Evidence of taxonomy for Developmental Topographical Disorientation: Developmental Landmark Agnosia Case 1.

We report Developmental Landmark Agnosia (DLA) in a 6-year-old boy (L.G.) who was referred to us for congenital prosopagnosia (see Pizzamiglio et al., 2017 , in which both testing and rehabilitation of Congenital Prosopagnosia are reported). We investigated his performance using a neuropsychological battery and eye movement recordings. The assessment showed the presence of deficits in recognizing familiar places (along with Congenital Prosopagnosia), but not common objects. Eye movement recordings confirmed his problems in recognizing familiar landmarks and misrecognition of unfamiliar places. L.G. is the first evidence of a DLA, suggesting identification of taxonomy of navigational disorders in Developmental Topographical Disorientation is possible, as in the Acquired Topographical Disorientation.

Congenital prosopagnosia in a child: Neuropsychological assessment, eye movement recordings and training.

Here we report the assessment and treatment of a 6-year-old boy (L.G.) who was referred to us for congenital prosopagnosia (CP). We investigated his performance using a test battery and eye movement recordings pre- and post-training. L.G. showed deficits in recognising relatives and learning new faces, and misrecognition of unfamiliar people. Eye movement recordings showed that L.G. focused on the lower part of stimuli in naming tasks based on familiar or unfamiliar incomplete or complete faces. The training focused on improving his ability to explore internal features of faces, to discriminate specific facial features of familiar and unfamiliar faces, and to provide his family with strategies to use in the future. At the end of the training programme L.G. no longer failed to recognise close and distant relatives and classmates and did not falsely recognise unknown people.

Neural foundation of human moral reasoning: an ALE meta-analysis about the role of personal perspective.

Moral sense is defined as a feeling of the rightness or wrongness of an action that knowingly causes harm to people other than the agent. The large amount of data collected over the past decade allows drawing some definite conclusions about the neurobiological foundations of moral reasoning as well as a systematic investigation of methodological variables during fMRI studies. Here, we verified the existence of converging and consistent evidence in the current literature by means of a meta-analysis of fMRI studies of moral reasoning, using activation likelihood estimation meta-analysis. We also tested for a possible neural segregation as function of the perspective used during moral reasoning i.e., first or third person perspectives. Results demonstrate the existence of a wide network of areas underpinning moral reasoning, including orbitofrontal cortex, insula, amygdala, anterior cingulate cortex as well as precuneus and posterior cingulate cortex. Within this network we found a neural segregation as a function of the personal perspective, with 1PP eliciting higher activation in the bilateral insula and superior temporal gyrus as well as in the anterior cingulate cortex, lingual and fusiform gyri, middle temporal gyrus and precentral gyrus in the left hemisphere, and 3PP eliciting higher activation in the bilateral amygdala, the posterior cingulate cortex, insula and supramarginal gyrus in the left hemisphere as well as the medial and ventromedial prefrontal cortex in the right hemisphere. These results shed some more light on the contribution of these areas to moral reasoning, strongly supporting a functional specialization as a function of the perspective used during moral reasoning.

Is the patient able to watch TV or read the newspaper? A functional semi-structured scale to observe Hemineglect symptoms in Activities of Daily Living (H-ADL).

We developed a functional semi-structured scale to observe Hemineglect symptoms in Activities of Daily Living (H-ADL). The scale could assist clinicians in assessing rehabilitation priorities aimed at correcting any persisting errors or omissions. In addition, the scale could also be used by caregivers to observe patients' progress and improve their participation. Two groups of right brain-damaged patients (25 with hemineglect; 27 without hemineglect) were tested twice: at admission and before discharge from hospital. A control group of healthy individuals matched to patients for age and education and patients' caregivers also participated. Two raters (A; B), experts in neuropsychology, observed patients and healthy individuals using the H-ADL. We found that the H-ADL final scores correlated with the standard hemineglect tests. The three groups differed in performance and differences also emerged between the first and the second assessment, suggesting an improvement due to the remission of hemineglect as a consequence of the treatment. Raters A and B did not differ in their observations, but there were some discrepancies with caregivers' observations. Therefore, although caregivers could help clinicians in detecting persistent hemineglect behaviour, the assessment should be performed by experts in neuropsychology.

Where does brain neural activation in aesthetic responses to visual art occur? Meta-analytic evidence from neuroimaging studies.

Here we aimed at finding the neural correlates of the general aspect of visual aesthetic experience (VAE) and those more strictly correlated with the content of the artworks. We applied a general activation likelihood estimation (ALE) meta-analysis to 47 fMRI experiments described in 14 published studies. We also performed four separate ALE analyses in order to identify the neural substrates of reactions to specific categories of artworks, namely portraits, representation of real-world-visual-scenes, abstract paintings, and body sculptures. The general ALE revealed that VAE relies on a bilateral network of areas, and the individual ALE analyses revealed different maximal activation for the artworks' categories as function of their content. Specifically, different content-dependent areas of the ventral visual stream are involved in VAE, but a few additional brain areas are involved as well. Thus, aesthetic-related neural responses to art recruit widely distributed networks in both hemispheres including content-dependent brain areas of the ventral visual stream. Together, the results suggest that aesthetic responses are not independent of sensory, perceptual, and cognitive processes.

Age effect in generating mental images of buildings but not common objects.

Imagining a familiar environment is different from imagining an environmental map and clinical evidence demonstrated the existence of double dissociations in brain-damaged patients due to the contents of mental images. Here, we assessed a large sample of young and old participants by considering their ability to generate different kinds of mental images, namely, buildings or common objects. As buildings are environmental stimuli that have an important role in human navigation, we expected that elderly participants would have greater difficulty in generating images of buildings than common objects. We found that young and older participants differed in generating both buildings and common objects. For young participants there were no differences between buildings and common objects, but older participants found easier to generate common objects than buildings. Buildings are a special type of visual stimuli because in urban environments they are commonly used as landmarks for navigational purposes. Considering that topographical orientation is one of the abilities mostly affected in normal and pathological aging, the present data throw some light on the impaired processes underlying human navigation.

A dedicated system for topographical working memory: evidence from domain-specific interference tests.

In the present study, we used single- and dual-task conditions to investigate the nature of topographical working memory to better understand what type of task can hamper performance during navigation. During dual-task conditions, we considered four different sources of interference: motor (M), spatial motor (SM), verbal (i.e. articulatory suppression AS) and spatial environmental (SE). In order to assess the nature of topographical working memory, we used the Walking Corsi Test, asking the participants to perform two tasks simultaneously (M, SM, AS and SE). Our results showed that only spatial-environmental interference hampers the execution of a topographical working memory task, suggesting a task-domain-specific effect. We also found general gender differences in the topographical working memory capabilities: men were more proficient than women, regardless of the type of interferences. However, like men, women performed worse when a spatial-environmental interference was present.

Peculiar body representation alterations in hemineglect: a case report.

We report the case of FP affected by personal and extrapersonal neglect and a body representation deficit characterized by delusional ideas. When FP performed the human figure, he placed body parts to the left, despite his extrapersonal neglect. Differently, when he performed the car figure, he placed all parts to the right, in line with his deficit. Comparing FP with a small patient group with the same clinical features without delusional ideas about body emerged that he was the only one to suffer from a specific body representation deficit characterized by a lack of body ownership sense.

One's own country and familiar places in the mind's eye: different topological representations for navigational and non-navigational contents.

Visual mental imagery is a process that draws on different cognitive abilities and is affected by the contents of mental images. Several studies have demonstrated that different brain areas subtend the mental imagery of navigational and non-navigational contents. Here, we set out to determine whether there are distinct representations for navigational and geographical images. Specifically, we used a Spatial Compatibility Task (SCT) to assess the mental representation of a familiar navigational space (the campus), a familiar geographical space (the map of Italy) and familiar objects (the clock). Twenty-one participants judged whether the vertical or the horizontal arrangement of items was correct. We found that distinct representational strategies were preferred to solve different categories on the SCT, namely, the horizontal perspective for the campus and the vertical perspective for the clock and the map of Italy. Furthermore, we found significant effects due to individual differences in the vividness of mental images and in preferences for verbal versus visual strategies, which selectively affect the contents of mental images. Our results suggest that imagining a familiar navigational space is somewhat different from imagining a familiar geographical space.

A selective egocentric topographical working memory deficit in the early stages of Alzheimer's disease: a preliminary study.

The aim of this study was to determine whether an egocentric topographical working memory (WM) deficit is present in the early stages of Alzheimer's disease (AD) with respect to other forms of visuospatial WM. Further, we would investigate whether this deficit could be present in patients having AD without topographical disorientation (TD) signs in everyday life assessed through an informal interview to caregivers. Seven patients with AD and 20 healthy participants performed the Walking Corsi Test and the Corsi Block-Tapping Test. The former test requires memorizing a sequence of places by following a path and the latter is a well-known visuospatial memory task. Patients with AD also performed a verbal WM test to exclude the presence of general WM impairments. Preliminary results suggest that egocentric topographical WM is selectively impaired, with respect to visuospatial and verbal WM, even without TD suggesting an important role of this memory in the early stages of AD.

Spatial location and pathway memory compared in the reaching vs. walking domains.

Spatial information processing is influenced by the space in which an individual acts and the nature of the stimulus. This distinction is also present in spatial memory, where stimuli are processed differently because of their nature and the space in which they are released. The aim of the present study was to compare college students' performance on spatial location and pathway memory tasks in two different domains (reaching and walking). Reaching space refers to the portion of space within "grasping distance" and walking space to that beyond arm's reach. Research results indicate that it is easier to remember a pathway in the walking than the reaching domain and to remember single spatial locations in the reaching domain. Women are more able to perform the task in the walking domain than the reaching domain and men perform equally well in both domains.

The Walking Corsi Test (WalCT): a normative study of topographical working memory in a sample of 4- to 11-year-olds.

We report normative data on topographical working memory collected through the Walking Corsi Test (WalCT; Piccardi et al., 2008 ) for developing a standard administration procedure to be used in clinical and educational practice. A total of 268 typically developing Italian children aged 4-11 years performed both WalCT and Corsi Block-Tapping Test (CBT; Corsi, 1972 ) a well-known visuo-spatial memory test. WalCT has already been validated in adults, demonstrating sensitivity in detecting topographical memory deficits even in individuals who have no other memory impairments. Our results showed that age, but not sex, affected performances. Both girls and boys had a larger span on the CBT than the WalCT. The youngest group did not differ in performing WalCT and CBT, but from 5.6 years of age children performed better on CBT than WalCT, suggesting that memory in reaching space develops before topographical memory. Only after 5 years of age do children learn to process specifically topographical stimuli, suggesting that this happens when their environmental knowledge becomes operational and they increase environmental independence. We also discuss the importance to introduce WalCT in the clinical assessment.

The Walking Corsi Test (WalCT): standardization of the topographical memory test in an Italian population.

Selective visuo-spatial memory deficits can seriously affect many aspects of daily life; for example, an individual may not remember where he put an object or which path he took to reach his destination. In general, visuo-spatial memory is assessed through pen-and-paper tests that mainly assess memory components in peripersonal space. Recent studies (Piccardi et al. in Exp Brain Res 206:171-177, 2010; Piccardi et al. in Neuropsychol Dev Cogn B Aging Neuropsychol Cogn 18:362-384, 2011) have shown that brain-damaged patients selectively fail on navigation memory tasks but not on other tests of visuo-spatial memory ability. These findings underline the need for a standardized test that measures memory in navigation separately from other types of visuo-spatial memory. Here, we report the validation of the Walking Corsi Test (WalCT: Piccardi et al. in Neurosci Lett 432:127-131, 2008) on 289 individuals aged 15-86 years. The WalCT is a new instrument that assesses topographical memory in real environments and reproduces on a large-scale version the Corsi Block-Tapping Test (CBT: Corsi in Unpublished doctoral dissertation, McGill University, Montreal, 1972). The WalCT has been used in clinical practice and has proven sensitive in detecting navigational memory deficits even in individuals who have no other memory impairments (Piccardi et al. in Exp Brain Res 206:171-177, 2010; Piccardi et al. in Neuropsychol Dev Cogn B Aging Neuropsychol Cogn 18:362-384, 2011; Bianchini et al. in Neuropsychologia 48:1563-1573, 2010 ).

Familiarity and environmental representations of a city: a self-report study.

"Sense of direction" is usually assessed by self-report. Several internal factors contribute to proficiency in navigation: spatial cognitive style, respondent's sex, and familiarity with the environment; however, questionnaires assessing sense of direction do not include all these factors. In a recent study, Nori and Piccardi reported that environmental familiarity was crucial for topographical orientation. Regardless of a person's spatial cognitive style (i.e., landmark, route, or survey), the greater their familiarity with the environment, the better their performance. In this study, a questionnaire was used, the Familiarity and Spatial Cognitive Style Scale, to measure 208 women's sense of direction and knowledge of their city of residence. Analysis showed that Spatial Cognitive Style predicted sense of direction but not town knowledge. By contrast, familiarity played a crucial role in both areas, confirming the importance of having a tool to assess this factor.

Mirror writing resulting from an egocentric representation disorder: a case report.

Buchwald (1878 ) used the term 'mirror writing' to indicate writing in the reverse direction to what is normal in a particular language and in which the individual letters are also reversed. Cases of healthy individuals (i.e., Leonardo da Vinci and Lewis Carroll) as well as brain-damaged patients have been described in the literature. Here, we report the case of PM, a 70-year-old right-handed woman who showed right hemiplegia and mirror writing following a stroke in the left lenticular nucleus and internal capsulae. PM underwent a complete neuropsychological evaluation, which included copying, dictation and spontaneous writing in both hemispaces with both hands. She was also tested for topographical disorientation, visuo-spatial disorders and body schema deficits. We observed isolated mirror writing only when PM wrote with the left hand, without differences between hemispaces. She also showed a left-right disorientation, a body topological map disorder and an egocentric misrepresentation. The presence of mirror writing not confined to one hemispace and the co-presence of executive function disorders, as well as anosognosia, suggests damage to our patient's sub-cortical frontal network. As no previous interpretation fits with PM's symptoms, we hypothesize that mirror writing resulted from damage to her egocentric frame of reference. This hypothesis allows us to interpret the patient's array of disorders, including mirror writing, body topological map disorder, left-right confusion and egocentric representation.

Dissociated deficits of visuo-spatial memory in near space and navigational space: evidence from brain-damaged patients and healthy older participants.

Defects confined to spatial memory can severely affect a variety of daily life activities, such as remembering the location of objects or navigating the environment, until now the skills involved have been mostly assessed with regard to the visual domain using traditional pencil and paper tests. Our aim was to test the efficacy of a recently developed psychometric instrument (Walking Corsi Test: WalCT) to assess the specific contribution of spatial memory to the complex task of retrieving route knowledge. The WalCT is a 3 × 2.5-m version of the well-known Corsi Block-tapping Test (CBT), in which patients are required to memorize (and replicate) a sequence of body displacements. We assessed the ability of left and right brain-damaged patients, as well as healthy young and senior controls, to perform both the CBT and the WalCT. Results showed differences related to age in the healthy individuals and specific functional dissociations in the brain-damaged patients. The double dissociations found in this study demonstrate the importance of having a task able to detect navigational disorders, because virtual reality tasks are often much too difficult for aged brain-damaged patients to perform.

Different spatial memory systems are involved in small- and large-scale environments: evidence from patients with temporal lobe epilepsy.

Recent reports show that humans and animals do not acquire information about routes and object locations in the same way. In spatial memory, a specific sub-system is hypothesized to be involved in encoding, storing and recalling navigational information, and it is segregated from the sub-system devoted to small-scale environment. We assessed this hypothesis in a sample of patients treated surgically for intractable temporal lobe epilepsy. We found double dissociations between learning and recall of spatial positions in large space versus small space. These results strongly support the hypothesis that two segregate systems process navigational memory for large-scale environments and spatial memory in small-scale environments.

Developmental topographical disorientation in a healthy subject.

We present the case of F.G., a healthy, normally developed 22-year-old male subject affected by a pervasive disorder in environmental orientation and navigation who presents no history of neurological or psychiatric disease. A neuro-radiological examination showed no evidence of anatomical or structural alterations to the brain. We submitted the subject for a comprehensive neuropsychological assessment of the different cognitive processes involved in topographical orientation to evaluate his ability to navigate the spatial environment. The results confirmed a severe developmental topographical disorder and deficits in a number of specific cognitive processes directly or indirectly involved in navigation. The results are discussed with reference to the sole previously described case of developmental topographical disorientation (Pt1; Iaria et al., 2009). F.G. differs from the former case due to the following: the greater severity of his disorder, his complete lack of navigational skills, the failure to develop compensatory strategies, and the presence of a specific deficit in processing the spatial relationships between the parts of a whole. The present case not only confirms the existence of developmental topographical-skill disorders, but also sheds light on the architecture of topographical processes and their development in human beings.

Pure representational neglect and navigational deficits in a case with preserved visuo-spatial working memory.

We describe a patient who, after right hemisphere damage, showed severe, persistent, pure representational neglect but no evidence of perceptual neglect and no deficits in spatial working memory when evaluated with a traditional clinical test (Corsi Block Tapping test). This finding provides evidence against a full explanation of representational neglect within the context of visuo-spatial working memory. Indeed, this patient showed a peculiar deficit in navigational tasks requiring re-orientation in a novel environment by means of his mental representation of the environment. Since no representational neglect was observed in tests requiring mental representation of single or multiple objects (i.e., o'clock test) we suggest that in our patient representational neglect is caused by damage to the cognitive system involved in coding and storing environmental information to be used during navigation.

Landmark based navigation in brain-damaged patients with neglect.

We tested navigational abilities of brain-damaged patients suffering from representational or perceptual neglect asking them to retrieve a location according to salient spatial cues included in a rectangular empty room. Both groups of patients showed difficulties in learning the spatial definition of the target location in relation to two landmarks. However in a delayed attempt performed after several trials the group of patients with perceptual neglect proved able to easily retrieve the target location. In this condition they performed as controls showing a spared ability to navigate according to a stable representation of the room in long-term memory. In contrast the difficulty of patients with representational neglect remained unchanged across experimental conditions. At variance with clinical assessment, in which patients show asymmetrical performances in describing a well-known environment from memory, this latter result depicts a behavioural counterpart of the disorder, namely the inability to orient in a new environment according to an inner representation. Data are further discussed in order to provide a description of the cognitive mechanisms required for space representation for navigation.