Paraneoplastic neurological disorders in children with benign ovarian tumors

• Published in: Brain & development (Tokyo. 1979) 2014-03, Vol.36 (3), p.248-253
• Main Authors: 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
• Format: Article
• Language: English
• Subjects: Adolescent; Brain - pathology; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Magnetic Resonance Imaging; Mental Disorders - diagnosis; Mental Disorders - epidemiology; Neurology; Ovarian Neoplasms - diagnosis; Ovarian Neoplasms - epidemiology; Ovarian Neoplasms - pathology; Ovarian Neoplasms - therapy; Paraneoplastic Syndromes, Nervous System - diagnosis; Paraneoplastic Syndromes, Nervous System - epidemiology; Paraneoplastic Syndromes, Nervous System - pathology; Paraneoplastic Syndromes, Nervous System - therapy; Prevalence; Prognosis; Retrospective Studies; Spinal Cord - pathology; Teratoma - diagnosis; Teratoma - epidemiology; Teratoma - pathology; Teratoma - therapy; Tomography, X-Ray Computed; Treatment Outcome
• ISSN: 0387-7604; 1872-7131
• DOI: 10.1016/j.braindev.2013.04.009

Abstract Aim: 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. Patients and methods: We retrospectively reviewed the charts of all female patients below 18 years of age diagnosed with a benign ovarian tumor proven by pathology between January 1993 and December 2010. All the clinical symptoms developed within 5 years of tumor diagnosis and the related investigations were recorded. Results: 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 10 years. 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. Conclusion: 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.