The "chapeau de gendarme" sign in focal epilepsy: A systematic review

The "chapeau de gendarme" sign, also known as "ictal pouting," is a distinctive facial expression observed in focal epilepsy, characterized by a turned-down mouth with symmetrical lip and chin contraction. This systematic review investigates its clinical relevance, anatomical origins, and diagnostic value, particularly in presurgical evaluation. The sign is most commonly associated with mesial frontal epilepsy, particularly involving the anterior cingulate cortex (ACC), though moderate evidence links it to a broader network including insulo-opercular and temporo-parietal regions. Variability in semiologic descriptions and the absence of standardized definitions present challenges to its consistent recognition. Focal cortical dysplasia (FCD) was identified as the most frequent pathological substrate linked to this sign, particularly in MRI-positive cases, while nearly half of the reviewed cases were MRI-negative emphasizing the importance of localizing signs such as the "chapeau de gendarme" in cases of suspected FCD. FDG-PET consistently showed hypometabolism in relevant regions, supporting its utility in identifying the epileptogenic zone in MRI-negative cases. The propagation of ictal discharges across interconnected brain regions highlights the involvement of specific network dynamics in generating this semiology. The "chapeau de gendarme" sign holds significant value in presurgical evaluations, particularly as part of a multimodal assessment integrating neuroimaging and electrophysiological data. Despite the descriptive nature of included studies and limited photo documentation, the sign remains a valuable diagnostic tool. Further research is needed to standardize reporting and clarify its role in guiding surgical management, especially in MRI-negative focal epilepsy.