ABSTRACTXenomelia is a rare condition characterized by a persistent and intense desire for amputation of one or more healthy limbs. Some frequent clinical manifestations suggest the involvement of distinct neural substrates. Specifically, recent aetiopathological hypotheses about xenomelia propose a neurodevelopmental origin, highlighting the putative contribution of the right parietal lobe and right insula, known to subserve the construction of a coherent representation of the body as a whole. This literature review is aimed at analysing relevant findings about structural and functional brain correlates of xenomelia, focusing on the identification of key regions and their hemispheric distribution. Finally, implications about the potential link between xenomelia and phylogenetic development of the right parietal lobe are discussed. Despite a certain degree of heterogeneity and the spatial extension of networks involved, signs of partial right-sided lateralization of cortical nodes and left-sided lateralization of subcortical nodes emerged. Indeed, some areas-rsPL, riPL, PMC and rInsula-have been consistently found altered in xenomelia. In conclusion, the presence of both structural and functional multi-layered brain abnormalities in xenomelia suggests a multifactorial aetiology; however, as the prevalence of correlational studies, causal relationships remain to be investigated.
Keywords: Xenomelia; amputation desire; body ownership; multi-sensory integration; right parietal lobe.