Introduction

The clinico-etiological spectrum of Acute leukoencephalopathy with restricted diffusion (ALERD) is not well known in Indian population. This is likely to vary between populations and ethnicities.

Methods

We retrospectively reviewed the clinicoetiological spectrum of ALERD at a tertiary care pediatric center, and described the clinical, imaging, etiological spectrum and short-term outcomes.

Results

Eleven out of 78 children with non-traumatic encephalopathy presenting to our center had a final diagnosis of ALERD. The mean age at presentation was 34.9 months (6–80 months) and 63.6% were males. The monophasic course (72.7%) and the diffuse pattern (63.6%) on neuroimaging were predominant in these children. Dengue haemorrhagic fever was the commonest underlying/triggering infection (5 of 11 children). Ten children required mechanical ventilation in view of neurogenic respiratory failure, with mean duration of ventilation of 6.4 days (Range 2–10 days). The duration of hospital stay varied from 11 to 25 days (Mean – 15.3 days). One child (9 %) died, 6 children (54.5 %) had varying degrees of cognitive impairment and 4 (36.3 %) children had a normal outcome. Children with a shorter duration of ventilation seemed to have a better outcome.

Conclusion

Dengue haemorrhagic fever was the commonest cause, and diffuse imaging pattern with monophasic course was the commonest presentation in Indian children with ALERD. The clinical presentation and factors influencing outcome are possibly different from previously described literature.

中文翻译：

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弥散受限的急性白质脑病谱 (ALERD)：病例系列和文献复习

介绍

急性弥散性白质脑病 (ALERD) 的临床病因谱在印度人群中尚不清楚。这可能因人口和种族而异。

方法

我们在三级保健儿科中心回顾性审查了 ALERD 的临床病因谱，并描述了临床、影像学、病因谱和短期结果。

结果

在我们中心就诊的 78 名非创伤性脑病儿童中有 11 名最终诊断为 ALERD。就诊时的平均年龄为 34.9 个月（6-80 个月），63.6% 为男性。在这些儿童中，神经影像学的单相病程 (72.7%) 和弥漫性模式 (63.6%) 占主导地位。登革出血热是最常见的潜在/触发感染（11 名儿童中的 5 名）。鉴于神经源性呼吸衰竭，10 名儿童需要机械通气，平均通气时间为 6.4 天（范围 2-10 天）。住院时间从 11 天到 25 天不等（平均 - 15.3 天）。1 名儿童 (9 %) 死亡，6 名儿童 (54.5 %) 有不同程度的认知障碍，4 名 (36.3 %) 儿童结果正常。通气时间较短的儿童似乎有更好的结果。

结论

登革出血热是最常见的病因，单相病程的弥漫性影像学模式是印度 ALERD 儿童最常见的表现。临床表现和影响结果的因素可能与先前描述的文献不同。