Authors :
Presenting Author: Christina Wombles, MSN, ARNP – AdventHealth Orlando
Holly Skinner, DO – Epilepsy – AdventHealth Orlando; Angel Claudio, MD – Epilepsy – AdventHealth Orlando; Ki Lee, MD – Epilepsy – AdventHealth Orlando; Emilyn Ballard, RN – Adult Nurse Coordinator, Epilepsy, AdventHealth Orlando; Bassel Raad, MD – AdventHealth Orlando; Amay Parikh, MD – AdventHealth Orlando; Okorie Okorie, MD – AdventHealth Orlando; Mahammed Khan, MD – AAH; Elakkat Gireesh, MD – Epileptologist, Epilepsy, AdventHealth Orlando
Rationale:
Our group previously published a case report illustrating our treatment of a patient with refractory anti-N-Methyl-D-Aspartate (NMDA) encephalitis with intrathecal rituximab. We now have a cohort of 15 patients treated with intrathecal rituximab for various autoimmune-related encephalopathies. This review highlights the safety of intrathecal rituximab as a treatment option for patients with autoimmune-related status epilepticus or refractory epilepsy.
Methods:
We performed a retrospective review of adult patients treated in our center with intrathecal rituximab for autoimmune encephalopathy with status epilepticus or refractory epilepsy from 2017 throught 2022 utilizing chart review.
Results:
Fifteen patients (10 females and five males) ages 20-53 received intrathecal rituximab from 2017 through 2022. None had clear epilepsy risk factors, although two had had recent COVID infection, one had a genetic variant of unknown significance, one had a prior closed head injury, and one had a history of cocaine abuse. Two had hypothyroidism, one had Hashimoto’s thyroiditis, three had bipolar disorder, two had GI symptoms, two had headaches, and one had schizophrenia. Positive antibodies included anti-NMDA (4), thyroid peroxidase or thyroglobulin antibodies (4), anti-glutamic acid decarboxylase (3), and anti-voltage-gated potassium channel (3). Seizure types included focal onset, myoclonic, convulsive, and absence seizures. EEG showed a delta brush pattern in five patients and status epilepticus in three patients. Patients were previously treated with multiple anti-seizure medications and were noted to have refractory epilepsy or super-refractory status epilepticus. Seven had normal MRI brain findings and abnormalities noted in the other patients did not have any cohesive features. Of the eight patients who had computerized tomography (CT) chest scans completed, none had any relevant findings. Of the twelve patients who had CT abdomen/pelvis completed, seven were normal, two showed colitis, two showed ovarian teratoma or cyst, and one showed a renal cyst.
After intrathecal rituximab administration, only three patients reported minimal, transient side effects including headache, urinary retention, chills, anxiety, dizziness, confusion, and nausea.
Two intensive care unit (ICU) patients had transient elevated liver enzymes and one ICU patient had hepatic failure which resolved with cessation of multiple potentially offending agents. Four intubated ICU patients had pneumonia or fever requiring antibiotics. These complications were not clearly attributed to intrathecal rituximab as these patients were intubated, in the ICU, and were on multi-modal treatment. One patient developed a subsequent positive QuantiFERON requiring treatment. All but one patient was discharged from the hospital in stable condition. One patient died of unrelated causes.
Conclusions:
Our data provides confidence that intrathecal rituximab can be used safely in patients with immune-mediated status epilepticus and refractory epilepsy.
Funding: None