Perhaps the most intriguing disorders of body representation are those that are not due to primary disease of brain tissue. Strange and sometimes painful phantom limb sensations can result from loss of afference to the brain; and Complex Regional Pain Syndrome (CRPS)—the subject of the current report—can follow limb trauma without pathology of either the central or peripheral nervous system. This enigmatic and vexing condition follows relatively minor trauma, and can result in enduring misery and a useless limb. It manifests as severe pain, autonomic dysfunction, motor disability and ‘neglect-like’ symptoms with distorted body representation. For this special issue on body representation we describe the case of a patient suffering from CRPS, including symptoms suggesting a distorted representation of the affected limb. We report contrasting effects of mirror box therapy, as well as a new treatment—prism adaptation therapy—that provided sustained pain relief and reduced disability. The benefits were contingent upon adapting with the affected limb. Other novel observations suggest that: (1) pain may be a consequence, not the cause, of a disturbance of body representation that gives rise to the syndrome; (2) immobilisation, not pain, may precipitate this reorganisation of somatomotor circuits in susceptible individuals; and (3) limitation of voluntary movement is neither due to pain nor to weakness but, rather, to derangement of body representation which renders certain postures from the repertoire of hand movements inaccessible.
It has been suggested that some cortically blind patients can process the emotional valence of visual stimuli via a fast, subcortical pathway from the superior colliculus (SC) that reaches the amygdala via the pulvinar. We provide in vivo evidence for connectivity between the SC and the amygdala via the pulvinar in both humans and rhesus macaques. Probabilistic diffusion tensor imaging tractography revealed a streamlined path that passes dorsolaterally through the pulvinar before arcing rostrally to traverse above the temporal horn of the lateral ventricle and connect to the lateral amygdala. To obviate artifactual connectivity with crossing fibers of the stria terminalis, the stria was also dissected. The putative streamline between the SC and amygdala traverses above the temporal horn dorsal to the stria terminalis and is positioned medial to it in humans and lateral to it in monkeys. The topography of the streamline was examined in relation to lesion anatomy in five patients who had previously participated in behavioral experiments studying the processing of emotionally valenced visual stimuli. The pulvinar lesion interrupted the streamline in two patients who had exhibited contralesional processing deficits and spared the streamline in three patients who had no deficit. Although not definitive, this evidence supports the existence of a subcortical pathway linking the SC with the amygdala in primates. It also provides a necessary bridge between behavioral data obtained in future studies of neurological patients, and any forthcoming evidence from more invasive techniques, such as anatomical tracing studies and electrophysiological investigations only possible in nonhuman species.
See Legrain (doi:10.1093/awx188) for a scientific commentary on this article. Some patients with complex regional pain syndrome report that movements of the affected limb are slow, more effortful, and lack automaticity. These symptoms have been likened to the syndrome that sometimes follows brain injury called hemispatial neglect, in which patients exhibit attentional impairments and problems with movements affecting the contralesional side of the body and space. Psychophysical testing of patients with complex regional pain syndrome has found evidence for spatial biases when judging visual targets distanced at 2 m, but not in directions that indicate reduced attention to the affected side. In contrast, when judging visual or tactile stimuli presented on their own body surface, or pictures of hands and feet within arm's reach, patients with complex regional pain syndrome exhibited a bias away from the affected side. What is not yet known is whether patients with complex regional pain syndrome only have biased attention for bodily-specific information in the space within arm's reach, or whether they also show a bias for information that is not associated with the body, suggesting a more generalized attention deficit. Using a temporal order judgement task, we found that patients with complex regional pain syndrome processed visual stimuli more slowly on the affected side (relative to the unaffected side) when the lights were projected onto a blank surface (i.e. when no bodily information was visible), and when the lights were projected onto the dorsal surfaces of their uncrossed hands. However, with the arms crossed (such that the left and right lights projected onto the right and left hands, respectively), patients' responses were no different than controls. These results provide the first demonstration of a generalized attention bias away from the affected side of space in complex regional pain syndrome patients that is not specifically related to bodily information. They also suggest a separate and additional bias of visual attention away from the affected hand. The strength of attention bias was predicted by scores on a self-report measure of body perception distortion; but not by pain intensity, time since diagnosis, or affected body side (left or right). At an individual level, those patients whose upper limbs were most affected had a higher incidence of inattention than those whose lower limbs were most affected. However, at a group level, affected limb (upper or lower) did not predict bias magnitude; nor did three measures designed to assess possible asymmetries in the distribution of movements across space. It is concluded that inattention in near space in complex regional pain syndrome may arise in parallel with a distorted perception of the body.10.1093/brain/awx152_video1awx152media15495542665001.
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