title: Autoimmune limbic encephalopathy and anti-Hu antibodies in children without cancer.
abstract: OBJECTIVE
  The aim of this study was to describe the clinical presentation of children and adolescents with anti-Hu antibodies (Hu-Abs).
  
  
  METHODS
  This was a retrospective study of children and adolescents with Hu-Abs collected by the French Paraneoplastic Neurological Syndrome (PNS) Reference Center between January 1, 2000 and December 31, 2011.
  
  
  RESULTS
  The center identified 251 patients with Hu-Abs. Only 8 patients were younger than 18 years. All of the 243 adult patients had PNS. In contrast, of the 8 children, only 2 (25%, Fisher exact test p = 0.0003) had neuroblastoma and opsoclonus-myoclonus. The other 6 children (5 female and 1 male) presented with limbic encephalitis (progressive personality changes, memory loss, and seizure) and were free of cancer (mean follow-up time: 50 months; range: 34-72 months). Brain MRI scans were abnormal in 4 of the 6 patients, with left, right, or bitemporal T2/fluid-attenuated inversion recovery hyperintensity. Protein levels and cell counts in the CSF were normal in all patients, but numerous oligoclonal bands were observed in 4 patients. All 6 patients received antiepileptic drugs and immunotherapy, but management of epilepsy was difficult in all of them. Five of the children developed cognitive impairments.
  
  
  CONCLUSIONS
  In children, as in adults, Hu-Abs can be a marker of PNS. However, in contrast to adults, Hu-Abs in children are also associated with an aggressive form of autoimmune nonparaneoplastic limbic encephalitis. Future studies should be conducted to determine the incidence of this syndrome and whether earlier diagnosis and T-cell-directed immunotherapies may improve its prognosis.
authors: French Reference Center on Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, Neurologie B, Bron, France. jerome.honnorat@chu-lyon.fr
authors: Jérôme
authors: J
authors: Honnorat
authors: Adrien
authors: A
authors: Didelot
authors: Evgenia
authors: E
authors: Karantoni
authors: Dorothée
authors: D
authors: Ville
authors: François
authors: F
authors: Ducray
authors: Laetitia
authors: L
authors: Lambert
authors: Kumaran
authors: K
authors: Deiva
authors: Marie
authors: M
authors: Garcia
authors: Phintip
authors: P
authors: Pichit
authors: Gaelle
authors: G
authors: Cavillon
authors: Véronique
authors: V
authors: Rogemond
authors: Jean-Yves
authors: JY
authors: DeLattre
authors: Marc
authors: M
authors: Tardieu
mesh_terms: D000293:Adolescent; D000328:Adult; D000974:Antibodies, Antinuclear / Q000096:biosynthesis* / Q000097:blood; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000276:immunology*; D015415:Biomarkers / Q000097:blood; D002648:Child; D051959:ELAV Proteins / Q000276:immunology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D020363:Limbic Encephalitis / Q000175:diagnosis* / Q000453:epidemiology / Q000276:immunology*; D008297:Male; D009369:Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000276:immunology; D012189:Retrospective Studies
chemical_list: D000974:Antibodies, Antinuclear; D015415:Biomarkers; D051959:ELAV Proteins