Abstracts

RNS in Patients with Suspected Rasmussen's Encephalitis

Abstract number : 1.377
Submission category : 18. Case Studies
Year : 2021
Submission ID : 1826708
Source : www.aesnet.org
Presentation date : 12/4/2021 12:00:00 PM
Published date : Nov 22, 2021, 06:55 AM

Authors :
Marytery Fajardo, MD - Nicklaus Children's Hospital; Ann Hyslop - Nicklaus Children's Hospital; Matt Lallas - Nicklaus Children's Hospital; John Ragheb - Nicklaus Children's Hospital; Shelly Wang - Nicklaus Children's Hospital

Rationale: Limited data is currently available regarding the effects of responsive neurostimulation (RNS) on epilepsia partialis continua (EPC) or on progressive diseases such as Rasmussen’s encephalitis. We present two such cases in which RNS was used for the treatment.

Methods: Patient A is a 25-year-old male who presented with focal seizures at age 15 characterized by goosebumps, then left facial pulling and left arm held tightly against chest. Serial magnetic resonance imaging (MRI) scans demonstrated right hemisphere atrophy suggestive of Rasmussen’s encephalitis, though biopsy was inconclusive. Following a two-staged right frontal resection and laser thermal ablation of right parietal region posterior to previous resection, he continued to have multiple daily left arm sensorimotor seizures. In January 2019 he had a Neuropace device implanted with 4 strip electrodes placed flanking the superior and inferior borders of the resection cavity.

Patient B is a 16-year-old female with inflammatory bowel disease who presented at age 14 years with EPC characterized by left lower extremity focal motor seizures without loss of awareness. She underwent a two-staged right paracentral resection. Pathology demonstrated inflammation thought to be suspicious for Rasmussen’s encephalitis. She was having multiple daily seizures with high doses of three anti-seizure medications just prior to Neuropace implantation. She also developed a left hemiparesis. She had three depth electrodes implanted in the right frontoparietal region in July 2020.

Results: Neither patient experienced adverse effects nor complications. Patient A has subsequently been followed for two years and experienced a 60% reduction in seizure burden. Patient B has been followed for nearly one year and has experienced greater than 60% reduction in seizure burden, including epochs of rare to no disabling seizures.

Conclusions: RNS was used in two patients with refractory focal sensorimotor seizures, both having experienced EPC throughout their clinical course with suspicion for Rasmussen’s encephalitis. Both patients experienced a greater than 50% reduction in seizure burden.

Funding: Please list any funding that was received in support of this abstract.: None.

Case Studies