Neurol Sci. 2025 Oct 24. doi: 10.1007/s10072-025-08582-0. Online ahead of print.
ABSTRACT
BACKGROUND: Subcortical band heterotopia (SBH) is a neuronal migration disorder within the agyria-pachygyria spectrum, ranging from subtle thin bands to diffuse lissencephaly. Adult presentations and prognostic factors remain poorly defined.
OBJECTIVE: To characterize the clinical, electrophysiological, radiological features, and long-term outcomes of adults with epilepsy and SBH.
METHODS: We retrospectively reviewed 16 adults diagnosed between 2000 and 2024. Demographics (including age at seizure onset), epilepsy risk factors, seizure semiology, treatment histories, and follow-up durations were obtained from hospital records. Interictal and ictal EEG data were gathered from epilepsy center archives and PACS, and MRI studies were analyzed for lesion distribution.
RESULTS: All 16 patients (12 females; mean age 40 years, range 25-55) remained alive at last follow-up. Seizure onset occurred between 6 months and 15 years of age. Developmental delay was noted in 7 patients (44%). All exhibited pharmacoresistant epilepsy. Interictal EEGs demonstrated focal (predominantly temporal in 11/16, 69%), multifocal, combined focal/multifocal, and generalized epileptiform discharges. Seven underwent video-EEG monitoring, revealing predominantly extratemporal seizure semiology in 6/7 (86%). Levetiracetam and carbamazepine were the most frequently employed antiseizure medications. Four patients received vagus nerve stimulation for drop attacks and generalized seizures; three experienced ≥ 50% seizure reduction.
CONCLUSION: The SBH in adults typically manifests as refractory focal epilepsy with frequent developmental delays. Given the absence of pathognomonic semiology, comprehensive evaluation-including multimodal EEG, high-resolution neuroimaging, and genetic testing when available-is essential for accurate diagnosis and tailored management.
PMID:41134416 | DOI:10.1007/s10072-025-08582-0
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