Summary:Purpose: Malformations of cortical development (MCDs) are usually highly epileptogenic, and their hyperexcitability could facilitate the occurrence of reflex seizures. We sought to characterize reflex seizures in patients with MCDs and refractory epilepsy.Methods: Clinical, electrographic, and neuroimaging data were reviewed in eight patients with MCDs who had reflex seizures reproduced during presurgical evaluation.Results: All eight patients had both reflex and spontaneous seizures. In six, however, drop attacks or axial myoclonic seizures occurred only upon specific sensory stimulation. Reflex seizures were induced by more than one type of stimulus in most patients, but anatomofunctional correlations could usually be invoked. Six patients had significant intellectual impairment. Surgical resection controlled seizures in two patients.Conclusions: Reflex seizures in patients with MCDs may be medically refractory and may often manifest as drop attacks or axial myoclonus. Surgical resection of focal lesions can bring reflex seizures under control. Putative mechanisms related to the relatively low frequency of reflex seizures in MCDs are discussed. Key Words: Reflex seizures-Malformations of cortical development-High degrees of epileptogenicity.Reflex seizures most commonly occur in patients with genetically related epilepsy syndromes (1-6), in which triggering stimuli are related to photosensitivity or higherorder cognitive processing (7,8). Seizure provocation through other stimuli and in patients with symptomatic epilepsies is less common, although perirolandic lesions are known to induce reflex seizures.A common assumption regarding reflex seizures is that hyperexcitable intracortical or corticosubcortical sensory circuits lead to fast and uninhibited recruiting and synchronization of neuronal pools (9-11). Converging clinical and experimental evidence suggests that malformations of cortical development (MCDs) are among the most hyperexcitable epileptogenic lesions (12,13). In addition, these malformations are often intrinsically epileptogenic