ArticleFrontiers in neuroscience2026
Case Report: Right frontal periventricular heterotopia underlying secondary network epilepsy with a Lennox-Gastaut phenotype: multimodal SEEG, CCEP, and rs-fMRI evidence.
Article in Frontiers in neuroscience, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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Abstract
Objective: Lennox-Gastaut syndrome (LGS) is generally regarded as a developmental and epileptic encephalopathy involving distributed brain networks, although a focal structural abnormality may occasionally recruit bilateral cortical and thalamic circuits and produce an LGS phenotype. We characterized the spatiotemporal organization of such a network and its changes after focal SEEG-guided radiofrequency thermocoagulation (RF-TC). Methods: A 50-year-old woman with drug-resistant epilepsy, multiple seizure types, diffuse slow spike-and-wave discharges, generalized paroxysmal fast activity, intellectual impairment, and right frontal periventricular heterotopia (PVH) underwent stereo-electroencephalography (SEEG). Cortico-cortical evoked potentials (CCEPs) were acquired before and after SEEG-guided radiofrequency thermocoagulation (RF-TC). Resting-state functional MRI (rs-fMRI), obtained before and 3 months after RF-TC, was analyzed using three seeds: the entire right frontal PVH and the right and left centromedian thalamic nuclei (CM). Results: SEEG identified the earliest sustained ictal activity within the sampled right frontal PVH-related medial contacts, accompanied by high-frequency activity. This was followed by ipsilateral lateral frontal recruitment, contralateral frontal synchronization, and subsequent bilateral CM involvement. Two-stage RF-TC of the onset and early-recruited networks was followed by seizure freedom through 9 months. After RF-TC, right-sided stimulation produced markedly attenuated lesion-related CCEP responses, whereas left-sided responses and responses between thalamic contacts showed little appreciable change. Descriptive rs-fMRI difference maps demonstrated decreased local frontal connectivity around the treated territory and increased long-range connectivity for the PVH seed; both CM seeds showed predominantly increased connectivity with distributed cortical and cerebellar regions. Conclusion: In this patient, a focal PVH-related epileptogenic network was associated with secondary bilateral cortical and thalamic recruitment underlying an LGS phenotype. CCEP and rs-fMRI captured complementary aspects of postoperative network change: attenuation of stimulation-evoked propagation and postoperative changes in intrinsic functional connectivity, respectively.
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