title: An 8-year old boy with continuous spikes and waves during slow sleep presenting with positive onconeuronal antibodies.
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.
  
  
  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.
  
  
  CONCLUSIONS
  This case suggests that onconeuronal antibodies may be involved in the pathogenesis of CSWS/ESES.
authors.affiliation: Department of Pediatrics, Chinese PLA General Hospital, Beijing 100853, China. Electronic address: huli3188@aliyun.com.
authors.forename: Lin-Yan
authors.initials: LY
authors.lastname: Hu
authors.affiliation: Department of Pediatrics, Chinese PLA General Hospital, Beijing 100853, China. Electronic address: shixiuyu@sina.com.
authors.forename: Xiu-Yu
authors.initials: XY
authors.lastname: Shi
authors.affiliation: Department of Pediatrics, Chinese PLA General Hospital, Beijing 100853, China. Electronic address: wfengchen1@sina.com.
authors.forename: Chen
authors.initials: C
authors.lastname: Feng
authors.affiliation: Department of Pediatrics, Chinese PLA General Hospital, Beijing 100853, China. Electronic address: wjwpatrick@sina.com.
authors.forename: Jian-Wen
authors.initials: JW
authors.lastname: Wang
authors.affiliation: Department of Pediatrics, Chinese PLA General Hospital, Beijing 100853, China. Electronic address: yangg301@sina.com.
authors.forename: Guan
authors.initials: G
authors.lastname: Yang
authors.affiliation: Department of Neurology, The F.M. Kirby Neurobiology Center, Boston Children's Hospital, Boston, MA, USA. Electronic address: stephen.lammers@childrens.harvard.edu.
authors.forename: Stephen H T
authors.initials: SH
authors.lastname: Lammers
authors.affiliation: Department of Pediatrics, Chinese PLA General Hospital, Beijing 100853, China. Electronic address: nkmsvan@gmail.com.
authors.forename: Xiao Fan
authors.initials: XF
authors.lastname: Yang
authors.affiliation: Department of Neurology, The F.M. Kirby Neurobiology Center, Boston Children's Hospital, Boston, MA, USA; Division of Inherited Metabolic Diseases & Pediatric Neurology, Department of Pediatrics, Heidelberg University Hospital, Ruprecht-Karls University, Heidelberg, Germany. Electronic address: d.ebrahimi-fakhari@uni-heidelberg.de.
authors.forename: Darius
authors.initials: D
authors.lastname: Ebrahimi-Fakhari
authors.affiliation: Department of Pediatrics, Chinese PLA General Hospital, Beijing 100853, China; Center of Epilepsy, Beijing Institute for Brain Disorders, Beijing 100069, China. Electronic address: zouliping21@hotmail.com.
authors.forename: Li-Ping
authors.initials: LP
authors.lastname: Zou
mesh_terms: D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000912:Antibodies, Neoplasm / Q000032:analysis*; D000951:Antigens, Neoplasm / Q000235:genetics; D001932:Brain Neoplasms / Q000150:complications; D002648:Child; D000069279:Drug Resistant Epilepsy / Q000150:complications; D004569:Electroencephalography; D004827:Epilepsy / Q000209:etiology*; D006801:Humans; D006867:Hydrolases; D008607:Intellectual Disability / Q000150:complications / Q000523:psychology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008869:Microtubule-Associated Proteins; D009419:Nerve Tissue Proteins / Q000235:genetics; D009447:Neuroblastoma / Q000150:complications; D053578:Opsoclonus-Myoclonus Syndrome / Q000150:complications / Q000503:physiopathology; D012890:Sleep / Q000502:physiology*; D013226:Status Epilepticus / Q000209:etiology / Q000503:physiopathology; D013285:Strabismus / Q000150:complications
chemical_list: D000893:Anti-Inflammatory Agents; D000912:Antibodies, Neoplasm; D000951:Antigens, Neoplasm; C119249:Ma2 antigen; D008869:Microtubule-Associated Proteins; D009419:Nerve Tissue Proteins; C415087:DPYSL5 protein, human; D006867:Hydrolases; D008775:Methylprednisolone
keywords: Anti-CV2/CRMP5; Anti-Ma2; CSWS; ESES; Neuroblastoma; Onconeuronal antibody; Steroid therapy