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IT-DEX and B cell depletion in a child with anti-GAD 65 autoimmune encephalitis presenting as NORSE: A case report
Jonathan M Yarimi1, Alexander J Sandweiss1, Karla P Salazar1
1Department 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.
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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