Intrathecal Dexamethasone in Febrile Infection-Related Epilepsy Syndrome

被引:9
作者
Mehta, Niyati P. [1 ]
Sawdy, Rachel [1 ]
Maloney, Kathleen [2 ]
Overlee, Brittany [3 ,4 ]
Johnson, Renee K. [3 ,4 ]
Howe, Charles L. [3 ,4 ]
Farias-Moeller, Raquel [1 ,2 ]
机构
[1] Med Coll Wisconsin, Dept Neurol, Viral Vector Prod Lab, Milwaukee, WI 53226 USA
[2] Med Coll Wisconsin, Dept Pediat, Milwaukee, WI 53226 USA
[3] Mayo Clin, Translat Neuroimmunol Lab, Rochester, MN 55905 USA
[4] Mayo Clin, Dept Neurol, Rochester, MN 55905 USA
关键词
D O I
10.1212/CPJ.0000000000200153
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Objectives Febrile infection-related epilepsy syndrome (FIRES) is characterized by explosive onset refractory status epilepticus (RSE) in healthy individuals that is refractory to antiseizure medication (ASM), continuous anesthetic infusions (CIs), and immunomodulators. Recently, a case series of patients receiving intrathecal dexamethasone (IT-DEX) was reported with improved RSE control.Methods We present a child with FIRES with favorable outcome after receiving concomitant anakinra and IT-DaEX. A 9-year-old male patient presented with encephalopathy following a febrile illness. He developed seizures evolving to RSE refractory to multiple ASM, 3 CIs, steroids, IVIG, plasmapheresis, ketogenic diet (KD), and anakinra. After continued seizures and inability to wean off CI, IT-DEX was initiated.Results He received 6 doses of IT-DEX with resolution of RSE, rapid wean off CI, and improved inflammatory markers. At discharge, he was ambulating with assistance, speaking 2 languages, and ingesting food orally.Discussion FIRES is a neurologically devastating syndrome with high mortality and morbidity. Proposed guidelines and various treatment strategies are becoming available in the literature. Although treatment with KD, anakinra, and tocilizumab has been successful in previous FIRES cases, our results suggest that the addition of IT-DEX may allow for faster weaning off CI and better cognitive outcomes when initiated early in the course.
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