Background:

Acute necrotizing encephalopathy (ANE) is a rare condition associated with rapid progression to coma and high incidence of morbidity and mortality.

Methods:

Clinical, electroencephalographic (EEG), and brain magnetic resonance imaging (MRI) characteristics and immunomodulatory therapy timing were retrospectively analyzed in children with ANE. ANE severity scores (ANE-SS) and MRI scores were also assessed. The associations of patient characteristics with 6-month modified Rankin scale (mRS) and length of hospitalization were determined using either univariate linear regression or one-way analysis of variance.

Results:

7 children were retrospectively evaluated. Normal EEG sleep spindles (P = .024) and early treatment (R ² = .57, P = .030) were associated with improved outcomes (ie, decreased mRS). Higher ANE-SS (R ² = .79, P = .011), higher age (R ² = .62, P = .038), and presence of brainstem lesions (P = .015) were associated with longer length of hospitalization. Other patient characteristics were not significantly associated with mRS or length of hospitalization.

Conclusion:

Early immunomodulatory therapy and normal sleep spindles are associated with better functional outcome in children with ANE.

中文翻译：

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儿童急性坏死性脑病后的治疗时机、脑电图、神经影像学和结果

背景：

急性坏死性脑病 (ANE) 是一种罕见的疾病，与快速进展为昏迷和高发病率和死亡率相关。

方法：

回顾性分析了 ANE 儿童的临床、脑电图 (EEG) 和脑磁共振成像 (MRI) 特征以及免疫调节治疗时机。还评估了 ANE 严重程度评分 (ANE-SS) 和 MRI 评分。使用单变量线性回归或单因素方差分析确定患者特征与 6 个月改良 Rankin 量表 (mRS) 和住院时间的关联。

结果：

对7名儿童进行了回顾性评估。正常的 EEG 睡眠纺锤体 ( P = .024) 和早期治疗 ( R ² = .57, P = .030) 与改善结果（即降低 mRS）相关。较高的 ANE-SS ( R ² = .79, P = .011)、较高的年龄 ( R ² = .62, P = .038) 和存在脑干病变 ( P = .015) 与较长的住院时间相关. 其他患者特征与 mRS 或住院时间没有显着相关性。

结论：

早期免疫调节治疗和正常的睡眠纺锤体与 ANE 儿童更好的功能结果相关。