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

J Neuroimmunol

Department of Pediatrics, Division of Pediatric Neurology and Developmental Neuroscience, Baylor College of Medicine and Texas Children's Hospital, 6701 Fannin Street, Ste 1250, Houston, TX 70030, United States of America. Electronic address:

Published: October 2024

AI Article Synopsis

  • New-onset refractory status epilepticus (NORSE) is a serious condition that can cause significant disability, often not responding well to standard treatments.
  • An 11-year-old girl with anti-GAD 65 encephalitis who experienced NORSE showed minimal improvement with regular anti-seizure medications and first-line therapies.
  • After receiving intrathecal dexamethasone (IT-DEX) along with rituximab, her neuroinflammation decreased, seizure frequency dropped, and she could reduce anesthesia, suggesting IT-DEX could be beneficial early on for various causes of refractory status epilepticus.

Article 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.

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Source
http://dx.doi.org/10.1016/j.jneuroim.2024.578430DOI Listing

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