Authors :
Presenting Author: Gloria Ortiz-Guerrero, MD – Mayo Clinic Rochester
Jeffrey Britton, MD – Mayo Clinic, Rochester MN, USA.
Divyanshu Dubey, MD – Mayo Clinic, Rochester MN, USA.
Brian Lundstrom, MD PhD – Mayo clinic, Rochester, Minnesota
Keith Starnes, MD – Mayo Clinic, Rochester MN, USA.
Gregory Worrell, MD,PhD – Mayo Clinics
Nicholas Gregg, MD – Mayo clinic, Rochester, Minnesota
Kelsey M. Smith, MD – Mayo Clinic, Rochester MN, USA.
Rationale:
Rasmussen Syndrome (RS) is an autoimmune disorder characterized by progressive, disabling neurological deficits that often lead to drug-resistant epilepsy (DRE). Recently, neuromodulation has emerged as a promising adjunctive therapy for DRE. This study evaluates response rates in patients with RS undergoing neuromodulation therapies.Methods:
Retrospective case series of patients diagnosed with RS based on the Bien et al. criteria (2005)1, who were evaluated between January 1, 2001, and June 1, 2023, at Mayo Clinic and treated with invasive and noninvasive neuromodulation. Data encompassed demographic, clinical, and neuromodulation information. Patients with a 50% reduction in seizure frequency at the last follow up appointment were considered responders. Results:
Ten patients were identified (nine females). The mean age of seizure onset was 11.4 years (± 7.3). Patients were treated with vagus nerve stimulation (VNS, n = 7), chronic subthreshold stimulation (CSS, n = 3), deep brain stimulation (DBS, n = 2), and transcranial magnetic stimulation (TMS, n=1). Among the VNS patients, one was a responder, one was indeterminate due to insufficient data, and the remaining were non-responders. Notably, all patients treated with DBS and TMS had previously failed to respond to VNS. Focusing on DBS, one patient was a responder and was implanted with 4 leads targeting the bilateral ANT, left CM, and left pulvinar nucleus. The DBS non-responder had leads targeting bilateral anterior thalamic (ANT) and centromedian (CM) nuclei. Among CSS patients, only one was a responder, with leads targeting the ANT, CM, anterior and posterior insula on the right. This same patient had a right anterior temporal lobectomy (ATL) and right inferior frontal/opercular laser interstitial thermal therapy (LITT) prior to neuromodulation. The two CSS non-responders had leads targeting the left primary motor area; and the second had leads placed over both the left primary motor area and supplementary motor area. One patient was treated with TMS during a hospitalization for epilepsia partialis continua. TMS led to resolution of EPC, and the patient remained a 50% responder at last follow-up.
Conclusions:
Neuromodulation in RS showed variable outcomes with five responders across different modalities (VNS: n=1, DBS: n=1, CSCS: n=1, TMS: n=1). However, six patients were non-responders. These results highlight the variable effectiveness of neuromodulation and the need for personalized treatment approaches and further research.
Funding: No funding was received.