IT-DEX and B cell depletion in a child with anti-GAD 65 autoimmune encephalitis presenting as NORSE: A case report

J Neuroimmunol. 2024 Oct 15:395:578430. doi: 10.1016/j.jneuroim.2024.578430. Epub 2024 Aug 8.

Abstract

New-onset refractory status epilepticus (NORSE) is a devastating clinical condition that often leads to severe disability. Intrathecal dexamethasone (IT-DEX) has been reported to improve refractory status epilepticus. We present an 11-year-old female with anti-GAD 65 encephalitis presenting as NORSE who had minimal response to standard anti-seizure medications and first-line immunotherapies. The patient received 6 doses of IT-DEX in conjunction with rituximab which correlated with subsequent decreased neuroinflammation, reduced seizure burden and aided in weaning anesthetic infusions. Our case with literature review suggests IT-DEX may be utilized as an early intervention in those with refractory status epilepticus from various etiologies.

Keywords: Autoimmune encephalitis; Cytokines; Glutamic acid decarboxylase antibody; Intrathecal dexamethasone; New-onset refractory status epilepticus.

Publication types

  • Case Reports

MeSH terms

  • Autoantibodies / blood
  • B-Lymphocytes* / immunology
  • Child
  • Dexamethasone* / administration & dosage
  • Dexamethasone* / therapeutic use
  • Encephalitis* / drug therapy
  • Encephalitis* / immunology
  • Female
  • Glutamate Decarboxylase / immunology
  • Hashimoto Disease / diagnosis
  • Hashimoto Disease / drug therapy
  • Hashimoto Disease / immunology
  • Humans
  • Injections, Spinal
  • Lymphocyte Depletion / methods
  • Rituximab / therapeutic use
  • Status Epilepticus* / drug therapy
  • Status Epilepticus* / etiology
  • Status Epilepticus* / immunology

Substances

  • Dexamethasone
  • Glutamate Decarboxylase
  • glutamate decarboxylase 2
  • Rituximab
  • Autoantibodies

Supplementary concepts

  • Hashimoto's encephalitis