Hsu Mei-Hsin, Huang Chao-Ching, Hung Pi-Lien, Huang Hsiu-Mei, Huang Li-Tung, Huang Chao-Cheng, Sheen Jiunn-Ming, Huang Song-Chei, Chang Ying-Chao
Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan.
Institute of Clinical Medicine and Department of Pediatrics, National Cheng Kung University College of Medicine, Tainan, Taiwan.
Brain Dev. 2014 Mar;36(3):248-53. doi: 10.1016/j.braindev.2013.04.009. Epub 2013 May 18.
Paraneoplastic neurological diseases (PND) are rare, but potentially treatable disorders. Paraneoplastic encephalitis is rapidly emerging as an important but likely under-recognized condition in children. The aim of this study was to assess the prevalence and spectrum of PND in children with benign ovary tumor and the long-term outcome.
We retrospectively reviewed the charts of all female patients below 18years of age diagnosed with a benign ovarian tumor proven by pathology between January 1993 and December 2010. All the clinical symptoms developed within 5years of tumor diagnosis and the related investigations were recorded.
There were total 133 children and adolescents with benign ovarian tumors, mostly mature teratoma. Six patients (4.5%) had neuropsychiatric manifestations and all but one were beyond age 10years. The most common neuropsychiatric presentations were depression or low mood (84%), headache (50%), mutism (50%), hypoventilation (50%), seizures (30%), hallucination (30%), vomiting and hypersalivation (30%). Three patients (2.2%) had serious PND including acute disseminated encephalomyelitis in 1 and anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis in 2. Although all of three improved after tumor removal, one without immunotherapy had neurological sequelae and prolonged ICU stay.
The prevalence of PND in benign ovary tumor is not so uncommon in children. It is important to survey ovary tumors in female adolescents with subacute presentation of multiple-level involvement of neuraxis where no clear alternate diagnosis is possible. Treatment of serious PND associated with ovary tumors should include immunotherapy in addition to tumor removal.
副肿瘤性神经系统疾病（PND）较为罕见，但有可能治愈。副肿瘤性脑炎正迅速成为儿童中一种重要但可能未得到充分认识的疾病。本研究的目的是评估患有良性卵巢肿瘤儿童的PND患病率、谱系及长期预后。
我们回顾性分析了1993年1月至2010年12月期间所有18岁以下经病理证实患有良性卵巢肿瘤的女性患者的病历。记录所有在肿瘤诊断后5年内出现的临床症状及相关检查。
共有133例患有良性卵巢肿瘤的儿童和青少年，大多为成熟畸胎瘤。6例患者（4.5%）有神经精神表现，除1例之外均超过10岁。最常见的神经精神表现为抑郁或情绪低落（84%）、头痛（50%）、缄默症（50%）、通气不足（50%）、癫痫发作（30%）、幻觉（30%）、呕吐和流涎过多（30%）。3例患者（2.2%）患有严重PND，其中1例为急性播散性脑脊髓炎，2例为抗N-甲基-D-天冬氨酸受体（NMDAR）脑炎。尽管3例患者在肿瘤切除后均有改善，但1例未接受免疫治疗的患者有神经后遗症且在重症监护病房停留时间延长。
儿童良性卵巢肿瘤中PND的患病率并非不常见。对于有亚急性轴索性多水平受累表现且无明确替代诊断的女性青少年，筛查卵巢肿瘤很重要。与卵巢肿瘤相关的严重PND的治疗除了肿瘤切除外还应包括免疫治疗。
