Authors :
Presenting Author: Muhammad Usman Khalid, MBBS – University of Kentucky
Ryan Cloyd, MD – Indiana University
Regan Martin, MD – University of Kentucky
Sally Mathias, MD – University of Kentucky
Timothy Ainger, PhD – University of Kentucky
Ghulam Q. Khan, MD – University of Kentucky
Zahra Haghighat, MD – University of Kentucky
Julie Youssefi, MD – University of Kentucky
Brooks Fowler, MSc – Neuropace
Flavius Raslau, MD – University of Kentucky
Farhan A. Mirza, MD – University of Kentucky
Rationale: Sturge-Weber syndrome (SWS) is often associated with refractory epilepsy and may require major surgery to achieve seizure control. Responsive neurostimulation (RNS) may represent a less invasive option. We present the first peer-reviewed case of RNS for SWS-related seizures, where the patient was unwilling to undergo a lobar disconnection of the seizure onset zone.
Methods:
Clinical and radiological reports were reviewed using the electronic health system. Surgical decision making was elucidated by the operating surgeon and images were marked by the radiologist to highlight key features.
Results: The patient underwent extensive pre-surgical workup, including fMRI that serendipitously occurred during the post-ictal period, revealing hypoperfusion in the right occipital lobe that correlated well with suspected seizure onset zone as identified by video-EEG and semiology. This was followed by placement of two depth RNS strips, implanted over the medial and lateral surfaces of the occipital lobe. Over two years of follow-up, the patient reports improvement in severity of experienced seizures and reduced post-ictal symptoms. The patient has thus far tolerated the RNS well and reported subjective improvements in seizures and overall
cognition. The long-term recordings from his device revealed multiple events that did not correlate with clinical symptoms, likely representing successfully aborted seizures
Conclusions:
This case provides an illustrative example of the utility of responsive neurostimulation for the treatment of seizures related to Sturge-Weber Syndrome. RNS provides an appealing alternative to other surgical procedures such as resection or disconnection and is efficacious in reducing seizure burden.
Funding: No funding was utilized in this research study.