Structure and function in acquired prosopagnosia: Lessons from a series of 10 patients with brain damage

Acquired prosopagnosia varies in both behavioural manifestations and the location and extent of underlying lesions. We studied 10 patients with adult‐onset lesions on a battery of face‐processing tests. Using signal detection methods, we found that discriminative power for the familiarity of famous faces was most reduced by bilateral occipitotemporal lesions that involved the fusiform gyri, and better preserved with unilateral right‐sided lesions. Tests of perception of facial structural configuration showed severe deficits with lesions that included the right fusiform gyrus, whether unilateral or bilateral. This deficit was most consistent for eye configuration, with some patients performing normally for mouth configuration. Patients with anterior temporal lesions had better configuration perception, though at least one patient showed a more subtle failure to integrate configural data from different facial regions. Facial imagery, an index of facial memories, was severely impaired by bilateral lesions that included the right anterior temporal lobe and marginally impaired by fusiform lesions alone; unilateral right fusiform lesions tended to spare imagery for facial features. These findings suggest that (1) prosopagnosia is more severe with bilateral than unilateral lesions, indicating a minor contribution of the left hemisphere to face recognition, (2) perception of facial configuration critically involves the right fusiform gyrus and (3) access to facial memories is most disrupted by bilateral lesions that also include the right anterior temporal lobe. This supports assertions that more apperceptive variants of prosopagnosia are linked to fusiform damage, whereas more associative variants are linked to anterior temporal damage. Next, we found that behavioural indices of covert recognition correlated with measures of overt familiarity, consistent with theories that covert behaviour emerges from the output of damaged neural networks, rather than alternative pathways. Finally, to probe the face specificity of the prosopagnosic defect, we tested recognition of fruits and vegetables: While face specificity was not found in most of our patients, the data of one patient suggested that this may be possible with more focal lesions of the right fusiform gyrus.     

Voice recognition and the posterior cingulate: An fMRI study of prosopagnosia

Voices, in addition to faces, enable person identification. Voice recognition has been shown to evoke a distributed network of brain regions that includes, in addition to the superior temporal sulcus (STS), the anterior temporal pole, fusiform face area (FFA), and posterior cingulate gyrus (pCG). Here we report an individual (MS) with acquired prosopagnosia who, despite bilateral damage to much of this network, demonstrates the ability to distinguish voices of several well‐known acquaintances from voices of people that he has never heard before. Functional magnetic resonance imaging (fMRI) revealed that, relative to speech‐modulated noise, voices rated as familiar and unfamiliar by MS elicited enhanced haemodynamic activity in the left angular gyrus, left posterior STS, and posterior midline brain regions, including the retrosplenial cortex and the dorsal pCG. More interestingly, relative to noise and unfamiliar voices, the familiar voices elicited greater haemodynamic activity in the left angular gyrus and medial parietal regions including the dorsal pCG and precuneus. The findings are consistent with theories implicating the pCG in recognizing people who are personally familiar, and furthermore suggest that the pCG region of the voice identification network is able to make functional contributions to voice recognition even though other areas of the network, namely the anterior temporal poles, FFA, and the right parietal lobe, may be compromised.     

Seeing minds in others: Mind perception modulates low-level social-cognitive performance and relates to ventromedial prefrontal structures

In social interactions, we rely on nonverbal cues like gaze direction to understand the behavior of others. How we react to these cues is affected by whether they are believed to originate from an entity with a mind, capable of having internal states (i.e., mind perception). While prior work has established a set of neural regions linked to social-cognitive processes like mind perception, the degree to which activation within this network relates to performance in subsequent social-cognitive tasks remains unclear. In the current study, participants performed a mind perception task (i.e., judging the likelihood that faces, varying in physical human-likeness, have internal states) while event-related fMRI was collected. Afterwards, participants performed a social attention task outside the scanner, during which they were cued by the gaze of the same faces that they previously judged within the mind perception task. Parametric analyses of the fMRI data revealed that activity within ventromedial prefrontal cortex (vmPFC) was related to both mind ratings inside the scanner and gaze-cueing performance outside the scanner. In addition, other social brain regions were related to gaze-cueing performance, including frontal areas like the left insula, dorsolateral prefrontal cortex, and inferior frontal gyrus, as well as temporal areas like the left temporo-parietal junction and bilateral temporal gyri. The findings suggest that functions subserved by the vmPFC are relevant to both mind perception and social attention, implicating a role of vmPFC in the top-down modulation of low-level social-cognitive processes.     

Dissociation of the neural systems for working memory maintenance of verbal and nonspatial visual information

Working memory for names and faces was investigated to ascertain whether verbal and nonspatial visual information is maintained in working memory by separate neural systems. The subjects performed a delayed match-to-sample task for famous or unfamous faces and names and a sensorimotor control task. Several occipital, temporal, parietal, and prefrontal areas were activated during all memory delays, in comparison with the control delays. Greater delay activity for unfamous faces than for names was obtained in the right fusiform gyrus, right inferior frontal gyrus (IFG), right IFG/ precentral gyrus, and right medial superior frontal gyrus, whereas greater delay activity for unfamous names than for faces was observed in the precuneus, left insula/postcentral gyrus, and left IFG/ precentral gyrus. There was no significant difference in the prefrontal activity in the comparison between famous faces and names. Greater delay activity for famous names than for faces was obtained in visual association and parietal areas. The results indicate that there is a functional dissociation based on information type within the neural system that is responsible for working memory maintenance of verbal and nonspatial visual information.     

Atypical Neuropsychological Profile in a Boy with 22q11.2 Deletion Syndrome Keywords:

In this article the general and specific cognitive impairments of the boy R.H. with a de novo deletion 22q11.2 are described. His full-scale IQ was 73, and he obtained only slightly better verbal than non-verbal subtest scores. Neuropsychological assessment revealed specific impairments in perceptual categorization of objects presented suboptimal, matching of unfamiliar faces, and verbal learning and memory. In contrast, he performed in accordance with his intelligence level on other visual perceptual tasks, on non-verbal learning and memory tasks, and on attention tasks. Voxel-wise statistical comparison of a high-resolution T1-weighted magnetic resonance image of R.H’s brain with similar images obtained from 14 normal control children revealed as major abnormalities a reduction of the right inferior parietal and superior occipital lobe, and a bilateral reduction of deep white matter behind the inferior frontal gyrus. These cognitive impairments and MRI abnormalities are not commonly described in 22q11.2 Deletion Syndrome and may indicate a larger heterogeneity in the neurocognitive phenotype than currently evidenced. At least in this boy the microdeletion seems to have interfered with the development and functioning of particular neural subsystems, while the structure and functioning of other subsystems was left intact.     

Atypical neuropsychological profile in a boy with 22q11.2 Deletion Syndrome.

In this article the general and specific cognitive impairments of the boy R.H. with a de novo deletion 22q11.2 are described. His full-scale IQ was 73, and he obtained only slightly better verbal than non-verbal subtest scores. Neuropsychological assessment revealed specific impairments in perceptual categorization of objects presented suboptimal, matching of unfamiliar faces, and verbal learning and memory. In contrast, he performed in accordance with his intelligence level on other visual perceptual tasks, on non-verbal learning and memory tasks, and on attention tasks. Voxel-wise statistical comparison of a high-resolution T1-weighted magnetic resonance image of R.H's brain with similar images obtained from 14 normal control children revealed as major abnormalities a reduction of the right inferior parietal and superior occipital lobe, and a bilateral reduction of deep white matter behind the inferior frontal gyrus. These cognitive impairments and MRI abnormalities are not commonly described in 22q11.2 Deletion Syndrome and may indicate a larger heterogeneity in the neurocognitive phenotype than currently evidenced. At least in this boy the microdeletion seems to have interfered with the development and functioning of particular neural subsystems, while the structure and functioning of other subsystems was left intact.     

Haptic face identification activates ventral occipital and temporal areas: An fMRI study

Many studies in visual face recognition have supported a special role for the right fusiform gyrus. Despite the fact that faces can also be recognized haptically, little is known about the neural correlates of haptic face recognition. In the current fMRI study, neurologically intact participants were intensively trained to identify specific facemasks (molded from live faces) and specific control objects. When these stimuli were presented in the scanner, facemasks activated left fusiform and right hippocampal/parahippocampal areas (and other regions) more than control objects, whereas the latter produced no activity greater than the facemasks. We conclude that these ventral occipital and temporal areas may play an important role in the haptic identification of faces at the subordinate level. We further speculate that left fusiform gyrus may be recruited more for facemasks than for control objects because of the increased need for sequential processing by the haptic system.     

Haptic face identification activates ventral occipital and temporal areas: an fMRI study

Many studies in visual face recognition have supported a special role for the right fusiform gyrus. Despite the fact that faces can also be recognized haptically, little is known about the neural correlates of haptic face recognition. In the current fMRI study, neurologically intact participants were intensively trained to identify specific facemasks (molded from live faces) and specific control objects. When these stimuli were presented in the scanner, facemasks activated left fusiform and right hippocampal/parahippocampal areas (and other regions) more than control objects, whereas the latter produced no activity greater than the facemasks. We conclude that these ventral occipital and temporal areas may play an important role in the haptic identification of faces at the subordinate level. We further speculate that left fusiform gyrus may be recruited more for facemasks than for control objects because of the increased need for sequential processing by the haptic system.     

Self-construal priming modulates neural substrates of self-awareness

We used functional magnetic resonance imaging to assess whether self-construal priming can change adults' self-awareness induced during face perception. After reading essays containing independent or interdependent pronouns (e.g., I or we), participants were scanned while judging the head orientation of images showing their own and familiar faces. Neural activity in the right middle frontal cortex was greater when participants viewed their own rather than familiar faces, and this difference was larger after independent than after interdependent self-construal priming. The increased right frontal activity for participants' own faces relative to familiar faces was associated with faster responses. Our findings suggest that the neural correlates of self-awareness associated with recognition of one's own face can be modulated by self-construal priming in human adults.     

Direct comparison of episodic encoding and retrieval of words: an event-related fMRI study

Functional magnetic resonance imaging (fMRI) was used to compare directly episodic encoding and retrieval. During encoding, subjects studied visually presented words and reported via keypress whether each word represented a pleasant or unpleasant concept (intentional, deep encoding). During the retrieval phase, subjects indicated (via keypress) whether visually presented words had previously been studied. No reliable differences were found during the recognition phase for words that had been previously studied and those that had not been studied. Areas preferentially active during encoding (relative to retrieval) included left superior frontal cortex, medial frontal cortex, left superior temporal cortex, posterior cingulate, left parahippocampal gyrus, and left inferior frontal gyrus. Regions more active in retrieval than encoding included bilateral inferior parietal cortex, bilateral precuneus, right frontal polar cortex, right dorsolateral prefrontal cortex, and right inferior frontal/insular cortex.     

Direct Comparison of Episodic Encoding and Retrieval of Words: An Event-related fMRI Study

Functional magnetic resonance imaging (fMRI) was used to compare directly episodic encoding and retrieval. During encoding, subjects studied visually presented words and reported via keypress whether each word represented a pleasant or unpleasant concept (intentional, deep encoding). During the retrieval phase, subjects indicated (via keypress) whether visually presented words had previously been studied. No reliable differences were found during the recognition phase for words that had been previously studied and those that had not been studied. Areas preferentially active during encoding (relative to retrieval) included left superior frontal cortex, medial frontal cortex, left superior temporal cortex, posterior cingulate, left parahippocampal gyrus, and left inferior frontal gyrus. Regions more active in retrieval than encoding included bilateral inferior parietal cortex, bilateral precuneus, right frontal polar cortex, right dorsolateral prefrontal cortex, and right inferior frontal/insular cortex.     

Altered topological patterns of large-scale brain functional networks during passive hyperthermia

In this study, we simulated environmental heat exposure to 18 participants, and obtained functional magnetic resonance image (fMRI) data during resting state. Brain functional networks were constructed over a wide range of sparsity threshold according to a prior atlas dividing the whole cerebrum into 90 regions. Results of graph theoretical approaches showed that although brain networks in both normal and hyperthermia conditions exhibited economical small-world property, significant alterations in both global and nodal network metrics were demonstrated during hyperthermia. Specifically, a lower clustering coefficient, maintained shortest path length, a lower small-worldness, a lower mean local efficiency were found, indicating a tendency shift to a randomized network. Additionally, significant alterations in nodal efficiency were found in bilateral gyrus rectus, bilateral parahippocampal gyrus, bilateral insula, right caudate nucleus, bilateral putamen, left temporal pole of middle temporal gyrus, right inferior temporal gyrus. In consideration of physiological system changes, we found that the alterations of normalized clustering coefficient, small-worldness, mean normalized local efficiency were significantly correlated with the rectal temperature alteration, but failed to obtain significant correlations with the weight loss. More importantly, behavioral attention network test (ANT) after MRI scanning showed that the ANT effects were altered and correlated with the alterations of some global metrics (normalized shortest path length and normalized global efficiency) and prefrontal nodal efficiency (right dorsolateral superior frontal gyrus, right middle frontal gyrus and left orbital inferior frontal gyrus), implying behavioral deficits in executive control effects and maintained alerting and orienting effects during passive hyperthermia. The present study provided the first evidence for human brain functional disorder during passive hyperthermia according to graph theoretical analysis using resting-state fMRI.     

Behavioral and fMRI evidence that cognitive ability modulates the effect of semantic context on speech intelligibility

Text cues facilitate the perception of spoken sentences to which they are semantically related (Zekveld, Rudner, et al., 2011). In this study, semantically related and unrelated cues preceding sentences evoked more activation in middle temporal gyrus (MTG) and inferior frontal gyrus (IFG) than nonword cues, regardless of acoustic quality (speech in noise or speech in quiet). Larger verbal working memory (WM) capacity (reading span) was associated with greater intelligibility benefit obtained from related cues, with less speech-related activation in the left superior temporal gyrus and left anterior IFG, and with more activation in right medial frontal cortex for related versus unrelated cues. Better ability to comprehend masked text was associated with greater ability to disregard unrelated cues, and with more activation in left angular gyrus (AG). We conclude that individual differences in cognitive abilities are related to activation in a speech-sensitive network including left MTG, IFG and AG during cued speech perception.     

Early development of letter specialization in left fusiform is associated with better word reading and smaller fusiform face area

A functional region of left fusiform gyrus termed “the visual word form area” (VWFA) develops during reading acquisition to respond more strongly to printed words than to other visual stimuli. Here, we examined responses to letters among 5‐ and 6‐year‐old early kindergarten children (N = 48) with little or no school‐based reading instruction who varied in their reading ability. We used functional magnetic resonance imaging (fMRI) to measure responses to individual letters, false fonts, and faces in left and right fusiform gyri. We then evaluated whether signal change and size (spatial extent) of letter‐sensitive cortex (greater activation for letters versus faces) and letter‐specific cortex (greater activation for letters versus false fonts) in these regions related to (a) standardized measures of word‐reading ability and (b) signal change and size of face‐sensitive cortex (fusiform face area or FFA; greater activation for faces versus letters). Greater letter specificity, but not letter sensitivity, in left fusiform gyrus correlated positively with word reading scores. Across children, in the left fusiform gyrus, greater size of letter‐sensitive cortex correlated with lesser size of FFA. These findings are the first to suggest that in beginning readers, development of letter responsivity in left fusiform cortex is associated with both better reading ability and also a reduction of the size of left FFA that may result in right‐hemisphere dominance for face perception.     

Perceptual asymmetries in left- and right-handers for cartoon and real faces

We examined 40 left-handers and 40 right-handers on two free-vision tests of face processing. A chimeric face composed of a smiling half-face joined to either a neutral half-face (real faces) or a sad half-face (cartoon faces) and its mirror image were presented on each trial. Subjects judged which chimeric face looked happier, the one with the smile to the left or the one with the smile to the right. Right-handers, but not left-handers, had a highly significant leftward attentional bias, since chimeras with the smile to the left were judged happier than those with the smile to the right. The cartoon- and real-face tasks did not differ in the mean perceptual asymmetries they elicited. However, correlations between attentional-asymmetry scores for the two tasks, although high for both left- and right-handers, were significantly smaller than task reliabilities. Thus, the cartoon- and real-face tasks overlap to a major extent in the lateralized processes they measure, but to a lesser extent, they also index different lateralized processes.     

Perception of facial expression depends on prior attention

Attending versus ignoring a stimulus can later determine how it will be affectively evaluated. Here, we asked whether attentional states could also modulate subsequent sensitivity to facial expressions of emotion. In a dual-task procedure, participants first rapidly searched for a gender-defined face among two briefly displayed neutral faces. Then a test face with the previously attended or ignored face’s identity was presented, and participants judged whether it was emotionally expressive (happy, angry, or fearful) or neutral. Intensity of expression in the test face was varied so that an expression detection threshold could be determined. When fearful or angry expressions were judged, expression sensitivity was worse for faces bearing the same identity as a previously ignored versus attended face. When happy expressions were judged, sensitivity was unaffected by prior attention. These data support the notion that the motivational value of stimuli may be reduced by processes associated with selective ignoring.     

Neural dichotomy of word concreteness: a view from functional neuroimaging

Our perception about the representation and processing of concrete and abstract concepts is based on the fact that concrete words are highly imagined and remembered faster than abstract words. In order to explain the processing differences between abstract and concrete concepts, various theories have been proposed, yet there is no unanimous consensus about its neural implication. The present study investigated the processing of concrete and abstract words during an orthography judgment task (implicit semantic processing) using functional magnetic resonance imaging to validate the involvement of the neural regions. Relative to non-words, both abstract and concrete words show activation in the regions of bilateral hemisphere previously associated with semantic processing. The common areas (conjunction analyses) observed for abstract and concrete words are bilateral inferior frontal gyrus (BA 44/45), left superior parietal (BA 7), left fusiform gyrus and bilateral middle occipital. The additional areas for abstract words were noticed in bilateral superior temporal and bilateral middle temporal region, whereas no distinct region was noticed for concrete words. This suggests that words with abstract concepts recruit additional language regions in the brain.     

Perceptual functions in prosopagnosia

Some patients with prosopagnosia may have an apperceptive basis to their recognition defect. Perceptual abnormalities have been reported in single cases or small series, but the causal link of such deficits to prosopagnosia is unclear. Our goal was to identify candidate perceptual processes that might contribute to prosopagnosia, by subjecting several prosopagnosic patients to a battery of functions that may be necessary for accurate facial perception. We tested seven prosopagnosic patients. Three had unilateral right occipitotemporal lesions, two had bilateral posterior occipitotemporal lesions, and one had right anterior-to-occipital temporal damage along with a small left temporal lesion. These lesions all included the fusiform face area, in contrast to one patient with bilateral anterior temporal lesions. Most patients had impaired performance on face-matching tests and difficulty with subcategory judgments for non-face objects. The most consistent deficits in patients with lesions involving the fusiform face area were impaired perception of spatial relations in dot patterns and reduced contrast sensitivity in the 4 to 8 cycles deg(-1) range. Patients with bilateral lesions were impaired in saturation discrimination. Luminance discrimination was normal in all but two patients, and spatial resolution was uniformly spared. Curvature and line-orientation discrimination were impaired in only one patient, who also had the most difficulty with more basic-level object recognition. We conclude that deficits in luminance, spatial resolution, curvature, line orientation, and contrast at low spatial frequencies are unlikely to contribute to apperceptive prosopagnosia. More relevant may be contrast sensitivity at higher spatial frequencies and the analysis of object spatial structure. Deficits in these functions may impair perception of subtle variations in object shape, and may be one mechanism by which the recognition defect in prosopagnosia can extend to other classes of object subcategorization.     

文盲与非文盲汉字字形和语音加工的脑机制

利用功能性磁共振成像(fMRI)技术探讨文盲和非文盲汉字字形和语音加工脑机制的差异。实验1使用汉字字形和图形比较了中国人文盲和非文盲字形加工过程脑机制的左侧差异。实验2使用汉字语音和纯音比较了文盲和非文盲语音加工过程脑机制的双侧差异。结果表明文盲与非文盲汉字字形和语音加工脑机制不同,且非文盲的脑活动强。