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A previously healthy 16-year-old boy presented to the emergency department with a peculiar, paroxysmal, stereotypical facial gesture (figure 1). One year earlier, he had first developed a sudden, involuntary ‘coarsening’ of his facial expression lasting several seconds. During this, he retained consciousness and was aware of the facial posture. He had no accompanying aura, fearfulness, anguish or autonomic disturbances. Over the following first 9 months, he had same facial grimace only twice, each time while at school during the daytime. However, in the 3 months before presentation, the paroxysms increased in frequency and became daily in the last month. His neurological examination was normal. Interictal electroencephalography was normal, but 3-day video electroencephalography captured multiple seizures with a possible right frontotemporal origin, each lasting less than 1 min (figure 2). The multiple daily seizures stopped completely after starting levetiracetam. MRI with gadolinium enhancement showed a brain tumour originating from the tip of the right temporal lobe (figure 3), and an anterior temporal lobectomy led to the diagnosis of glioblastoma. He underwent radiation therapy and has remained seizure free for 6 months.
His characteristic seizure semiology (figure 1) comprised jaw tightening, widely open eyelids, depressed angle of the mouth and frowning eyebrows, an appearance that closely resembles the face of an actor in traditional Japanese theatre known as kabuki (figure 4). Similar downwards turning of the mouth during seizures—‘ictal pouting’—is sometimes termed ‘chapeau de gendarme’,1–3 after the shape of an officier de gendarmerie’s hat at the time of the French Revolution (figure 5). Diagnostic delay is common if physicians do not recognise recurrent pouting as a seizure manifestation.
The epileptogenic zone in ictal pouting involves the mesial frontal and anterior cingulate cortices.3 This patient’s peculiar facial expression (kabuki-like visage or chapeau de gendarme) derived from a temporal lobe glioblastoma. This is a good example of the apparent discrepancy between the symptomatogenic zone (mesial frontal and anterior cingulate cortices) and the epileptogenic zone (right temporal lobe). Since the emotional motor circuit involved in facial expressions consists of tight projections from the limbic system (close to the lesion in our patient) to the mesial frontal motor areas,4 5 we assume that his kabuki-like seizure semiology at least partly resulted from the pathological activation of this limbic-mesial frontal circuitry.
Ictal pouting (kabuki visage or chapeau de gendarme) is easily overlooked as a distinctive seizure manifestation.
An epileptogenic zone in the temporal lobe may cause seizure semiology of frontal lobe epilepsy via the emotional motor circuit (tight projections from the limbic system to the mesial frontal motor areas).
Contributors IH took care of the patient, designed the study and drafted the initial manuscript. MK supervised the care and intensively revised the manuscript.
Funding This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent Parental/guardian consent obtained.
Ethics approval National Center for Child Health and Development.
Provenance and peer review Not commissioned; externally peer reviewed by Rob Powell, Swansea, UK.