Stereoencephalography (SEEG) is becoming a widespread diagnostic procedure for drug-resistant epilepsy investigation. Techniques include frame-based and robot-assisted implantation, and more recently, frameless neuronavigated systems (FNSs). Despite its recent use, the accuracy and safety of FNS are still under investigation.
To assess in a prospective study the accuracy and safety of a specific FNS use for SEEG implantation.
Twelve patients who underwent SEEG implantation using FNS (Varioguide [Brainlab]) were included in this study. Data were collected prospectively and included demographic data, postoperative complications, functional results, and implantation characteristics (i.e., duration and number of electrodes). Further analysis included accuracy at entry point and target using measurements of the euclidean distance between planned and actual trajectories.
Eleven patients underwent SEEG-FNS implantation from May 2019 to March 2020. One patient did not undergo surgery because of a bleeding disorder. The mean target deviation was 4.06 mm, and mean entry point deviation was 4.2 mm, with insular electrodes significantly more deviated. Results excluding insular electrodes showed a mean target deviation of 3.66 mm and a mean entry point deviation of 3.77 mm. No severe complications occurred; a few mild to moderate adverse events were reported (1 superficial infection, 1 seizure cluster, and 3 transient neurologic impairments). The mean implantation duration by electrodes was 18.5 minutes.
Implantation of depth electrodes for SEEG using FNS seems to be safe, but larger prospective studies are needed to validate these results. Accuracy is sufficient for noninsular trajectories but warrant caution for insular trajectories with statistically significantly less accuracy.

Epilepsy is a rare clinical manifestation in Williams-Beuren syndrome patients. However, some studies report the presence of infantile spasms and epilepsy in patients carrying larger deletions. Herein, we describe a 13-year-old female affected by Williams-Beuren syndrome and pharmacoresistant epilepsy reporting a de novo large heterozygous 7q11.21q21 deletion (19.4 Mb) also including the YWHAG gene. Studies indicate that cannabidiol is effective as adjunctive therapy for seizures associated with tuberous sclerosis complex, and it is under investigation also in focal cortical dysplasia. When treated with cannabidiol, our patient showed a significant reduction in seizure frequency and intensity, and improved motor and social skills. We hypothesized that CBD could exert a gene/disease-specific effect.

Objective: Sleep-related hypermotor epilepsy (SHE) is a sleep-related focal epilepsy which is often misdiagnosed. Despite active pharmacological therapy in the management of this disorder, satisfactory seizure control still cannot be achieved. Therefore, the aim of the present study was to identify this disorder among people who were seeking medical advice at Cairo University Epilepsy Unit (CUEU), characterizing its clinical, electroencephalographic and imaging features besides identifying possible indicators of inadequate seizure control on drug-therapy. Patients and methods: This study was carried out on 26 patients with SHE who were subjected to detailed history taking and examination in addition to home video recording, video electroencephalographic (EEG) monitoring and brain imaging. Ictal semiology and EEGs were reviewed and analyzed by experienced neurologists. Results: SHE is an uncommon sleep-related focal epilepsy. In our series, median age of the patients was 18.5 years. It is characterized by being sporadic, with often frontal lobe seizure onset (14/26, 53.8%) and with occasional occurrence in wakefulness. Adolescence age at disease onset (11 years, 6-15), duration less than 1 min, clustering, lack of auras and often uninformative brain imaging (22/25, 88%) are all documented features. Moreover, it has a relatively poor outcome on pharmacological therapy (16/26, 61.5%). Longer disease duration (>4.5 years) was a significant feature of the patients exhibiting inadequate seizure control. Conclusions: Our data show relatively poor prognosis of SHE on medical therapy. Its outcome is significantly related to disease duration at the time of diagnosis. Abbreviation: SHE = Sleep related hypermotor epilepsy.