An 8-year old boy with continuous spikes and waves during slow sleep presenting with positive onconeuronal antibodies

作者：Hu, Lin-Yan; Shi, Xiu-Yu; Feng, Chen; Wang, Jian-Wen; Yang, Guan; Lammers, Stephen H. T.; Yang, Xiao Fan; Ebrahimi-Fakhari, Darius; Zou, Li-Ping^*

来源：European Journal of Paediatric Neurology, 2015, 19(2): 257-261.

DOI：10.1016/j.ejpn.2014.12.012

• Onconeuronal antibody
• Anti-Ma2
• Anti-CV2/CRMP5
• Neuroblastoma
• CSWS
• ESES
• Steroid therapy

Objective: To determine the etiology of epilepsy with continuous spikes and waves during slow sleep (CSWS)/electrical status epilepticus during sleep (ESES) in an 8-year old boy with a history of neuroblastoma and opsoclonus-myoclonus.
Material & methods: A combination of clinical characterization and follow-up, video EEG and laboratory investigations.
Results: We report the case of an 8-year old boy with a history of neuroblastoma and opsoclonus-myoclonus, who presented with intellectual disability, pharmacotherapy-resistant epilepsy and CSWS/ESES. Although the patient's neuroblastoma had been successfully treated 8 years prior to presentation and an extensive workup did not show a tumor reoccurrence, testing for onconeuronal antibodies was positive for anti-Ma2 and anti-CV2/CRMP5 antibodies. High-dose intravenous methylprednisolone and a taper of oral methylprednisolone were given, leading to a significant clinical improvement. During the taper the patient's condition and EEG manifestations deteriorated again necessitating another cycle of steroid therapy, which lead to a stable improvement. During a 6-month follow-up no CSWS/ESES was seen on EEG and anti-Ma2 and anti-CV2/CRMP5 antibodies remained undetectable.
Conclusion: This case suggests that onconeuronal antibodies may be involved in the pathogenesis of CSWS/ESES.

• 出版日期2015-3
• 单位中国人民解放军总医院