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Reduced empathy is a common symptom in frontotemporal dementia (FTD). Although empathy deficits have been extensively researched in sporadic cases, few studies have explored the differences in familial forms of FTD.

Functional neuroimaging and patient studies demonstrated significant involvement of ventral area V4α, located in the anterior ventral pathway, in color vision. A low number of case studies reported lesions in close vicinity to this region leading to symptoms of hemiachromatopsia indicating hemifield-specific processing of color information. With the present study, we present the first group study investigating hemiachromatopsia after injury to anterior ventral brain areas. In lateral stimulus presentations with several color perception tasks, we observed symptoms of hemiachromatopsia, which were specific to patients with unilateral lesions to the ventral pathway. Particularly, we identified unilateral lesions to area V4α as an important contribution to color perception deficits under demanding viewing conditions. Our results suggest that color information processed along the anterior ventral path is hemifield-specific and that selective deficits in color perception cannot be fully compensated by the intact contralesional visual stream.

Line Bisection is a simple task frequently used in stroke patients to diagnose disorders of spatial perception characterized by a directional bisection bias to the ipsilesional side. However, previous anatomical and behavioural findings are contradictory, and the diagnostic validity of the line bisection task has been challenged. We hereby aimed to re-analyse the anatomical basis of pathological line bisection by using multivariate lesion-symptom mapping and disconnection-symptom mapping based on support vector regression in a sample of 163 right hemispheric acute stroke patients. In line with some previous studies, we observed that pathological line bisection was related to more than a single focal lesion location. Cortical damage primarily to right parietal areas, particularly the inferior parietal lobe, including the angular gyrus, as well as damage to the right basal ganglia contributed to the pathology. In contrast to some previous studies, an involvement of frontal cortical brain areas in the line bisection task was not observed. Subcortically, damage to the right superior longitudinal fasciculus (I, II and III) and arcuate fasciculus as well as the internal capsule was associated with line bisection errors. Moreover, white matter damage of interhemispheric fibre bundles, such as the anterior commissure and posterior parts of the corpus callosum projecting into the left hemisphere, was predictive of pathological deviation in the line bisection task.

Neurological patients with apraxia of pantomime provide us with a unique opportunity to study the neural correlates of higher-order motor function. Previous studies using lesion-behaviour mapping methods led to inconsistent anatomical results, reporting various lesion locations to induce this symptom. We hypothesised that the inconsistencies might arise from limitations of mass-univariate lesion-behaviour mapping approaches if our ability to pantomime the use of objects is organised in a brain network. Thus, we investigated apraxia of pantomime by using multivariate lesion behaviour mapping based both on support vector regression and sparse canonical correlations in a sample of 130 left-hemisphere stroke patients. Both multivariate methods identified multiple areas to underlie high-order motor control, including inferior parietal lobule, precentral gyrus, posterior parts of middle temporal cortex, and insula. Further, long association fibres were affected, such as the superior longitudinal fascicle, inferior occipito-frontal fascicle, uncinated fascicle, and superior occipito-frontal fascicle. The findings underline the benefits of multivariate lesion-behaviour mapping in brain networks and provide new insights into the brain networks underlying higher-order motor control.

Pantomime of tool use is typically affected in neurological patients with apraxia, while at the same time these patients are able to perform the use of the actual tool with less or no errors. This discrepancy is commonly explained by differences in afferent input, in particular a lack of visual online feedback from the object in pantomime. The present study investigated the role of visual feedback in apraxia of pantomime by testing neurological patients with apraxia and healthy controls in a task requiring the pantomime of tool use as well as real tool use. Visual feedback was systematically removed at different phases of the action using shutter glasses that were controlled online based on real-time motion-capturing. Data analyses revealed more errors in pantomime than in real tool use. These differences were similar in patients as well as in controls. Removal of visual feedback did not affect apractic errors specifically; it neither increased patients' apractic errors during pantomime of tool use nor transformed the patients' normal movements with a real tool into movements with apractic errors. Our findings contradict the hypothesis that apraxia patients pathologically over-rely on visual feedback. Instead, we propose that pantomime of tool use requires cognitive processes that are not necessary for real tool use and independent of visual online feedback.

Numerous studies have reported that temporal order perception is biased in neurological patients with extinction and neglect. These individuals tend to perceive two objectively simultaneous stimuli as occurring asynchronously, with the ipsilesional item being perceived as appearing prior to the contralesional item. Likewise, they report that two stimuli occurred simultaneously in situations where the contralesional item is presented substantially prior to the ipsilesional item. Therefore, they exhibit a biased point of subjective simultaneity (PSS). Here we demonstrate that the magnitude of this effect is modulated by the relative position of the stimuli with respect to the patient's trunk. This effect was only observed in patients who still exhibited neglect symptoms, and neither the pathological bias nor substantial modulation were observed in individuals who had recovered from neglect, those who never had neglect or neurologically healthy controls. Crucially, our design kept the retinal and head-centered coordinates of these stimuli constant, providing a pure measure for the influence of egocentric trunk position. This finding emphasizes the interaction of egocentric spatial position on the temporal symptoms observed in these individuals.

Electrophysiological monkey and human neuroimaging studies have reported a lateralization of signal processing in object perception. However, it is unclear whether these results point to a unique topographically organized signal processing in either hemisphere, or if these results represent a rather negligible spatial organization of otherwise redundant object perception systems in both hemispheres. We tested a group of 10 patients with lesions to ventral object processing regions and spared primary visual functions with lateral presentations of different categories of object stimuli. Object perception in the contralesional visual field was impaired while object perception on the ipsilesional hemifield was intact. These results demonstrate that the object perception system needs two intact ventral pathways for unimpaired object perception across the whole visual field; the loss of one system cannot be fully compensated by its contralateral homolog or spared parts of the lesioned ventral stream.

Optic ataxia (OA) is a neurological disorder that is characterised by misreaching to targets in the visual periphery. The anatomy of OA thus provides important information for the neural representation of visually guided reaching in humans. In 2005 a lesion mapping analysis of OA localised the critical lesion site at the parieto-occipital junction (POJ) (Karnath & Perenin, 2005). This work was accompanied by the discovery of a peripheral reaching module at the POJ in an fMRI study (Prado et al., 2005). The ostensible overlap between the territory typically affected in patients with OA and the findings of Prado et al. (2005) had a tremendous influence on the search for a cortical peripheral reaching module. However, a close inspection of the functional Magnetic Resonance Imaging (fMRI) study revealed that a comparison between reaching towards visible targets in the peripheral visual field and reaching to visible targets in the central visual field – which is the key aspect in clinical examinations of OA – was not conducted. Moreover, whereas main effects of reaching overlapped with the OA lesion site, specific interaction effects did not overlap. We performed a direct comparison between reaching to visible peripheral targets and reaching to visible central targets to address the inconsistencies between the aforementioned studies. Our analysis shows that Prado et al.'s study cannot be taken as evidence for a delineated module for peripheral reaching. In contrast to Prado et al. we found a combined system of POJ, IPS and SPL areas − the posterior human 7A, mIPS, V6A and the posterior IPS – with increased signals during reaching to peripheral targets.

In patients with left-sided lesions, anosognosia for hemiparesis (AHP) seems to be a rare phenomenon. It has been discussed whether this rareness might be due to an inevitable bias due to language dysfunction and whether the left hemisphere's role for our self-awareness of motor actions thus is underestimated. By applying functional magnetic resonance imaging (fMRI) we examined whether patients with AHP following a left hemisphere stroke show a regular, left-sided or a reversed, right-sided lateralization of language functions. Only the former observation would argue for an original role of the left hemisphere in self-awareness about limb function. In a consecutive series of 44 acute left-sided stroke patients, only one patient (=2%) was identified showing AHP. In this case, we could verify by using fMRI that lateralization of AHP and spatial neglect on the one hand and of language functions on the other hand were reversed. The present single case observation thus argues against an original role of the left hemisphere in self-awareness about limb function. We discuss the data in the context of previous observations in the literature.

The brain transforms sensory input to motor coordinates to accommodate for changes of posture and gaze direction. Neurophysiological and neuropsychological evidence supports the existence of multiple representations of space. A debated issue regards whether objects that we see are encoded in egocentric frames only or also maintain an object-centered frame of reference. Previous clinical studies were unable to discriminate between these models as the stimuli used to determine object-based (allocentric) biases are contaminated by body-centered (egocentric) effects. To address this, we used stimuli where allocentric side was elicited by gestalt configuration rather than egocentric location. We then displayed these stimuli at different egocentric positions allowing us to independently measure the effects of allocentric position, egocentric position as well as their interaction. In a group of stroke patients with neglect we demonstrate that allocentric biases are modulated as a function of egocentric position. These findings help adjudicate between the different models of space representation, demonstrating that specific allocentric deficits not only exist but also often co-exist with egocentric biases.

Visual extinction is a common consequence of brain injury where individuals fail to detect a contralesional target when it is presented with a competing ipsilesional target. This disorder is often seen as either a consequence of biased competitive interactions or as a consequence of an attentional disengagement deficit. A study of neurological patients by Rorden et al. (2008)found that extinction is maximal at stimulus simultaneity when the target stimuli straddle the central gaze fixation location, while it is maximal when the ipsilesional stimulus has a temporal lead on the contralesional stimulus when both target stimuli are presented in the ipsilesional visual field. The authors argued that these results are most parsimoniously explained as a consequence of low-level biased competitive interactions due to cortical magnification, but acknowledged that an attentional disengagement deficit could also accommodate the results. The present study set out to adjudicate between these models by examining the performance of neurologically healthy subjects who exhibit normal cortical magnification but do not have pathological attentional biases. We presented (a)synchronous double stimulation trials where both targets to be identified could straddle the central gaze fixation location or be presented in either the left or the right visual field. We found that in both the left and the right visual field target performance accuracy was poorest when the more peripheral stimulus led. This suggests that the findings from Rorden et al. can indeed be explained by a low-level physiological bias due to cortical magnification and argues against the notion that neurological extinction represents an attentional disengagement deficit.

Perception of verticality can be perturbed after cortical stroke. However, a relationship between lesion location and pathologic perception of verticality is still a matter of debate since previous studies revealed contradictory results. Thus, the aim of the current study was to test whether specific cortical lesions were associated with tilts of subjective visual vertical (SVV) and to determine the critical brain areas that cause such tilts in the case of a lesion.

Sex differences exist in the structural organization of the human brain and are related to cognitive abilities. Females usually outperform men in verbal fluency, verbal memory, perceptual speed, numerical calculation, and fine motor skills, whereas males are superior in visuospatial abilities, throwing accuracy, and mathematical reasoning. Here we demonstrated a male advantage in spatial abilities for the auditory modality. We employed a sound localization task based on the so-called “cocktail party situation”, requiring extraction of auditory information of a specific sound source when multiple competing sound sources were present. The results indicated better performance of males than females for localizing target sounds in a multi-source sound environment. This finding suggests a sex difference in the attentional mechanisms extracting spatial information of one acoustic event of interest from an auditory scene composed of multiple sound sources. It seems that the known male superiority in spatial abilities may be supramodal, rather than a specificity of the visual modality.

The perception of global scenes and objects consisting of multiple constituents is based on the integration of local elements or features. Gestalt grouping cues, such as proximity or similarity, can aid this process. Using functional MRI we investigated whether grouping guided by different gestalt cues rely on distinct networks in the brain or share a common network. Our study revealed that gestalt grouping involved the inferior parietal cortex, middle temporal gyrus and prefrontal cortex irrespective of the specific cue used. These findings agree with observations in neurological patients, which suggest that inferior parietal regions may aid the integration of local features into a global gestalt. Damage to this region results in simultanagnosia, a deficit in perceiving multiple objects and global scenes.

The phenomenon of spatial neglect after right brain damage greatly helps our understanding of the normal mechanisms of directing and maintaining spatial attention, of spatial orientation, and the characteristics of neural representation of space. The intriguing symptom is a spontaneous orientation bias towards the right leading to neglect of objects or persons on the left. Interestingly, we observe similar symptoms namely a spontaneous bias of eyes and head along the horizontal dimension of space in patients with unilateral vestibular dysfunction. Further similarities concern anatomical findings. Both spatial neglect and vestibular processing at cortical level show dominance in the right hemisphere and involve common brain areas. Lesion studies in human and monkey, electrical and transcranial magnetic stimulation, as well as functional imaging results have revealed the superior temporal cortex, insula and the temporo-parietal junction to be substantial parts of the multisensory (vestibular) system as well as to be affected in spatial neglect. We argue that these structures are not strictly ‘vestibular’ but rather have a multimodal character representing a significant site for the neural transformation of converging vestibular, auditory, neck proprioceptive and visual input into higher order spatial representations. Neurons of these regions provide us with redundant information about the position and motion of our body in space. They seem to play an essential role in adjusting body position relative to external space. This view may initiate further development of those strategies to treat spatial neglect that use routes to rehabilitation based on specific manipulations of sensory input feeding into this system.

Perfusion-weighted imaging (PWI) is used to identify brain regions that are receiving enough blood supply to remain structurally intact, but not enough to function normally. Previous observations suggest that spatial neglect due to subcortical stroke can be explained by dysfunction of cortical areas rather than through the neuronal loss in the subcortical structures itself. The present study aimed to identify the dysfunctional cortical regions induced by basal ganglia stroke in patients with spatial neglect. In a patient group with stroke lesions centring on the basal ganglia, we examined the common area(s) of structurally intact but dysfunctional cortical tissue by using spatial normalization of PWI maps as well as symmetric voxel-wise inter-hemispheric comparisons. These new techniques allow comparison of the structurally intact but abnormally perfused areas of different individuals in the same stereotaxic space, and at the same time avoid problems due to regional perfusion differences and to possible observer-dependent biases. We found that strokes centring on the right basal ganglia which provoke spatial neglect induce abnormal perfusion in a circumscribed area of intact cortex that typically involves those three regions that have previously been described to provoke spatial neglect when damaged directly by cortical infarction: the superior temporal gyrus, the inferior parietal lobule and the inferior frontal gyrus. The data suggest that spatial neglect following a right basal ganglia lesion typically is caused by the dysfunction of (part of) these specific cortical areas.

The NOA‐03 trial explored high‐dose methotrexate alone in 37 patients with primary central nervous system lymphoma. The overall median survival was 25 months. After 4 years, the rate of leukoencephalopathy in patients surviving more than 12 months was 58% with and 10% without whole‐brain radiotherapy given at relapse (p= 0.11). Attention deficits were found in all six tested patients, and memory deficits in four patients. Two patients had normal, three had moderately restricted, and one had markedly restricted quality of life. Thus, high‐dose methotrexate with deferred radiotherapy had only moderate efficacy and was associated with significant neurotoxicity in long‐term surviving patients. Ann Neurol 2005;57:843–847

Recent findings argue for a pathway in humans for sensing the orientation of gravity and controlling upright body posture, separate from the one for orientation perception of the visual world. Stroke patients with contraversive pushing were shown to experience their body as oriented upright when actually tilted about 20° to the ipsilesional side, in spite of normal visual-vestibular functioning. A recent study suggested the involvement of posterolateral thalamus typically associated with the disorder.

Um Handbewegungen präzise auf visuelle Ziele zu führen, müssen räumliche Informationen verschiedener Bezugssysteme integriert werden. Die Untersuchung der Verhaltensdefizite von Patienten mit Hirnschädigungen, die Untersuchungen der Hirnaktivität von Gesunden und neurophysiologische Untersuchungen an Affen ermöglichen uns einen Einblick in die Verknüpfung von Sensorik und Motorik für die Ausführung zielgerichteter Bewegungen. Bei Patienten mit parietalen Läsionen kann ein spezifisches Defizit visuell geführter Bewegungen beobachtet werden. Zeige- und Greifbewegungen auf Ziele, die in der visuellen Peripherie lokalisiert sind, weichen erheblich vom Ziel ab. Dieses Fehlverhalten wurde als „Optische Ataxie“ bezeichnet. Die perzeptive Einschätzung von Objektgrößen und das Erkennen von Gegenständen ist dagegen bei Patienten beeinträchtigt, die nach Läsionen inferior temporaler Hirnregionen eine „Visuelle Formagnosie“ aufweisen. Zusammen mit tierexperimentellen Befunden haben diese Beobachtungen wesentlich zu der Vorstellung beigetragen, dass unser Gehirn visuelle Informationen in zwei getrennten Verarbeitungswegen für motorische und perzeptive Prozesse aufbereitet. Ein dorsaler visueller Pfad soll ausgehend vom occipitalen Kortex über posteriore parietale Areale eine handlungsspezifische Informationsverarbeitung leisten, während ein ventraler occipito-temporaler Pfad Informationen für die bewusste Wahrnehmung und Erinnerung verarbeitet. Jüngere Untersuchungen geben Anlass zu einer Neubewertung dieses Modells. Sie zeigen, dass die streng dichotome Vorstellung der visuellen Informationsverarbeitung vermutlich nicht zutreffend ist.

“Pusher syndrome” is a clinical disorder following left or right brain damage in which patients actively push away from the nonhemiparetic side, leading to a loss of postural balance. The mechanism underlying this disorder and its related anatomy have only recently been identified. Investigation of patients with severe pushing behavior has shown that perception of body posture in relation to gravity is altered. The patients experience their body as oriented “upright” when the body actually is tilted to the side of the brain lesion (to the ipsilesional side). In contrast, patients with pusher syndrome show no disturbed processing of visual and vestibular inputs determining visual vertical. These new insights have allowed the authors to suggest a new physical therapy approach for patients with pusher syndrome where the visual control of vertical upright orientation, which is undisturbed in these patients, is the central element of intervention.

Neuroimaging studies investigated the attentional systems of the human brain revealing two networks, one for voluntary allocation of attention and another for stimulus-driven attentional processes. Whereas lesions of the latter system were supposed to lead to spatial neglect, we show that such lesions rather are typical for the occurrence of visual extinction. Extinction describes the inability of brain-damaged patients to detect a contralesional target in the presence of a competing ipsilesional stimulus. In a sample of consecutively admitted patients with right hemisphere stroke, we found dissociable cortical substrates for spatial neglect and visual extinction. There was a surprising congruency between the typical lesion site in patients with extinction and the activation clusters found in previous neuroimaging studies of healthy subjects. The results show that the temporo-parietal junction (TPJ), considered to be a crucial part of the stimulus-driven attentional network, is the neural substrate of visual extinction.

Various studies have documented that right hemispheric lesions restricted to the basal ganglia or to the thalamus may evoke spatial neglect. However, for methodological reasons, the exact anatomical correlate of spatial neglect within these two subcortical structures still remained uncertain. The present study identified these locations by comparing the anatomy of subcortical lesions to the basal ganglia or thalamus between neglect and control patients. Analysis revealed that the putamen, the pulvinar and, to a smaller degree, the caudate nucleus are the subcortical structures typically associated with spatial neglect in humans. All these structures have direct anatomical connections to the superior temporal gyrus (STG), which recently has been identified as the neural correlate of spatial neglect in the human cortex. Therefore, it is assumed that the right putamen, caudate nucleus, pulvinar and STG form a coherent corticosubcortical anatomical network in the genesis of spatial neglect in humans.

Various studies have documented that right hemispheric lesions restricted to the basal ganglia or to the thalamus may evoke spatial neglect. However, for methodological reasons, the exact anatomical correlate of spatial neglect within these two subcortical structures still remained uncertain. The present study identified these locations by comparing the anatomy of subcortical lesions to the basal ganglia or thalamus between neglect and control patients. Analysis revealed that the putamen, the pulvinar and, to a smaller degree, the caudate nucleus are the subcortical structures typically associated with spatial neglect in humans. All these structures have direct anatomical connections to the superior temporal gyrus (STG), which recently has been identified as the neural correlate of spatial neglect in the human cortex. Therefore, it is assumed that the right putamen, caudate nucleus, pulvinar and STG form a coherent corticosubcortical anatomical network in the genesis of spatial neglect in humans.

Zusammenfassung:Zur Diagnose eines Neglects werden im klinischen Alltag häufig Durchstreichaufgaben oder das Linienhalbieren angewendet. Die vorliegende Arbeit gibt einen Überblick darüber, wie treffsicher diese Verfahren einen Patienten mit Neglect identifizieren können. Es zeigt sich, daß die Durchstreichaufgaben, insbesondere der “Letter cancellation Test” und der “Bells Test”, die höchsten Trefferquoten aufweisen. Mit der Linienhalbierungsaufgabe werden hingegen ca. ein Drittel der Patienten mit Neglect fälschlicherweise nichtdiagnostiziert. Darüber hinaus sind Defizite im Linienhalbieren nicht spezifisch für Patienten mit Neglect. Auch andere Patientengruppen, wie etwa Patienten mit Hemianopsie, zeigen Abweichungen bei dieser Aufgabe. Demgegenüber weisen bei Durchstreichaufgaben allein Patienten mit einem Neglect lateralisierte Defizite auf. Wir schlußfolgern, daß beide Aufgabentypen nicht gleichermaßen für die Diagnose einer Neglect-Symptomatik geeignet sind. Im Gegensatz zu den Vernachlässigungen bei Durchstreichaufgaben erlaubt. Das Abweichen bzw. Nicht-Abweichen beim Linienhalbieren erlaubt keinen sicheren Schluß auf das Vorliegen bzw. Nicht-Vorliegen eines Neglects.

We investigated the effect of vestibular stimulation on the lateralization of dichotic sound by cold-water irrigation of the external auditory canal in human subjects. Subjects adjusted the interaural level difference of the auditory stimulus to the subjective median plane of the head. In those subjects in whom dizziness and nystagmus indicated sufficient vestibular stimulation, these adjustments were significantly shifted toward the cooled ear compared with the control condition (irrigation with water at body temperature); i.e., vestibular stimulation induced a shift of the sound image toward the nonstimulated side. The mean magnitude of the shift was 7.3 dB immediately after vestibular stimulation and decreased to 2.5 dB after 5 min. As shown by an additional control experiment, this effect cannot be attributed to a unilateral hearing loss induced by cooling of the auditory periphery. The results indicate the involvement of vestibular afferent information in the perception of sound location during movements of the head and/or the whole body. We thus hypothesize that vestibular information is used by central-nervous mechanisms generating a world-centered representation of auditory space.

Abstract:  It has been suggested that the movement impairments experienced by patients with neglect are not restricted to spatial disorders, but also affect higher-order kinematics (velocity and acceleration) to the extent that movements towards the neglected side are slower than movements away from it. In a recent study, we could not confirm this hypothesis, but found that patients with unilateral neglect exhibited no distinct direction-specific deficits in hand velocity when performing goal-directed reaching movements. Here we investigated whether neglect patients might reveal direction-specific deficits during exploratory hand movements. Six patients with left-sided neglect and six age-matched healthy control subjects scanned with their right hands the surface of a large table searching for a (non-existent) tactile target. Movements were performed in darkness. Time-position data of the hand were recorded with an optoelectronic camera system. Median activity of the patients’ exploratory hand movements was shifted to the right (Karnath and Perenin 1998). Hand trajectories were partitioned into sections of leftward/rightward or, along the sagittal plane, into sections of near/far movements. For each movement section average and peak velocities were computed. The patients’ hand movements were bradykinetic when compared with the control group. However, we found no evidence that average or peak velocities of leftward intervals were systematically lower than during rightward motion. Direction-specific deficits in velocity were also not observed for movements to and away from the body (sagittal plane). In conclusion, we found evidence for general bradykinesia in neglect patients but not for a direction-specific deficit in the control of hand velocity during exploratory hand movements.

Abstract:  The effect of transcutaneous vibration of the posterior neck muscles on the lateralization of dichotic sound was investigated in human subjects. Two-alternative forced-choice (left/right) judgements were made on acoustic stimuli presented with different interaural level differences via headphones during neck-muscle vibration. A shift of the subjective auditory median plane toward the side contralateral of vibration was found, indicating that the sound was perceived as shifted toward the side of vibration. The mean magnitude of the vibration-induced intracranial shift was 1.5 dB. The results demonstrate a neck-proprioceptive influence on sound lateralization and suggest that this proprioceptive input is used for a central-nervous transformation of auditory spatial coordinates onto a body-centered frame of reference.

RECENT studies investigated the pattern of visual exploration along the horizontal axis in patients with neglect. They found a deviated distribution of activity with the centre shifted −150° to the right of the body's mid-sagittal plane. The present study reports comparable observations concerning tactile exploration of space in neglect. We found the whole distribution of patients' exploratory hand movements shifted towards the right. The median activity lay 17.7 cm right of the body's mid-sagittal plane. The frequency of tactile exploration decreased towards the periphery of peripersonal space on the right and the left side. The findings are in clear contrast to the predictions of the attentional gradient model of neglect. They rather favour a deviation of the whole frame for exploratory behaviour towards a new equilibrium on the right. The character of this frame appears to be supramodal in that it determines the distribution of exploratory movements irrespective of whether the subject explores the surround visually or tactually.

Karnath (1988) and Gainotti, D'Erme and De Bonis (1989). suggested that the typical and severe neglect symptomology that is seen predominantly after lesions of the right hemisphere, is due to the combined effect of at least two different components: (1) an imbalance of lateral orienting tendencies with spontaneously primary orienting towards the ipsilesional side; together with (2) a deficit in orienting attention (from this primary focus), as well as eye or limb movements, to the contralesional side of the sagittal midplane.

Damage to the human parietal cortex leads to disturbances of spatial perception and of motor behaviour. Within the parietal lobe, lesions of the superior and of the inferior lobule induce quite different, characteristic deficits. Patients with inferior (predominantly right) parietal–lobe lesions fail to explore the contralesional part of space by eye or limb movements (spatial neglect). In contrast, superior parietal lobe lesions lead to specific impairments of goal–directed movements (optic ataxia). The observations reported in this paper support the view of dissociated functions represented in the inferior and the superior lobule of the human parietal cortex. They suggest that a spatial reference frame for exploratory behaviour is disturbed in patients with neglect. Data from these patients’ visual search argue that their failure to explore the contralesional side is due to a disturbed input transformation leading to a deviation of egocentric space representation to the ipsilesional side. Data further show that this deviation follows a rotation around the earth–vertical body axis to the ipsilesional side rather than a translation towards that side. The results are in clear contrast to explanations that assume a lateral gradient ranging from a minimum of exploration in the extreme contralesional to a maximum in the extreme ipsilesional hemispace. Moreover, the failure to orient towards and to explore the contralesional part of space appears to be distinct from those deficits observed once an object of interest has been located and releases reaching. Although patients with neglect exhibit a severe bias of exploratory movements, their hand trajectories to targets in peripersonal space may follow a straight path. This result suggests that (i) exploratory and (ii) goal–directed behaviour in space do not share the same neural control mechanisms. Neural representation of space in the inferior parietal lobule seems to serve as a matrix for spatial exploration and for orienting in space but not for visuomotor processes involved in reaching for objects. Disturbances of such processes rather appear to be prominent in patients with more superior parietal lobe lesions and optic ataxia.

Four neglect patients without visual field defects, one with a lesion of the right basal ganglia and three with a right, predominantly parietal lesion, were examined with a cancellation and a copying task before, during and after neck muscle vibration, during transcutaneous electrical stimulation of neck muscles and during vibration of hand muscles on the left side. In all patients, neck muscle vibration improved task performance, while transcutaneous electrical stimulation and hand vibration had little or no effect. The present results demonstrate that the effect of neck muscle vibration cannot be explained as arousal and activation due to unspecific sensory stimulation on the contralesional side of the body. They rather argue for the assumption that the compensatory effect of neck muscle vibration on neglect is an effect induced by the predominant activation of afferent Ia nerve fibres and their specific contribution to the central representation of egocentric space.

Do patients with unilateral neglect exhibit direction-specific deficits in the control of movement velocity when performing goal-directed arm movements? Five patients with left-sided neglect performed unrestrained three-dimensional pointing movements to visual targets presented at body midline, the left and right hemispace. A group of healthy adults and a group of patients with right-hemispheric brain damage but no neglect served as controls. Pointing was performed under normal room light or in darkness. Time-position data of the hand were recorded with an opto-electronic camera system. We found that compared to healthy controls, movement times were longer in both patient groups due to prolonged acceleration and deceleration phases. Tangential peak hand velocity was lower in both patient groups, but not significantly different from controls. Single peak, bell-shaped velocity profiles of the hand were preserved in all right hemispheric patients and in three out of five neglect patients. Most important, the velocity profiles of neglect patients to leftward targets did not differ significantly from those to targets in the right hemispace. In summary, we found evidence for general bradykinesia in neglect patients, but not for a direction-specific deficit in the control of hand velocity. We conclude that visual neglect induces characteristic changes in exploratory behavior, but not in the kinematics of goal-directed movements to objects in peripersonal space.

Eye movements of neglect patients with right parietal lesions were recorded during ocular searching for a (non-existent) target in complete darkness. With respect to the objective orientation of the sagittal midplane, ocular exploration was biased toward the ipsilesional side. However, in relation to the patients' subjective localization of the sagittal midplane in space, exploratory eye movements were symmetrically distributed to the subjective “left” and “right” as observed in non-brain-damaged controls. The present results further support the hypothesis that the essential aspect leading to spatial neglect is a disturbance of those cortical structures that are crucial for computing egocentric, body-centred coordinates that allow use to determine our body position in space and that are necessary for visuomotor coordination and exploration of space. In neglect patients the central coordinate transformation seems to work with a systematic error resulting in a deviation of the spatial reference frame to the ipsilesional side. Consequences of this deviation are a displacement of subjective localization of body orientation and—to the same degree—of the spatial area in which motor behavior (here exploratory eye movements) is executed.

Hemiparetic stroke patients with disturbed awareness for their motor weakness (anosognosia for hemiparesis/-plegia AHP) may exhibit further abnormal attitudes toward or perceptions of the affected limb(s). The present study investigated the clinical relationship and the anatomy of such abnormal attitudes and AHP.

Generally it is accepted that spatial neglect occurs predominantly after stroke of the human right hemisphere. In contrast, it remained controversial whether extinction follows the same hemispheric asymmetry. The opinion prevails that the laterality of visual extinction is not as pronounced as it is for spatial neglect.

Abstract: Two different models have argued that neglect of contralateral stimuli following brain damage might be associated with either a compressed or an anisometric neural representation of space along the earth-horizontal axis. We tested these models by determining neglect patients’ perception of spatial distances in the horizontal plane. We found no evidence for any compression or expansion or for anisometry along the earth-horizontal axis. The findings argue against a distortion of subjective space along the horizontal axis in patients with neglect, which could account for their failure to orient towards and to explore the contralesional parts of space.

In patients with spatial neglect, contralesional reflexive saccades toward suddenly appearing targets show direction-specific deficits. We examined whether these deficits also occur during free exploration of space. Neglect patients’ voluntary eye movements showed reduced amplitudes for saccades in all directions but no direction-specific deficit. The results argue against an interpretation of spatial neglect as a general deficit to disengage attention or to program saccades in contralesional direction.

Three patients with a right, predominantly parietal lesion and marked left-sided neglect without visual field defects were asked to direct a laser point to the position which they felt to lie exactly ‘straight ahead’ of their bodies orientation. Whereas in both light and darkness, the subjective body orientation was close to the objective body position in the control groups, the three neglect patients localized the body’s sagittal midplane-15° to the right of the objective orientation. No relevant differences of ‘straight ahead’ were found between the neglect patients and controls in the vertical plane. The neglect patients horizontal displacement of sagittal midplane to the right could be compensated for either by neck muscle vibration or by caloric vestibular stimulation on the left side. When vestibular stimulation was combined with neck muscle vibration, the horizontal deviation linearly combined by adding or neutralizing the effects observed when both types of stimulation were applied exclusively in the control groups as well as in the neglect patients. Moreover, data analysis revealed that the neglect patients ipsilesionally displaced subjective body orientation does not result from a disturbed primary perception or disturbed transmission of the vestibular or proprioceptive input from the periphery. The present results support the hypothesis that the essential aspect leading to neglect in brain-damaged patients is a disturbance of those cortical structures that are crucial for transforming the sensory input coordinates from the peripheral sensory organs—here the retina, neck muscle spindles and cupulae—into an egocentric, body-centred coordinate frame of reference. In neglect patients the coordinate transformation seems to work with a systematic error and deviation of the spatial reference frame to the ipsilesional side leading to a corresponding displacement of subjective localization of body orientation. It can be concluded further that neck muscle proprioception and vestibular stimulation directly interact in contributing to the subjects mental representation of space. The data suggest that the afferent information from these different input channels is used simultanenously for computing egocentric, body-centred coordinates that allow us to determine our body position in space.