Objectives

To describe neurological characteristics and CNS involvement on MRI in secondary hemophagocytic lymphohistiocytosis (sHLH) and differentiate it from primary hemophagocytic lymphohistiocytosis (pHLH) and acute disseminated encephalomyelitis (ADEM).

Methods

Nine children with sHLH who had neurological symptoms were retrospectively included. Characteristics of brain MRI were compared to those of 15 children with pHLH and neurological involvement and 44 children with ADEM.

Results

Three children (33%) presented with isolated neurological symptoms. Neurological signs occurred within one month following Epstein-Barr virus primary infection or systemic juvenile arthritis exacerbation in 8 patients (89%). Eight children (89%) had MRI lesions. sHLH MRI lesions were distinct of those of pHLH by morphology and signal with more frequent hyposignal intensities on T1-weighted sequences (p = 0.01) and well-defined and less fuzzy lesions (p = 0.03). All patients survived and one patient presented severe motor and cognitive disability.

Conclusion

Neurological symptoms of sHLH are non-specific and their outcome is favorable in most of the children. MRI at onset may help to differentiate this condition from pHLH.

中文翻译：

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儿童继发性噬血细胞淋巴组织细胞增生症的神经系统受累

目标

描述继发性噬血细胞淋巴组织细胞增生症 (sHLH) 的 MRI 神经学特征和 CNS 受累，并将其与原发性噬血细胞淋巴组织细胞增生症 (pHLH) 和急性播散性脑脊髓炎 (ADEM) 区分开来。

方法

9 名有神经系统症状的 sHLH 儿童被回顾性纳入。将脑部 MRI 的特征与 15 名 pHLH 和神经系统受累儿童和 44 名 ADEM 儿童的特征进行比较。

结果

三名儿童 (33%) 出现孤立的神经系统症状。8 名患者 (89%) 在 Epstein-Barr 病毒原发感染或全身性幼年关节炎恶化后 1 个月内出现神经系统体征。8 名儿童 (89%) 有 MRI 病变。sHLH MRI 病变在形态学和信号方面与 pHLH 不同，在 T1 加权序列上出现更频繁的低信号强度（p = 0.01）和清晰且模糊的病变（p = 0.03）。所有患者均存活，一名患者出现严重的运动和认知障碍。

结论

sHLH 的神经系统症状是非特异性的，而且大多数儿童的预后良好。开始时的 MRI 可能有助于将这种情况与 pHLH 区分开来。