John Libbey Eurotext

Temporal lobe epilepsy and emotion recognition without amygdala: a case study of Urbach-Wiethe disease and review of the literature Volume 16, numéro 4, December 2014


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1 Department of Biomedical Sciences, Metabolism, and Neurosciences; University of Modena and Reggio Emilia, Modena
2 Department of Life and Reproduction Sciences, University of Verona, Verona
3 IRCCS Institute of Neurological Sciences, Neurology Unit, Bellaria Hospital, Bologna
4 IRCCS Institute of Neurological Sciences, Neuroradiology Unit, Bellaria Hospital, Bologna
5 Department of Neuroscience, University of Parma, Parma, Italy
6 Danish Epilepsy Centre, Filadelfia/University of Copenhagen, Dianalund, Denmark
* Correspondence: Guido Rubboli Danish Epilepsy Center, Kolonivej 1-4293 Dianalund, Denmark

We describe the epilepsy features and emotion recognition abilities (recognition of basic facial emotions and recognition of emotional prosody) in a patient with Urbach-Wiethe disease with bilateral amygdala calcifications. Our data, supported by ictal video-EEG recording, indicated that our patient suffered from mesial temporal lobe epilepsy. Emotion recognition abilities were compared to those of healthy controls and those of patients with bilateral mesial temporal lobe epilepsy. Our patient showed a selective impairment of the recognition of facial expression of fear, whereas recognition of emotional prosody was preserved, in contrast to bilateral mesial temporal lobe epilepsy patients that presented with deficits in both domains. We also reviewed the literature on epilepsy in Urbach-Wiethe disease (41 patients). Our findings suggest that in Urbach-Wiethe disease, the circumscribed damage of both amygdalae results in a selective dysfunction of fearful face processing, in contrast to bilateral mesial temporal lobe epilepsy patients who present with a widespread and multimodal impairment in the judgement of emotional stimuli.