Prosopagnosie et syndrome de Capgras : deux syndromes en miroir ?

Abstract

First described in the 19th century in patients with brain lesions, prosopagnosia was named in 1947 by Joachim Bodamer (1910–1985), a German neuropsychiatrist. The term refers to the inability to recognise a familiar face, a rarely isolated symptom. Prosopagnosia is thought to result from brain lesions, predominantly at the ventral right temporo-occipital junction. There are more common cases of developmental prosopagnosia, where no lesion has been identified. Capgras syndrome (CS), described by Joseph Capgras (1873–1950), a psychiatrist, consists of the persistent belief that a close relative, identified, has been replaced by a look-alike. SC, considered a delusional disorder of identification or familiarity, has been described in chronic psychoses, mainly schizophrenia. It is also reported in the course of neurodegenerative diseases (mainly Alzheimer's disease, Lewy body dementia, Parkinson's dementia). Lesion-based forms have recently been described. Prosopagnosia and SC were initially thought to result from mirror-image lesions involving a single factor, one affecting an overt ventral circuit for face recognition (in the case of prosopagnosia), the other a covert dorsal circuit involved in emotional and vegetative responses (in the case of CS). Lesion network mapping has been used in both lesion-related conditions and suggests a more complex figure. Lesions causing prosopagnosia were correlated with one of the main regions associated with face recognition, the right fusiform face area, and anticorrelated with left frontal regions possibly involved in visual attentional control. In lesional forms of CS or other delusional misidentification syndromes, there was a correlation with left retrosplenial cortex, an area associated with familiarity processing, and a correlation with right frontal regions associated with belief evaluation. This finding supports the intervention of two factors in CS, one generating the impaired familiarity associated with perception and the other the genesis and persistence of a delusional idea. The debate on the mechanisms of CS is not settled, especially as it is limited to its lesional forms, but studies using lesion network mapping already mark a more general paradigm shift, with attention shifting from lesions (clinico-anatomical method) to circuit dysfunction.