视神经脊髓炎
医学
病因学
儿科
后脑区
神经影像学
胶质瘤
病理
内科学
多发性硬化
精神科
中枢神经系统
癌症研究
作者
Yuriko Tomari,Yu Igata,Pin Fee Chong,Kenta Kajiwara,Eriko Hatai,Yuri Sonoda,Utako Oba,Noriyuki Kaku,Yuhki Koga,Yasunari Sakai,Shouichi Ohga
标识
DOI:10.1016/j.pediatrneurol.2023.12.010
摘要
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.
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