title: Temporal lobe epilepsy and emotion recognition without amygdala: a case study of Urbach-Wiethe disease and review of the literature.
abstract: 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.
authors.affiliation: Department of Biomedical Sciences, Metabolism, and Neurosciences; University of Modena and Reggio Emilia, Modena.
authors.forename: Stefano
authors.initials: S
authors.lastname: Meletti
authors.affiliation: Department of Life and Reproduction Sciences, University of Verona, Verona.
authors.forename: Gaetano
authors.initials: G
authors.lastname: Cantalupo
authors.affiliation: IRCCS Institute of Neurological Sciences, Neurology Unit, Bellaria Hospital, Bologna.
authors.forename: Francesca
authors.initials: F
authors.lastname: Santoro
authors.affiliation: Department of Biomedical Sciences, Metabolism, and Neurosciences; University of Modena and Reggio Emilia, Modena.
authors.forename: Francesca
authors.initials: F
authors.lastname: Benuzzi
authors.affiliation: IRCCS Institute of Neurological Sciences, Neuroradiology Unit, Bellaria Hospital, Bologna.
authors.forename: Anna Federica
authors.initials: AF
authors.lastname: Marliani
authors.affiliation: Department of Neuroscience, University of Parma, Parma, Italy.
authors.forename: Carlo Alberto
authors.initials: CA
authors.lastname: Tassinari
authors.affiliation: IRCCS Institute of Neurological Sciences, Neurology Unit, Bellaria Hospital, Bologna, Danish Epilepsy Centre, Filadelfia/University of Copenhagen, Dianalund, Denmark.
authors.forename: Guido
authors.initials: G
authors.lastname: Rubboli
mesh_terms: D000328:Adult; D000679:Amygdala / Q000503:physiopathology*; D004644:Emotions; D004833:Epilepsy, Temporal Lobe / Q000175:diagnosis / Q000503:physiopathology* / Q000523:psychology; D005149:Facial Expression; D005260:Female; D006801:Humans; D008065:Lipoid Proteinosis of Urbach and Wiethe / Q000175:diagnosis / Q000503:physiopathology* / Q000523:psychology
keywords: Urbach-Wiethe disease; amygdala; emotion recognition; emotional prosody; lipoid proteinosis; temporal lobe epilepsy