An 8-year old boy with continuous spikes and waves during slow sleep presenting with positive onconeuronal antibodies. (March 2015)

• Record Type: Journal Article
• Authors: 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
• Is Part Of: European journal of paediatric neurology. Volume 19:Number 2(2015:Mar.)
• Page Start: 257
• Page End: 261
• Publication Date: 2015-03
• Subjects: Pediatric neurology -- Periodicals; Nervous System Diseases -- Periodicals; Child -- Periodicals; Infant -- Periodicals; Neurologie pédiatrique -- Périodiques; Pediatric neurology; Electronic journals; Periodicals; Electronic journals; 618.928
• DOI: 10.1016/j.ejpn.2014.12.012
• Languages: English
• ISSNs: 1090-3798

Abstract

<abstract xml:lang="en" abstract-type="author" id="abs0010"> <title id="sectitle0010">Abstract</title> <sec> <title id="sectitle0016">Objective</title> <p id="abspara0011">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.</p> </sec> <sec> <title id="sectitle0021">Material & methods</title> <p id="abspara0016">A combination of clinical characterization and follow-up, video EEG and laboratory investigations.</p> </sec> <sec> <title id="sectitle0028">Results</title> <p id="abspara0021">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.</p> </sec> <sec> <title id="sectitle0032">Conclusion</title> <p id="abspara0026">This case suggests that onconeuronal antibodies may be involved in the pathogenesis of CSWS/ESES.</p> </sec> </abstract>