Authors :
Presenting Author: Linda Sherine Alfred, MD – University of Tennessee Health Science Center. Department of Neurology. Memphis, TN.
Juan Goyanes-Vasquez, MD – University of Tennessee Health Science Center. Department of Neurology. Memphis, TN.
Haewon Shin, MD – University of Tennessee Health Science Center. Department of Neurology. Memphis, TN.
Rationale:
Autoimmune encephalitis, a rare underdiagnosed serious medical condition with prolonged morbidity, disability and even mortality has been more recognized as a potentially treatable etiology of new onset seizures and refractory status epilepticus. Early diagnosis and management with immunomodulatory therapy is crucial to improve the outcome[1-5]. We aim to determine the epidemiological characteristics of the new onset refractory status epilepticus from autoimmune encephalitis in adult patients in the mid-south region, in comparison with the current literature.
Methods:
We reviewed a 5-year retrospective case series of patients older than 16 years old with a diagnosis of status epilepticus from autoimmune encephalitis from January 1, 2017 to December 31, 2022, using medical records upon previous hospital IRB approval. The data collected was based on demographic characteristics, clinical presentation, radiological and EEG findings, ENC 2 panel results along with treatment response and outcome.
Results:
A total of 7 patients were included [3 anti-NMDA, 1 anti-GAD and anti-VGKC, 1 autoimmune autonomic ganglionopathy and 2 seronegative] with female (57%) and black (71%) predominance. All of them developed illnesses either during summer or winter[2]. Three female patients (42.8 %) younger than 30 years old had anti-NMDA receptor encephalitis, with frontal or temporal onset seizures and behavioral issues. One of them had positive antibody to EBV IgG in CSF and the other patient had a mature cystic teratoma, which are potential triggers for anti-NMDA receptor encephalitis[3,4]. One out of three patients with anti-NMDA receptor encephalitis, had EEG extreme delta-brush pattern with poor prognosis [3] and died without any response to therapy. All other 6 patients (85%) improved despite prolonged ICU stay (14 to 54 days) and respiratory failure. Two seronegative patients (28.5%) presented with focal seizures from the frontal or temporal region followed by focal status which responded to IVIG and high dose steroids. One patient with anti-GAD and anti-VGKC positive (14.3%) presented with seizures and psychosis who improved with high dose steroids, PLEX and IVIG. The patient with anti-ganglionic nicotinic ACh receptor encephalitis presented with seizures along with focal motor weakness developed cardiac arrest possibly due to autonomic dysfunction [5], resuscitated and later improved with PLEX and IVIG followed by immunosuppression with mycophenolate mofetil. The seizures were treated predominantly with levetiracetam, lacosamide, phenobarbital and perampanel. Conclusions:
Our retrospective case series study showed that despite having long ICU stay and ventilator days, 85% of the patients with new onset refractory status epilepticus due to autoimmune encephalitis improved with high dose steroids, PLEX and/ or IVIG except for one patient with anti-NMDA receptor positive with extreme delta brush pattern who died [3]. EBV IgG positive in CSF in one patient with anti-NMDA receptor encephalitis suggests the possibility of EBV infection being trigger for anti-NMDA encephalitis[4].
Funding: None