An 8-year old boy with continuous spikes and waves during slow sleep presenting with positive onconeuronal antibodies
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 characteri...
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| Main Authors: | , |
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| Format: | Article (Journal) |
| Language: | English |
| Published: |
2015
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| In: |
European journal of paediatric neurology
Year: 2014, Volume: 19, Issue: 2, Pages: 257-261 |
| ISSN: | 1532-2130 |
| DOI: | 10.1016/j.ejpn.2014.12.012 |
| Online Access: | Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.ejpn.2014.12.012 Verlag, lizenzpflichtig, Volltext: http://www.sciencedirect.com/science/article/pii/S109037981400213X 
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| Author Notes: | 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 |
| Summary: | 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. |
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| Item Description: | Available online 23 December 2014 Gesehen am 02.06.2020 |
| Physical Description: | Online Resource |
| ISSN: | 1532-2130 |
| DOI: | 10.1016/j.ejpn.2014.12.012 |