Visuoperceptual Deficits Research Articles

Peer Support for Caregivers of People Living with Posterior Cortical Atrophy in Melbourne, Australia: A Feasibility Study.

Posterior Cortical Atrophy (PCA) is a rare form of young-onset dementia that causes early visuospatial and visuoperceptual deficits. The symptom profile of Posterior Cortical Atrophy leads to very specific care needs for those affected, who often rely on informal caregivers (including friends and family). Rare dementia support groups can be useful for both patients and their caregivers to assist with knowledge sharing, psychoeducation, and the provision of psychosocial support. Despite this, few such support groups exist. The purpose of this study was to examine a PCA support group for caregivers of individuals living with PCA. We held a structured psychoeducation support group comprised of four sessions with the aim being to provide education, strategies for the management of the disease, and peer support. Caregivers' mental health and quality of life were assessed. The results of our study showed that support group participation was a positive experience and assisted with increasing the knowledge of caregivers and fostering social connections. We suggest that peer support groups may be beneficial for both people living with PCA and their caregivers. We recommend that future quantitative and qualitative research is conducted to further assess health-promotion benefits to people living with PCA and their caregivers, and to assess their development and implementation in different contexts.

Pareidolias are a function of visuoperceptual impairment.

Pareidolias, or the misperception of ambiguous stimuli as meaningful objects, are complex visual illusions thought to be phenomenologically similar to Visual Hallucination (VH). VH are a major predictor of dementia in Parkinson's Disease (PD) and are included as a core clinical feature in Dementia with Lewy Bodies (DLB). A newly developed Noise Pareidolia Test (NPT) was proposed as a possible surrogate marker for VH in DLB patients as increased pareidolic responses correlated with informant-corroborated accounts of VH. This association could, however, be mediated by visuoperceptual impairment. To understand the drivers of performance on the NPT, we contrasted performances in patient groups that varied both in terms of visuoperceptual ability and rates of VH. N = 43 patients were studied of whom n = 13 had DLB or PD with Dementia (PDD); n = 13 had PD; n = 12 had typical, memory-onset Alzheimer's Disease (tAD); and n = 5 had Posterior Cortical Atrophy (PCA) due to Alzheimer's disease. All patient groups reported pareidolias. Within the Lewy body disorders (PD, DLB, PDD), there was no significant difference in pareidolic response rates between hallucinating and non-hallucinating patients. Visuoperceptual deficits and pareidolic responses were most frequent in the PCA group-none of whom reported VH. Regression analyses in the entire patient cohort indicated that pareidolias were strongly predicted by visuoperceptual impairment but not by the presence of VH. These findings suggest that pareidolias reflect the underlying visuoperceptual impairment of Lewy body disorders, rather than being a direct marker for VH.

Visual mental imagery in Lewy Body Dementia patients with visual hallucinations: preliminary results

AbstractBackgroundLewy body dementia (LBD) is characterized by the presence of visual hallucinations (VH), however mechanisms underpinning these phenomena are still unknown. Visual mental imagery (VMI) is defined as the ability to see with the mind’s eye, in the absence of adequate sensory input (Kosslyn, 1987). Hallucinations are defined as percepts in the absence of an appropriate stimulus from the external world. Both visual mental images and VH consist of a “stimulus‐independent” image production, however the association between VH and VMI has not yet been investigated in LBD. The aim of the study was to investigate VMI processes in patients LBD with VH.MethodNineteen LBD patients (mean age = 75.4, SD = 6.9; mean education = 10.2, SD = 4) attending the “Sapienza” University Hospital in Rome and 13 Healthy controls (HC) (mean age = 71.9,DS = 7.9; mean education = 12.8, SD = 4.9) matched for age and education were enrolled. VMI was assessed in patients and HC by means of an adapted version of The Complete Visual Mental Imagery Battery (CVMIB; Palermo et al., 2016), including tasks probing the generation of different visual mental images (i.e. object; building and face), maintenance, inspection and transformation processes, and through the O’Clock test (Grossi et al.,1994) including a perceptual and imaginative tasks.ResultNon‐parametric analyses revealed that LBD performed worse than HC in all CVMIB tasks. Furthermore LBD performed worse than HC in both O’clock tests, imaginative and perceptual ones, suggesting a role of the visuoperceptual deficits in VMI alterations. It is worth noting LBD and HC did not differ in response time.ConclusionLBD patients with VH show alterations of VMI compared to HC. Alterations of VMI process might influence VH occurrence. Thus, it is possible that VH and VMI share neural mechanisms and are placed on a continuum along the perceptual processes. Future studies are needed to disentangle the possible role of perceptual difficulties in VMI alterations in LBD and to clarify how VMI alterations are related to VH occurrence. Furthermore, investigating VMI and its relationship with visuoperceptual skills in LBD patients could help to better characterize their neuropsychological profile.

Posterior Cortical Atrophy: Primary Occipital Variant

AbstractBackgroundPosterior Cortical Atrophy (PCA) is one of the atypical Alzheimer’s Disease (AD) variants, characterized by predominant visuospatial and visuoperceptual deficits, with dorsal and ventral subtypes recognized clinically and radiographically. A third primary occipital (caudal) variant has been suggested. The objective of this study was to determine its demographics, clinical manifestations, and biomarker findings.MethodFifty‐two PCA patients with positive amyloid PET scans were investigated. Patients underwent neuropsychological assessment, 3T MRI imaging and FDG‐, amyloid‐ and tau‐PET scans. Regional FDG‐PET values were normalized to the pons and represented as z‐scores relative to a control population. Patients were divided into “primary occipital” and “other PCA” subgroups according to FDG‐PET defined criteria, with primary occipital defined as patients in which the z‐scores for occipital subregions were at least one standard deviation lower (i.e., more abnormal) than the z scores in all other brain regions. Global amyloid‐PET, temporoparietal FDG‐PET and temporal tau‐PET regions‐of‐interest (ROI) were calculated. Student’s T test and Fisher’s exact analyses were used to investigate differences in demographics, clinical findings, and imaging biomarkers across the two groups.ResultNine patients were classified as primary occipital; primary occipital patients were older (64.8±6.9 versus 59.3±6.8, p=0.034) and had more years of education (17.7±1.7 versus 15.1±2.6, p=0.007) compared to the other PCA patients. Clinical evaluation revealed worse performance for the primary occipital group on the Ishihara test for color perception (p<0.001), while the other PCA patients performed worse on the Western Aphasia Battery (WAB) praxis scale (p=0.005). Overall neuropsychiatric symptom burden as defined by the NPI‐Q was lower in the primary occipital group (p<0.001). The FDG‐PET meta‐ROI (weighted median uptake in angular gyrus, posterior cingulate, and inferior temporal ROIs, normalized to the pons and vermis) was higher in the primary occipital subtype (p=0.006), but no differences were observed in amyloid and tau‐PET.ConclusionClinical and biomarker findings suggest primary occipital PCA is characterized by an older age at onset, more color perception dysfunction, less severe ideomotor apraxia and less hypometabolism in temporal‐parietal meta‐ROI compared to more established phenotypes.

Suspected dementia, the eye and the ‘mind’s eye’: How primary care eye specialists could support early identification of Alzheimer’s Disease and its variants

The current article outlines the nature of visuoperceptual deficits in Alzheimer’s Disease (AD) and its ‘atypical’ variants. Evidence to highlight that visual and visuoperceptual difficulties are common in dementias driven by Alzheimer’s-based pathology will be discussed. Following this, the ways in which early identification of visual and lower-level visuoperceptual symptoms of dementia may be facilitated within primary care optical health settings will be considered. It is hoped that this may inspire work to support opticians and optometrists to identify where timely signposting to dementia services may be beneficial for people with suspected dementia who are experiencing visuoperceptual deficits. This is particularly important given that visual and visuoperceptual deficits can be early signs of dementia and often have a significant impact on activities of daily living (e.g. dressing, increased falls risk, accidents whilst driving, difficulties navigating familiar environments; Tippett et al., 2019). An illustrative case example is provided, outlining how timely detection of suspected dementia could have enabled early provision of appropriate treatment and support to enhance quality of life (e.g. medication for symptom management, provision of cognitive strategies and functional aids; Armstrong & Kergoat, 2015).

Steady-state visual evoked potentials in children with neurofibromatosis type 1: associations with behavioral rating scales and impact of psychostimulant medication

BackgroundNeurofibromatosis type 1 (NF1) is a genetic disorder often associated with cognitive dysfunctions, including a high occurrence of deficits in visuoperceptual skills. The neural underpinnings of these visuoperceptual deficits are not fully understood. We used steady-state visual evoked potentials (SSVEPs) to investigate possible alterations in the synchronization of neural activity in the occipital cortex of children with NF1.MethodsSSVEPs were measured using electroencephalography and compared between children with NF1 (n = 28) and neurotypical controls (n = 28) aged between 4 and 13 years old. SSVEPs were recorded during visual stimulation with coloured icons flickering at three different frequencies (6 Hz, 10 Hz, and 15 Hz) and analyzed in terms of signal-to-noise ratios. A mixed design ANCOVA was performed to compare SSVEP responses between groups at the three stimulation frequencies. Pearson’s correlations with levels of intellectual functioning as well as with symptoms of ADHD, ASD and emotional/behavioral problems were performed. The impact of psychostimulant medication on the SSVEP responses was analyzed in a subset of the NF1 group (n = 8) with paired t-tests.ResultsWe observed reduced signal-to-noise ratios of the SSVEP responses in children with NF1. The SSVEP responses were negatively correlated with symptoms of inattention and with symptoms of emotional/behavioral problems in the NF1 group. The SSVEP response generated by the lowest stimulation frequency (i.e., 6 Hz) was rescued with the intake of psychostimulant medication.ConclusionsImpaired processing of rhythmic visual stimulation was evidenced in children with NF1 through measures of SSVEP responses. Those responses seem to be more reduced in children with NF1 who exhibit more symptoms of inattention and emotional/behavioral problems in their daily life. SSVEPs are potentially sensitive electrophysiological markers that could be included in future studies investigating the impact of medication on brain activity and cognitive functioning in children with NF1.

Spatial frequency processing and its modulation by emotional content in severe alcohol use disorder.

Visuo-perceptive deficits in severe alcohol use disorder (SAUD) remain little understood, notably regarding the respective involvement of the two main human visual streams, i.e., magnocellular (MC) and parvocellular (PC) pathways, in these deficits. Besides, in healthy populations, low-level visual perception can adapt depending on the nature of visual cues, among which emotional features, but this MC and PC pathway adaptation to emotional content is unexplored in SAUD. To assess MC and PC functioning as well as their emotional modulations in SAUD. We used sensitivity indices (d') and repeated-measures analyses of variance to compare orientation judgments of Gabor patches sampled at various MC- and PC-related spatial frequencies in 35 individuals with SAUD and 38 matched healthy controls. We then explored how emotional content modulated performances by introducing neutral or fearful face cues immediately before the Gabor patches and added the type of cue in the analyses. SAUD patients showed a general reduction in sensitivity across all spatial frequencies, indicating impoverished processing of both coarse and fine-scale visual content. However, we observed selective impairments depending on facial cues: individuals with SAUD processed intermediate spatial frequencies less efficiently than healthy controls following neutral faces, whereas group differences emerged for the highest spatial frequencies following fearful faces. Altogether, SAUD was associated with mixed MC and PC deficits that may vary according to emotional content, in line with aflexible but suboptimal use of low-level visual content. Such subtle alterations could have implications for everyday life's complex visual judgments.

Posterior cortical atrophy: Primary occipital variant.

Posterior cortical atrophy (PCA) is one of the atypical Alzheimer's disease variants, characterized by predominant visuospatial and visuoperceptual deficits, with established dorsal and ventral subtypes. A third primary occipital (caudal) variant has been suggested. We aimed to determine its demographics, clinical manifestations, and biomarker findings. Fifty-two PCA patients were investigated. Patients underwent neuropsychological assessment, magnetic resonance imaging, and fluorodeoxyglucose (FDG)-, amyloid-, and tau-positron emission tomography (tau-PET) scans. Normalized regional FDG-PET values were represented as z-scores relative to a control population. Patients were divided into "primary occipital" and "other PCA" subgroups according to FDG-PET-defined criteria, with primary occipital defined as patients in which the z-scores for occipital subregions were at least one standard deviation lower (SD) (i.e., more abnormal) than the z-scores in all other brain regions. Global amyloid-PET, temporo-parietal FDG-PET, and temporal tau-PET regions-of-interest (ROIs) were calculated. Nine patients were classified as primary occipital; they were older (p=0.034) and had more years of education (p=0.007) than other PCA patients. The primary occipital group performed worse on the Ishihara test for color perception (p<0.001), while other PCA patients performed worse on the Western Aphasia Battery (WAB) praxis scale (p=0.005). Overall neuropsychiatric symptom burden was lower in the primary occipital group (p<0.001). The FDG-PET meta-ROI was higher in the primary occipital subtype (p=0.006), but no differences were observed in amyloid- and tau-PET. Our findings suggest that primary occipital PCA is characterized by an older age at onset, more color perception dysfunction, less severe ideomotor apraxia, and less hypometabolism in temporo-parietal meta-ROI compared to established phenotypes.

Oscillatory Neural Signatures of Visual Perception Across Developmental Stages in Individuals With 22q11.2 Deletion Syndrome

BackgroundNumerous behavioral studies have highlighted the contribution of visual perceptual deficits to the nonverbal cognitive profile of individuals with 22q11.2 deletion syndrome. However, the neurobiological processes underlying these widespread behavioral alterations are yet to be fully understood. Thus, in this paper, we investigated the role of neural oscillations toward visuoperceptual deficits to elucidate the neurobiology of sensory impairments in deletion carriers. MethodsWe acquired 125 high-density electroencephalography recordings during a visual grating task in a group of 62 deletion carriers and 63 control subjects. Stimulus-elicited oscillatory responses were analyzed with 1) time-frequency analysis using wavelets decomposition at sensor and source level, 2) intertrial phase coherence, and 3) Granger causality connectivity in source space. Additional analyses examined the development of neural oscillations across age bins. ResultsDeletion carriers had decreased theta-band (4–8 Hz) and gamma-band (58–68 Hz) spectral power compared with control subjects in response to the visual stimuli, with an absence of age-related increase of theta- and gamma-band responses. Moreover, adult deletion carriers had decreased gamma- and theta-band responses but increased alpha/beta desynchronization (10–25 Hz) that correlated with behavioral performance. Granger causality estimates reflected an increased frontal-occipital connectivity in the beta range (22–40 Hz). ConclusionsDeletion carriers exhibited decreased theta- and gamma-band responses to visual stimuli, while alpha/beta desynchronization was preserved. Overall, the lack of age-related changes in deletion carriers implicates developmental impairments in circuit mechanisms underlying neural oscillations. The dissociation between the maturation of theta/gamma- and alpha/beta-band responses may indicate a selective impairment in supragranular cortical layers, leading to compensatory top-down connectivity.

An Unusual Case of Posttraumatic Visual Agnosia Posing Challenges to Rehabilitation

Acquired focal visuoperceptual deficits are rarely reported, and rehabilitation strategies are not well established. This is a report on visuoperceptual deficit after traumatic brain injury that initially went unnoticed. Missing visual agnosia is not unusual especially when perceptual deficits present with visual field defects, impaired insight, and inattention. This case is made more interesting because of the rarity of visual agnosia with predominant ventral pathway involvement, affecting object and face recognition. This report provides a brief discussion on visual agnosia spectrum deficits and rehabilitation measures.

Prestimulus Low-Alpha Frontal Networks Are Associated with Pareidolias in Parkinson's Disease.

Background: Pareidolias are visual phenomena wherein ambiguous, abstract forms or shapes appear meaningful due to incorrect perception. In Parkinson's disease (PD), patients susceptible to visual hallucinations experience visuo-perceptual deficits in the form of pareidolias. Although pareidolias necessitate top-down modulation of visual processing, the cortical dynamics of internally generated perceptual priors on these visual misperceptions is unknown. Objectives: To study prestimulus-related electroencephalography (EEG) spectral and network abnormalities in PD patients experiencing pareidolias. Methods: Twenty-one PD in-patients and 10 age-matched controls were evaluated. Neuropsychological assessments included tests for cognition, attention, and executive functions. Pareidolias were quantified by using the "noise pareidolia test" with simultaneous EEG recording. The PD patients were subdivided into two groups-those with high pareidolia counts (n = 10) and those without (n = 11). The EEG was analyzed 1000 msec before stimulus presentation in the spectral domain (theta, low-alpha, and high-alpha frequencies) with corresponding graph networks to evaluate network properties. Statistical analysis included analysis of variance and multiple regression to evaluate the differences. Results: The PD patients with high pareidolia counts were older with lower scores on neuropsychological tests. Their prestimulus EEG low-alpha band showed a tendency toward higher frontal activity (p = 0.07). Graph networks showed increased normalized clustering coefficient (p = 0.05) and lower frontal degree centrality (p = 0.005). These network indices correlated positively to patients' pareidolia scores. Discussion: We suggest that pareidolias in PD are a consequence of an abnormal top-down modulation of visual processing; they are defined by their frontal low-alpha spectral and network alterations in the prestimulus phase due to a dissonance between patients' internally generated mental processing with external stimuli. Impact statement Pareidolias in Parkinson's disease (PD) are considered to be promising early markers of visual hallucinations and an indicator of PD prognosis. In certain susceptible PD patients, pareidolias can be evoked and studied. Here, via electroencephalography, we aimed at understanding this visual phenomenon by studying how neural information is processed before stimulus presentation in such patients. Using spectral and graph network measures, we revealed how top-down modulated internally generated processes affect visual perception in patients with pareidolias. Our findings highlight how prestimulus network alterations in the frontal cortex shape poststimulus pareidolic manifestations in PD.

Category-selective deficits are the exception and not the rule: Evidence from a case-series of 64 patients with ventral occipito-temporal cortex damage

The organisational principles of the visual ventral stream are still highly debated, particularly the relative association/dissociation between word and face recognition and the degree of lateralisation of the underlying processes. Reports of dissociations between word and face recognition stem from single case-studies of category selective impairments, and neuroimaging investigations of healthy participants. Despite the historical reliance on single case-studies, more recent group studies have highlighted a greater commonality between word and face recognition. Studying individual patients with rare selective deficits misses (a) important variability between patients, (b) systematic associations between task performance, and (c) patients with mild, severe and/or non-selective impairments; meaning that the full spectrum of deficits is unknown. The Back of the Brain project assessed the range and specificity of visual perceptual impairment in 64 patients with posterior cerebral artery stroke recruited based on lesion localization and not behavioural performance. Word, object, and face processing were measured with comparable tests across different levels of processing to investigate associations and dissociations across domains. We present two complementary analyses of the extensive behavioural battery: (1) a data-driven analysis of the whole patient group, and (2) a single-subject case-series analysis testing for deficits and dissociations in each individual patient. In both analyses, the general organisational principle was of associations between words, objects, and faces even following unilateral lesions. The majority of patients either showed deficits across all domains or in no domain, suggesting a spectrum of visuo-perceptual deficits post stroke. Dissociations were observed, but they were the exception and not the rule: Category-selective impairments were found in only a minority of patients, all of whom showed disproportionate deficits for words. Interestingly, such selective word impairments were found following both left and right hemisphere lesions. This large-scale investigation of posterior cerebral artery stroke patients highlights the bilateral representation of visual perceptual function.

Visuoperceptive Impairments in Severe Alcohol Use Disorder: A Critical Review of Behavioral Studies.

The present literature review is aimed at offering a comprehensive and critical view of behavioral data collected during the past seventy years concerning visuoperception in severe alcohol use disorders(AUD). To pave the way for a renewal of research and clinical approaches in this very little understood field, this paper (1) provides a critical review of previous behavioral studies exploring visuoperceptive processing in severe AUD, (2) identifies the alcohol-related parameters and demographic factors that influence the deficits, and (3) addresses the limitations of this literature and their implications for current clinical strategies. By doing so, this review highlights the presence of visuoperceptive deficits but also shows how the lack of in-depth studies exploring the visual system in this clinical population results in the current absence of integration of these deficits in the dominant models of vision. Given the predominance of vision in everyday life, we stress the need to better delineate the extent, the specificity, and the actual implications of the deficits for severe AUD.

Neural correlates of visuoperceptive changes in severe alcohol use disorder: A critical review of neuroimaging and electrophysiological findings.

Visuoperceptive deficits are frequently reported in severe alcohol use disorder (SAUD) and are considered as pervasive and persistent in time. While this topic of investigation has previously driven researchers' interest, far fewer studies have focused on visuoperception in SAUD since the '90s, leaving open central questions regarding the origin and implications of these deficits. To renew research in the field and provide a solid background to work upon, this paper reviews the neural correlates of visuoperception in SAUD, based on data from neuroimaging and electrophysiological studies. Results reveal structural and functional changes within the visual system but also in the connections between occipital and frontal areas. We highlight the lack of integration of these findings in the dominant models of vision which stress the dynamic nature of the visual system and consider the presence of both bottom-up and top-down cerebral mechanisms. Visuoperceptive changes are also discussed in the framework of long-lasting debates regarding the influence of demographic and alcohol-related factors, together stressing the presence of inter-individual differences. Capitalizing on this review, we provide guidelines to inform future research, and ultimately improve clinical care.

Impaired face-like object recognition in premanifest Huntington's disease

Progressive striatal atrophy has long been considered the pathological hallmark of Huntington's disease (HD), but is it now recognized that malfunction and degeneration of posterior-cortical territories are also prominent characteristics of the disease. The limited knowledge about the functional impact of these posterior-cortical changes could be partially attributed to the lack of sensitive measures to capture them. We hypothesized that early malfunction of specific territories of the ventral visual pathway in premanifest HD would lead to difficulties in the recognition of complex stimuli and to differences in their neurophysiological correlates. To test this idea, we used an object, face and face-like object recognition task to be conducted during an electroencephalographic recording. Compared to healthy-matched controls, premanifest participants showed a significantly increased number of recognition errors in the face-like object condition. Moreover, premanifest participants showed a dramatic decrease in the N170 component elicited for the face-like objects. This N170 decrease was significantly associated with the number of recognition errors and with severity of apathy and global cognitive performance. The lack of differences in other clinical and cognitive measures supports a selective deficit in recognition of face-like objects and their neurophysiological correlates in premanifest HD. These deficits occurred in participants up to 15 years before the estimated time to disease onset and correlated strongly with cognitive and behavioral measures known to be sensitive to HD progression. This finding highlights the existence of selective visuoperceptive deficits years before motor-based onset of HD and emphasizes the need to develop sensitive measures to capture early visual system changes in this population. Assessing the integrity of the visual cortex and its related functions in HD could help to identify early markers of disease progression.

Visual perceptual disorders in Alzheimer's disease

Visual perception is humans' preferred way for taking information on the surrounding world. Visual perception is frequently impaired in patients with Alzheimer's disease, lessening patients' quality of life, and making evaluation of other cognitive deficits more complicated. Our review covers the recent literature describing visual perception deficits in patients suffering from Alzheimer's disease by classifying them according to their neuroanatomical correspondence: retina, visual pathway, subcortical structures, occipital visual cortex, occipito-temporal "what" and occipito-parietal "where" pathways. Overall, both low-level and high-level visual perception disorders seem quite common in Alzheimer's disease, including, on a low-level, loss of visual field, decreased acuity and contrast sensitivity, and impaired color vision, and on a high-level, impaired color vision, motion perception, visuospatial deficits, object agnosia, prosopagnosia and impaired recognition of facial emotional expressions. Professionals working with Alzheimer's disease should be aware of visuoperceptual deficits, which could impair the quality of life of the patients, and distort the results of neuropsychological tests using visual material. Moreover, some tests assessing visual perception could be of interest for early diagnosis of the disease.

060 Impaired color discrimination is associated with hallucinations in dementia with lewy bodies

IntroductionEmerging evidence indicates that color discrimination impairments can predict the development of dementia across a range of prodromal Lewy body conditions. However, color vision deficits are not seen uniformly in patients with Dementia with Lewy Bodies (DLB), suggesting a more nuanced association. Visual hallucinations(VH) represent a discriminating feature of DLB, and recent evidence implicates visual pathway dysfunction as a significant contributor to this phenomenon. We therefore hypothesized that color impairment will more closely associate with VH in DLB rather than general cognition.MethodsIn this study, we examined the relationship between color vision impairment and VH, along with other clinical and neuropsychological features in 24 patients with DLB alongside 25 age-matched controls. Color discrimination was assessed using the Farnsworth-Munsell-100 Hue (FM-100) test.ResultsColor discrimination impairment was seen in 16/24 DLB participants (67%) with a higher error score relative to controls(p=0.001). We demonstrate for the first time a strong association between color discrimination errors and both the presence and severity of VH in DLB based on clinician-derived(p=0.008) and questionnaire-derived(p=0.03) measures. Correlation with clinical and neuropsychological variables revealed that color discrimination is significantly related to visuospatial impairment(p=0.02) but not to global measures of cognition, motor severity, age or disease duration. Factor analysis confirmed a unique relationship between color discrimination, visual hallucinations and visuospatial function.ConclusionOur results suggest that color impairments may be a specific biomarker of visual hallucinations and associated visuoperceptual deficits in evolving Lewy body disorders rather than dementia per se and thus providing insight into a shared pathophysiological substrate.

Neural correlates of early cognitive dysfunction in Parkinson's disease.

ObjectiveDementia is a common and feared aspect of Parkinson's disease but there are no robust predictors of cognitive outcome. Visuoperceptual deficits are linked to risk of dementia in Parkinson's disease but whether they predict cognitive change is not known, and the neural substrates of visuoperceptual dysfunction in Parkinson's have not yet been identified.MethodsWe compared patients with Parkinson's disease and unaffected controls who underwent BOLD fMRI while performing our previously validated visuoperceptual task and tested how functional connectivity between task‐specific regions and the rest of the brain differed between patients who performed well and poorly in the task.ResultsWe show that task performance at baseline predicts change in cognition in Parkinson's disease after 1 year. Our task‐based fMRI study showed that the performance in this task is associated with activity in the posterior cingulate cortex/precuneus. We found that functional connectivity between this region and dorsomedial prefrontal cortex was reduced in poor performers compared with good performers of this task.InterpretationOur findings suggest that functional connectivity is reduced between posterior and anterior hubs of the default mode network in Parkinson's patients who are likely to progress to worsening cognitive dysfunction. Our work implicates posterior default mode nodes and their connections as key brain regions in early stages of dementia in Parkinson's disease.

White matter changes associated with cognitive visual dysfunctions in children with cerebral palsy: A diffusion tensor imaging study.

Children with cerebral palsy often present with cognitive-visual dysfunctions characterized by visuo-perceptual and/or visuo-spatial deficits associated with a malfunctioning of visual-associative areas. The neurofunctional model of this condition remains poorly understood due to the lack of a clear correlation between cognitive-visual deficit and morphological brain anomalies. The aim of our study was to quantify the pattern of white matter abnormalities within the whole brain in children with cerebral palsy, and to identify white matter tracts sub-serving cognitive-visual functions, in order to better understand the basis of cognitive-visual processing. Nine subjects (three males, mean age 8 years 9 months) with cerebral palsy underwent a visual and cognitive-visual evaluation. Conventional brain MRI and diffusion tensor imaging were performed. The fractional anisotropy maps were calculated for every child and compared with data from 13 (four males, mean age 10 years 7 months) healthy children. Children with cerebral palsy showed decreased fractional anisotropy (a marker of white matter integrity) in corticospinal tract bilaterally, left superior longitudinal fasciculus and bilateral hippocampus. Focusing on the superior longitudinal fasciculus, the mean fractional anisotropy values were significantly lower in children affected by cerebral palsy with cognitive-visual deficits than in those without cognitive-visual deficits. Our findings reveal an association between cognitive-visual profile and the superior longitudinal fasciculus integrity in children with cerebral palsy, supporting the hypothesis that visuo-associative deficits are related to changes in fibers connecting the occipital cortex with the parietal-frontal cortices. Decreased fractional anisotropy within the superior longitudinal fasciculus could be considered a biomarker for cognitive-visual dysfunctions.

Abnormal Functional Connectivity Density in Amyotrophic Lateral Sclerosis.

Purpose: Amyotrophic lateral sclerosis (ALS) is a motor neuro-degenerative disorder that also damages extra-motor neural pathways. A significant proportion of existing evidence describe alterations in the strengths of functional connectivity, whereas the changes in the density of these functional connections have not been explored. Therefore, our study seeks to identify ALS-induced alternations in the resting-state functional connectivity density (FCD).Methods: Two groups comprising of 38 ALS patients and 35 healthy participants (age and gender matched) were subjected to the resting-state functional magnetic resonance imaging (MRI) scanning. An ultra-fast graph theory method known as FCD mapping was utilized to calculate the voxel-wise short- and long-range FCD values of the brain for each participant. FCD values of patients and controls were compared based on voxels in order to discern cerebral regions that possessed significant FCD alterations. For areas demonstrating a group effect of atypical FCD in ALS, seed-based functional connectivity analysis was then investigated. Partial correlation analyses were carried out between aberrant FCDs and several clinical variables, controlling for age, gender, and total intracranial volume.Results: Patients with ALS were found to have decreased short-range FCD in the primary motor cortex and increased long-range FCD in the premotor cortex. Extra-motor areas that also displayed extensive FCD alterations encompassed the temporal cortex, insula, cingulate gyrus, occipital cortex, and inferior parietal lobule. Seed-based correlation analysis further demonstrated that these regions also possessed disrupted functional connectivity. However, no significant correlations were identified between aberrant FCDs and clinical variables.Conclusion: FCD changes in the regions identified represent communication deficits and impaired functional brain dynamics, which might underlie the motor, motor control, language, visuoperceptual and high-order cognitive deficits in ALS. These findings support the fact that ALS is a disorder affecting multiple systems. We gain a deeper insight of the neural mechanisms underlying ALS.