Abstract Details

Title Multi-thalamic Neuromodulation Improves Seizure Outcome in SCN1A-Dravet Syndrome: Insights from Ambulatory Electrothalamogram

Topic Epilepsy/Clinical Neurophysiology (EEG)

Presentation(s) P3 - Poster Session 3 (5:30 PM-6:30 PM)

Poster/Presentation
Number 1-008

Background DS is a rare genetic epileptic encephalopathy caused by loss-of function SCN1A gene mutation encoding for voltage-gated sodium channel Na_v 1.1. These patients are usually poor candidates for resective epilepsy surgery due to the complexity of their epileptogenic networks involving widespread cortical and subcortical nodes. Thalamic DBS neuromodulation can be a useful treatment alternative for these patients.

Objective

We present a case of Dravet Syndrome (DS) with drug-resistant multifocal epilepsy who we successfully treated using FDA-approved sensing-enabled Percept DBS. Our therapeutic approach involved precise targeting of multiple thalami (centromedian and pulvinar) for DBS to engage and modulate putative epileptogenic circuits.

Design/Methods Our approach to selecting the thalamic neuromodulatory target in DS was driven by mapping the patient's epileptogenic circuit, which could be effectively engaged by thalamic stimulation to reduce excitability. We utilized thalamic recordings as ambulatory electronic seizure diaries to understand seizure cycles. Additionally, we tried to the explore potential mechanisms by which high-frequency continuous stimulation may intricately modulate thalamic nodal excitability.

Results

Patient underwent DBS placement targeting the left centro-median and left pulvinar nuclei and stimulation parameters were gradually advanced during monthly follow-up. Patient experienced gradual reduction in seizure frequency and improvement in quality of life. Analysis of thalamic recordings derived from the device showed that both CM and Pulvinar presented significant circadian as well as multi-day cyclicity. We were also able to show that high-frequency stimulation of CM and Pulvinar modulates thalamic neural activity.

Conclusions

This pioneering case study unveils a groundbreaking exploration of DS by delving into the intricacies of SCN1A channelopathy. It marks the first-ever documentation of seizure cycles, seizure clusters, and thalamic recordings in this context and underscores the immense potential of thalamic neuromodulation in alleviating the formidable seizure burden associated with DS. This case signifies progress in circuit-based therapeutics and automated seizure burden monitoring for severe channelopathies.

Authors/Disclosures
Chetan Nayak, MD (University of Texas Health Science Center at Houston) PRESENTER	Dr. Nayak has nothing to disclose.
Vladimir Vashin	No disclosure on file
Yash Vakilna (UTHealth Houston)	No disclosure on file
Samden Lhatoo, MD (University Hospitals Case Medical Center)	The institution of Dr. Lhatoo has received research support from NIH. Dr. Lhatoo has received intellectual property interests from a discovery or technology relating to health care. Dr. Lhatoo has received publishing royalties from a publication relating to health care.
Sandipan Pati (University of Texas Health Science Center at Houston, Mc Govern Medical School)	No disclosure on file