Objective

To analyze etiologic factors of pediatric acute ataxia and to identify the severity of its underlying causes for urgent medical intervention.

Methods

Clinical data of children diagnosed with acute ataxia between December 2015 and December 2021 from one national medical center were analyzed retrospectively.

Results

A total of 99 children (59 boys, 40 girls), median age at disease onset 55 (range: 12–168) months, were enrolled. The median follow period was 46 (range 6–78) months. Eighty-six (86.9 %) children were diagnosed with immune-associated acute ataxia, among which acute post-infectious cerebellar ataxia (APCA) was the most common diagnosis (50.5 %), followed by demyelinating diseases of the central nervous system (18.2 %) and Guillain-Barré syndrome (9.1 %). On cerebrospinal fluid (CSF) examination, 35/73 (47.9 %) patients had pleocytosis (>5 cells/mm³), and 18/73 (24.7 %) had elevated protein levels. Thirty-one patients (31.3 %) had an abnormal cerebral MRI. Children with other immune-associated acute cerebellar ataxia had more extracerebellar symptoms, intracranial MRI lesions, abnormal CSF results, longer hospital stay, higher recurrence rates and incidence of neurological sequelae than children with APCA.

Conclusion

Immune-associated acute ataxia is the main cause of pediatric acute ataxia, among which APCA is the most common phenotype. However, some immune-associated diseases, especially autoantibody-mediated disease, which has a higher recurrence rate and neurological sequelae account for an increasing proportion of pediatric acute ataxia. When children present with extracerebellar symptoms, abnormal cranial MRI or CSF results, and without prodromal infection, prudent differential diagnosis is recommended.

中文翻译：

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全国单一儿童医疗中心急性共济失调的病因及临床特征

客观的

分析小儿急性共济失调的病因，并确定其根本原因的严重程度，以进行紧急医疗干预。

方法

回顾性分析2015年12月至2021年12月某国家医疗中心诊断为急性共济失调儿童的临床资料。

结果

共有 99 名儿童（59 名男孩，40 名女孩）入组，发病中位年龄为 55 个月（范围：12-168）个月。中位随访期为 46（范围 6-78）个月。86 名（86.9%）儿童被诊断为免疫相关急性共济失调，其中急性感染后小脑共济失调（APCA）是最常见的诊断（50.5%），其次是中枢神经系统脱髓鞘疾病（18.2%） ）和格林-巴利综合征（9.1%）。脑脊液（CSF）检查显示，35/73（47.9%）患者出现细胞增多（>5个细胞/mm ³），18/73（24.7%）患者蛋白质水平升高。31 名患者 (31.3%) 的脑部 MRI 异常。其他免疫相关性急性小脑性共济失调患儿的小脑外症状、颅内 MRI 病变、脑脊液结果异常、住院时间较长、复发率和神经系统后遗症发生率均高于 APCA 患儿。

结论

免疫相关性急性共济失调是小儿急性共济失调的主要原因，其中APCA是最常见的表型。然而，一些免疫相关疾病，特别是自身抗体介导的疾病，其复发率较高，且有神经系统后遗症，导致小儿急性共济失调的比例越来越高。当儿童出现小脑外症状、头颅 MRI 或 CSF 结果异常且无前驱感染时，建议谨慎鉴别诊断。