Giuseppe d'Orsi1, Paolo Tinuper. 1. Epilepsy Center, Neurological Unit, Casa Divina Provvidenza, Opera Don Uva, Bisceglie (Bari), Italy. giudorsi@yahoo.it
Abstract
PURPOSE: Some consider the "voices" of Joan of Arc to have been ecstatic epileptic auras, such as Dostoevsky's epilepsy. We performed a critical analysis of this hypothesis and suggest that the "voices" may be the expression of an epileptic syndrome recently described: idiopathic partial epilepsy with auditory features (IPEAF). METHODS: Joan's symptoms were obtained from the documentation of her Trial of Condemnation. We investigated Joan of Arc from a strictly semiologic point of view, focusing on symptoms and possible trigger factors. RESULTS: From ages 13 to 19, the episodes were characterized by a prevalent auditory component, followed by "a great light" or images that Joan identified as saints. Sometimes, the visual component was missing and replaced by comprehension verbal disturbance. The spells were sudden, brief in duration, and frequent, and also occurred during sleep. In some cases, the sound of bells could trigger the "voices." CONCLUSIONS: Joan's spells were characterized by a constant auditory component, complex, spontaneous, or evoked by sudden auditory stimuli, that could be associated with an inconstant visual component, sometimes simple and, more often, complex, and comprehension verbal disturbance. These spells differ from ecstatic epilepsy with respect to clinical features and involvement of cerebral regions. The negative family history, the ictal semiology, and the possibility that the spells were triggered by acoustic stimuli suggest IPEAF, and the search for the epitempin/LGI1 gene or other new gene mutations on a hair of the Maid of Orléans may enhance our knowledge about her presumed epilepsy.
PURPOSE: Some consider the "voices" of Joan of Arc to have been ecstatic epileptic auras, such as Dostoevsky's epilepsy. We performed a critical analysis of this hypothesis and suggest that the "voices" may be the expression of an epileptic syndrome recently described: idiopathic partial epilepsy with auditory features (IPEAF). METHODS: Joan's symptoms were obtained from the documentation of her Trial of Condemnation. We investigated Joan of Arc from a strictly semiologic point of view, focusing on symptoms and possible trigger factors. RESULTS: From ages 13 to 19, the episodes were characterized by a prevalent auditory component, followed by "a great light" or images that Joan identified as saints. Sometimes, the visual component was missing and replaced by comprehension verbal disturbance. The spells were sudden, brief in duration, and frequent, and also occurred during sleep. In some cases, the sound of bells could trigger the "voices." CONCLUSIONS: Joan's spells were characterized by a constant auditory component, complex, spontaneous, or evoked by sudden auditory stimuli, that could be associated with an inconstant visual component, sometimes simple and, more often, complex, and comprehension verbal disturbance. These spells differ from ecstatic epilepsy with respect to clinical features and involvement of cerebral regions. The negative family history, the ictal semiology, and the possibility that the spells were triggered by acoustic stimuli suggest IPEAF, and the search for the epitempin/LGI1 gene or other new gene mutations on a hair of the Maid of Orléans may enhance our knowledge about her presumed epilepsy.