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

• Verfasst von: Hu, Lin-Yan [VerfasserIn]
• Verfasst von: Ebrahimi-Fakhari, Darius [VerfasserIn]
• Titel: An 8-year old boy with continuous spikes and waves during slow sleep presenting with positive onconeuronal antibodies
• Verf.angabe: Lin-Yan Hu, Xiu-Yu Shi, Chen Feng, Jian-Wen Wang, Guan Yang, Stephen H.T. Lammers, Xiao Fan Yang, Darius Ebrahimi-Fakhari, Li-Ping Zou
• Jahr: 2015
• Jahr des Originals: 2014
• Umfang: 5 S.
• Fussnoten: Available online 23 December 2014 ; Gesehen am 02.06.2020
• Titel Quelle: Enthalten in: European journal of paediatric neurology
• Ort Quelle: Burlington, Mass. : Harcourt, 1997
• Jahr Quelle: 2015
• Band/Heft Quelle: 19(2015), 2, Seite 257-261
• ISSN Quelle: 1532-2130
• DOI: doi:10.1016/j.ejpn.2014.12.012
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: Anti-CV2/CRMP5
• Sach-SW: Anti-Ma2
• Sach-SW: CSWS
• Sach-SW: ESES
• Sach-SW: Neuroblastoma
• Sach-SW: Onconeuronal antibody
• Sach-SW: Steroid therapy
• K10plus-PPN: 1699088756

Abstract

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.