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Autoimmune limbic encephalopathy and anti-Hu antibodies in children without cancer

Jérôme Honnorat1, Adrien Didelot, Evgenia Karantoni

  • 1French Reference Center on Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, Neurologie B, Bron, France. jerome.honnorat@chu-lyon.fr

Neurology
|May 10, 2013
PubMed

Insights

Anti-Hu antibodies (Hu-Abs) in children can indicate paraneoplastic neurological syndrome (PNS) or aggressive autoimmune limbic encephalitis. Early diagnosis and T-cell therapies may improve outcomes for this rare condition.

Area of Science:

  • Neurology
  • Immunology
  • Pediatrics

Background:

  • Anti-Hu antibodies (Hu-Abs) are typically associated with paraneoplastic neurological syndrome (PNS) in adults.
  • Understanding the role of Hu-Abs in pediatric neurological disorders is crucial for diagnosis and treatment.

Observation:

  • A retrospective study identified 8 pediatric patients with Hu-Abs out of 251 total patients.
  • Only 2 children had neuroblastoma and opsoclonus-myoclonus; the other 6 presented with autoimmune limbic encephalitis without cancer.

Findings:

  • Pediatric Hu-Abs are associated with both PNS and a distinct, aggressive autoimmune limbic encephalitis.
  • Limbic encephalitis cases showed abnormal MRI findings and difficult-to-manage epilepsy, with 5 children developing cognitive impairments.

Implications:

  • Hu-Abs in children may signify an aggressive autoimmune encephalitis, distinct from adult PNS presentations.
  • Further research is needed to establish the incidence of this syndrome and explore the benefits of early diagnosis and T-cell-directed immunotherapies.
Abstract

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