Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Department of Pediatrics, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
Pediatr Neurol. 2024 Mar;152:11-15. doi: 10.1016/j.pediatrneurol.2023.12.010. Epub 2023 Dec 18.
Area postrema syndrome (APS), a rare childhood condition, manifests as intractable nausea and hiccups. APS has high diagnostic significance in neuromyelitis optica syndrome spectrum disorders (NMOSD) and can be the initial presentation of other critical diseases, including brainstem glioma.
We described two representative cases of unrelated Japanese patients with APS. An etiologic evaluation, including a detailed intracranial neuroradiological examination and autoantibodies assessment, was performed. We also reviewed the literature focusing on the prognosis of pediatric APS symptoms.
A 14-year-old girl with aquaporin-4 antibody-positive NMOSD showed a good prognosis with immunotherapy, whereas another nine-year-old girl with irresectable medullary low-grade glioma had persistent symptoms for more than 10 years. All reported children aged >12 years were diagnosed with NMOSD, and patients aged <13 years showed heterogeneous etiologies.
Distinctive time courses and neuroimaging features were key clinical findings for the diagnostic and therapeutic processes in these patients. This literature review highlights the wide spectrum and prognosis of pediatric-onset APS.
顶盖后区综合征（APS）是一种罕见的儿童疾病，表现为顽固性恶心和呃逆。APS 在视神经脊髓炎谱系疾病（NMOSD）中有很高的诊断意义，也可能是其他严重疾病（包括脑干胶质瘤）的首发表现。
我们描述了两例无关的日本 APS 患者的典型病例。进行了病因评估，包括详细的颅内神经影像学检查和自身抗体评估。我们还回顾了文献，重点关注儿科 APS 症状的预后。
14 岁的水通道蛋白-4 抗体阳性 NMOSD 女孩经免疫治疗后预后良好，而另一位 9 岁的不可切除的髓质低度胶质瘤女孩持续出现症状 10 多年。所有报告的年龄 >12 岁的儿童均被诊断为 NMOSD，年龄 <13 岁的患者病因不同。
不同的病程和神经影像学特征是这些患者诊断和治疗过程中的关键临床发现。本次文献复习强调了儿科 APS 的广泛谱和预后。
