New Onset Refractory Status Epilepticus (NORSE) as a Presentation of NMDA Encephalitis in a Pediatric Patient

Authors

  • Amir Farooq Khan Department of Neurosurgery, Pakistan Institute of Medical Sciences Islamabad
  • Syed Muhammad Hur Abbas Khyber Medial College Peshawar
  • Gulsam Bashir Khyber Medial College Peshawar

Keywords:

Encephalitis, NORSE, NMDA

Abstract

 

Anti-N-methyl D-aspartate (NMDA) receptor (anti-NMDAR) encephalitis is caused by immunoreactivity against the NR1 subunit of the NMDA receptor. We present the case of a thirteen years old boy with history of sudden onset of seizure fort five days back. After a detailed history and a thorough clinical examination the autoimmune encephalitis antibodies profile were sent. This came positive for NMDA receptor and negative for CASPR-2, Type AMPA1/2, LGI-1, DPPX, and GABAB receptor antibodies. He was started on sodium valproate, carbamazepine, phenobarbital, Clonazepam along with full dose corticosteroids but did not respond significantly. Patient was shifted to ICU and intubated. Anesthesia with ketamine and propofol was started. After twenty days the patient was seizure free. He was given IVIGs and immunosuppressive therapy. He was sent home and two weeks follow up was advised. New onset refractory status epilepticus preceded the diagnosis of NMDA Encephalitis. The diagnosis of autoimmune encephalitis should be considered a top differential in relapsing patients as it has a very wide range of presentation. Prompt diagnosis and early treatment are the need of the hour as the ramifications of a mis or missed diagnosis could be detrimental.

References

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Published

2020-10-26

How to Cite

Khan, A. F., Abbas, S. M. H., & Bashir, G. (2020). New Onset Refractory Status Epilepticus (NORSE) as a Presentation of NMDA Encephalitis in a Pediatric Patient. THE STETHO, 1(1), 4-6. Retrieved from http://thestetho.com/index.php/ts/article/view/8

Issue

Section

Case Reports