to January 2017. Results Among the 73 children, 41 (56%) had a history of infection before onset and 7 (10%) had a history of vaccination. All children had the symptoms of encephalopathy, including disturbance of consciousness in 47 children (64%) and mental and behavioral disorders in 54 children (74%). Pyrexia was observed in 53 children (73%), dyskinesia in 47 children (64%), headache in 47 children (64%) and vomiting in 40 children (55%). Brain MRI was performed for 65 children and the results showed involvement of the subcortical white matter (83%, 54/65), the deep nuclei (60%, 39/65), the brain stem (58%, 38/65) and the cerebellum (42%, 27/65). Spinal cord involvement was observed in 20 children (20/43, 47%). A total of 15 children experienced recurrence during follow-up. Compared with the non-recurrence group, the recurrence group had signifcantly higher percentages of children with deep nucleus involvement (P < 0.05), with injury in ≥ 3 spinal segments (P < 0.01) and with a time from disease onset to gamma-globulin/hormone treatment of >2 weeks (P < 0.05). Conclusions ADEM in children have various clinical manifestations. A small number of children may experience recurrence. Deep nucleus involvement on MRI, long spinal segmental injury (≥ 3 segments) and late treatment with gamma-globulin/hormone (>2 weeks) may be associated with the recurrence of ADEM.