Long-lasting pure topographical disorientation due to heading disorientation following left retrosplenial infarction: A report of two cases.

Topographical disorientation is linked to lesions in the right hemisphere and typically resolves within a few months post-stroke. Persistent topographical disorientation is uncommon and frequently accompanied by impairments in visual memory, complicating the analysis of the underlying mechanisms. Herein, we report two cases of sustained pure topographical disorientation following cerebral hemorrhages in the left retrosplenial region. The patients exhibited disorientation in both familiar and unfamiliar settings, attributable to heading disorientation, a deficit in determining the directional relationship between one's current position and a target location or external frames. The patients struggled with reconstructing large-scale spatial frameworks and integrating new egocentric and allocentric perspectives upon changes in body orientation. There were no landmark agnosia, egocentric disorientation, or anterograde disorientation. Although mild verbal memory deficits were observed, no other cognitive impairments, including visual memory deficits, were detected. Our findings imply that lesions confined to the left retrosplenial region can induce enduring heading disorientation and suggest a significant role for this area in processing and integrating spatial information necessary for large-scale navigation. Clarifying the features of topographical disorientation will significantly impact the therapeutic approaches, enhancing the quality of life for affected patients by restoring their independence and mobility.

Humans' extreme face recognition abilities challenge the well-established familiarity effect.

Classification performance is better for learned than unlearned stimuli. This was also reported for faces, where identity matching of unfamiliar faces is worse than for familiar faces. This familiarity advantage led to the conclusion that variability across appearances of the same identity is partly idiosyncratic and cannot be generalized from familiar to unfamiliar identities. Recent advances in machine vision challenge this claim by showing that the performance for untrained (unfamiliar) identities reached the level of trained identities as the number of identities that the algorithm is trained with increases. We therefore asked whether humans who reportedly can identify a vast number of identities, such as super recognizers, may close the gap between familiar and unfamiliar face classification. Consistent with this prediction, super recognizers classified unfamiliar faces just as well as typical participants who are familiar with the same faces, on a task that generates a sizable familiarity effect in controls. Additionally, prosopagnosics' performance for familiar faces was as bad as that of typical participants who were unfamiliar with the same faces, indicating that they struggle to learn even identity-specific information. Overall, these findings demonstrate that by studying the extreme ends of a system's ability we can gain novel insights into its actual capabilities.

Symptomatology after damage to the angular gyrus through the lenses of modern lesion-symptom mapping.

Brain-behavior relationships are complex. For instance, one might know a brain region's function(s) but still be unable to accurately predict deficit type or severity after damage to that region. Here, I discuss the case of damage to the angular gyrus (AG) that can cause left-right confusion, finger agnosia, attention deficit, and lexical agraphia, as well as impairment in sentence processing, episodic memory, number processing, and gesture imitation. Some of these symptoms are grouped under AG syndrome or Gerstmann's syndrome, though its exact underlying neuronal systems remain elusive. This review applies recent frameworks of brain-behavior modes and principles from modern lesion-symptom mapping to explain symptomatology after AG damage. It highlights four major issues for future studies: (1) functionally heterogeneous symptoms after AG damage need to be considered in terms of the degree of damage to (i) different subdivisions of the AG, (ii) different AG connectivity profiles that disconnect AG from distant regions, and (iii) lesion extent into neighboring regions damaged by the same infarct. (2) To explain why similar symptoms can also be observed after damage to other regions, AG damage needs to be studied in terms of the networks of regions that AG functions with, and other independent networks that might subsume the same functions. (3) To explain inter-patient variability on AG symptomatology, the degree of recovery-related brain reorganisation needs to account for time post-stroke, demographics, therapy input, and pre-stroke differences in functional anatomy. (4) A better integration of the results from lesion and functional neuroimaging investigations of AG function is required, with only the latter so far considering AG function in terms of a hub within the default mode network. Overall, this review discusses why it is so difficult to fully characterize the AG syndrome from lesion data, and how this might be addressed with modern lesion-symptom mapping.

Autistic adults have insight into their relative face recognition ability.

The PI20 is a self-report questionnaire that assesses the presence of lifelong face recognition difficulties. The items on this scale ask respondents to assess their face recognition ability relative to the rest of the population, either explicitly or implicitly. Recent reports suggest that the PI20 scores of autistic participants exhibit little or no correlation with their performance on the Cambridge Face Memory Test-a key measure of face recognition ability. These reports are suggestive of a meta-cognitive deficit whereby autistic individuals are unable to infer whether their face recognition is impaired relative to the wider population. In the present study, however, we observed significant correlations between the PI20 scores of 77 autistic adults and their performance on two variants of the Cambridge Face Memory Test. These findings indicate that autistic individuals can infer whether their face recognition ability is impaired. Consistent with previous research, we observed a wide spread of face recognition abilities within our autistic sample. While some individuals approached ceiling levels of performance, others met the prevailing diagnostic criteria for developmental prosopagnosia. This variability showed little or no association with non-verbal intelligence, autism severity, or the presence of co-occurring alexithymia or ADHD.

Unilateral tactile agnosia as an onset symptom of corticobasal syndrome.

Tactile agnosia is the inability to recognize objects via haptic exploration, in the absence of an elementary sensory deficit. Traditionally, it has been described as a disturbance in extracting information about the physical properties of objects ("apperceptive agnosia") or in associating object representation with its semantic meaning ("associative agnosia"). However, tactile agnosia is a rare and difficult-to-diagnose condition, due to the frequent co-occurrence of sensorimotor symptoms and the lack of consensus on the terminology and assessment methods. Among tactile agnosia classifications, hyloagnosia (i.e., difficulty in quality discrimination of objects) and morphoagnosia (i.e., difficulty in shape and size recognition) have been proposed to account for the apperceptive level. However, a dissociation between the two has been reported in two cases only. Indeed, very few cases of pure tactile agnosia have been described, mostly associated with vascular damages in somatosensory areas, in pre- and postcentral gyrus, intraparietal sulcus, supramarginal gyrus, and insular cortex. An open question is whether degenerative conditions affecting the same areas could lead to similar impairments. Here, we present a single case of unilateral right-hand tactile agnosia, in the context of corticobasal syndrome (CBS), a rare neurodegenerative disease. The patient, a 55-year-old woman, initially presented with difficulties in tactile object recognition, apraxia for the right hand, and an otherwise intact cognitive profile. At the neuroimaging level, she showed a lesion outcome of a right parietal oligodendroglioma removal and a left frontoparietal atrophy. We performed an experimental evaluation of tactile agnosia, targeting every level of tactile processing, from elementary to higher order tactile recognition processes. We also tested 18 healthy participants as a matched control sample. The patient showed intact tactile sensitivity and mostly intact hylognosis functions. Conversely, she was impaired with the right hand in exploring geometrical and meaningless shapes. The patient's clinical evolution in the following 3 years became consistent with the diagnosis of CBS and unilateral tactile apperceptive agnosia as the primary symptom onset in the absence of a cognitive decline. This is the third case described in the literature manifesting morphoagnosia with almost completely preserved hylognosis abilities and the first description of such dissociation in a case with CBS.

Biopsychosocial Factors That Influence Depression, Anxiety, and Neuropsychological Functions in the Aging Process.

BACKGROUND: Aging is a process inherent to the human condition that is characterized by various biological, psychological, and social changes that can lead to certain mental health problems. OBJECTIVE: This study aimed to understand and characterize how biological, psychological, and social factors influence mental health symptoms and neuropsychological function in the aging process. METHODS: A sample consisting of 616 participants (73.8% female) aged between 50 and 90 years, (mean [SD]: 69.6 [17.2] years). Data collection involved a neuropsychological assessment carried out by a psychologist in a clinical interview session and the self-completion of a questionnaire. RESULTS: Regression models revealed that depressive symptoms are explained by anxiety, psychological and environmental quality of life, and sleep habits. Anxiety symptoms are explained by depression, physical quality of life, and sleep habits. Statistically significant differences were identified between participants with and without anxiety in neuropsychological terms at the level of agnosia and praxis. Statistically significant differences were identified between participants with and without depression in neuropsychological terms at the level of memory, language, agnosia and praxis, and executive function (eg, prefrontal tests). CONCLUSIONS: Depression and anxiety symptoms are associated and could negatively influence the aging process. Aging should be understood in an ecological way taking into account the action of biological, psychological, social, and environmental factors, as well as the relationship and influence between the different levels and factors. This knowledge is important for diagnosis, for clinical practice, and for the development and definition of public policies to promote healthy aging.

Design and Interrater Reliability of the Pediatric Version of the Race Scale: PedRACE.

BACKGROUND: Acute ischemic stroke is a leading cause of pediatric death and disability. A clinical scale adapted for children can ensure early detection of candidates for urgent acute ischemic stroke treatment. The Rapid Arterial Occlusion Evaluation (RACE) scale for adults, which scores 5 items (facial palsy 0-2; arm motor function 0-2; leg motor function 0-2; head/gaze deviation 0-1; and aphasia or agnosia 0-2), has good sensitivity and specificity in detecting large vessel occlusion. METHODS: We adapted the previously validated RACE scale for use in children as the Pediatric RACE scale. This adapted scale was tested by prehospital/emergency room staff attending to patients covered by the Catalan Pediatric Stroke Code and child neurologists for its correlation with the Pediatric National Institutes of Health Stroke Scale and for interrater reliability. RESULTS: The study included 50 children, 18 with confirmed strokes (7 acute ischemic strokes and 11 hemorrhagic strokes). Prehospital/emergency staff and child neurologists agreed fully regarding 82% of patients and 100% regarding head/gaze deviation and agnosia. The Pediatric RACE scale correlated strongly with the Pediatric National Institutes of Health Stroke Scale in evaluations by child neurologists (Spearman ρ, 0.852; P<0.001) and prehospital/emergency staff (Spearman ρ, 0.781; P<0.001). The median Pediatric RACE score was significantly higher in patients with large vessel occlusion (6.5; interquartile range, 6-7) than with other etiologies. CONCLUSIONS: Pediatric RACE, showing good interrater reliability and correlation with the Pediatric National Institutes of Health Stroke Scale, is a simple scale to detect candidates for pediatric acute stroke treatment, designed for both prehospital and in-hospital use by non-neurologist medical staff.

Improving the DSM-5 approach to cognitive impairment: Developmental prosopagnosia reveals the need for tailored diagnoses.

The Diagnostic Statistical Manual of Mental Disorders (DSM-5) recommends diagnosing neurocognitive disorders (i.e., cognitive impairment) when a patient scores beyond - 1 SD below neurotypical norms on two tests. I review how this approach will fail due to cognitive tests' power limitations, validity issues, imperfect reliabilities, and biases, before summarizing their resulting negative consequences. As a proof of concept, I use developmental prosopagnosia, a condition characterized by difficulties recognizing faces, to show the DSM-5 only diagnoses 62-70% (n1 = 61, n2 = 165) versus 100% (n1 = 61) through symptoms alone. Pooling the DSM-5 missed cases confirmed the presence of group-level impairments on objective tests, which were further evidenced through meta-analyses, thus validating their highly atypical symptoms. These findings support a paradigm shift towards bespoke diagnostic approaches for distinct cognitive impairments, including a symptom-based method when validated effective. I reject dogmatic adherence to the DSM-5 approach to neurocognitive disorders, and underscore the importance of a data driven, transdiagnostic approach to understanding patients' subjective cognitive impairments. This will ultimately benefit patients, their families, clinicians, and scientific progress.

Familiarity enhances functional connectivity between visual and nonvisual regions of the brain during natural viewing.

We explored the neural correlates of familiarity with people and places using a naturalistic viewing paradigm. Neural responses were measured using functional magnetic resonance imaging, while participants viewed a movie taken from Game of Thrones. We compared inter-subject correlations and functional connectivity in participants who were either familiar or unfamiliar with the TV series. Higher inter-subject correlations were found between familiar participants in regions, beyond the visual brain, that are typically associated with the processing of semantic, episodic, and affective information. However, familiarity also increased functional connectivity between face and scene regions in the visual brain and the nonvisual regions of the familiarity network. To determine whether these regions play an important role in face recognition, we measured responses in participants with developmental prosopagnosia (DP). Consistent with a deficit in face recognition, the effect of familiarity was significantly attenuated across the familiarity network in DP. The effect of familiarity on functional connectivity between face regions and the familiarity network was also attenuated in DP. These results show that the neural response to familiarity involves an extended network of brain regions and that functional connectivity between visual and nonvisual regions of the brain plays an important role in the recognition of people and places during natural viewing.

Rare presentation of Klüver-Bucy syndrome following subarachnoid hemorrhage.

Background: Klüver-Bucy syndrome (KBS) is a rare neuropsychiatric disorder, and it can be associated with a variety of neurological disorders. It is characterized by visual agnosia, placidity, hyperorality, hypersexuality, dietary changes, amnesia, and hypermetamorphosis. KBS is mainly a clinical diagnosis, with at least three symptoms sufficient to diagnose the condition. Case Description: The case describes a 49-year-old Filipino woman with a history of hypertension who presented with symptoms strongly suggesting KBS following subarachnoid hemorrhage, including behaviors such as hyperorality, hypermobility, placidity, hypermetamorphosis, and hypersexuality along with memory disturbance. She was managed as a case of brief psychotic disorder initially with olanzapine, then on the second presentation as a case of delirium with risperidone. Conclusion: Among many symptoms of KBS, only three symptoms are required for the diagnosis clinically. Numerous neurological conditions can cause KBS. Symptomatic treatment is the mainstream treatment currently for KBS.[3] While different differential diagnoses are present, neurologists, psychiatrists, neurosurgeons, and radiologists should collaborate and be vigilant for the diagnosis of KBS, especially with the presence of one of its etiologies.

Normative data of the Italian Famous Face Test.

The faces we see in daily life exist on a continuum of familiarity, ranging from personally familiar to famous to unfamiliar faces. Thus, when assessing face recognition abilities, adequate evaluation measures should be employed to discriminate between each of these processes and their relative impairments. We here developed the Italian Famous Face Test (IT-FFT), a novel assessment tool for famous face recognition in typical and clinical populations. Normative data on a large sample (N = 436) of Italian individuals were collected, assessing both familiarity (d') and recognition accuracy. Furthermore, this study explored whether individuals possess insights into their overall face recognition skills by correlating the Prosopagnosia Index-20 (PI-20) with the IT-FFT; a negative correlation between these measures suggests that people have a moderate insight into their face recognition skills. Overall, our study provides the first online-based Italian test for famous faces (IT-FFT), a test that could be used alongside other standard tests of face recognition because it complements them by evaluating real-world face familiarity, providing a more comprehensive assessment of face recognition abilities. Testing different aspects of face recognition is crucial for understanding both typical and atypical face recognition.

The Rehabilitation for Visual Cognitive Impairment due to Hippocampal Infarction: A Case Report.

Introduction: Disturbances in the visual pathway cause visual cognitive impairment. There is a lack of information regarding the effect of rehabilitation on individuals affected by this condition. Therefore, it is crucial to understand the effectiveness of rehabilitation interventions in this condition. Case Presentation: We present the case of an 87-year-old woman with hippocampal infarction. While the patient's ability to perform daily activities and engage in conversations was normal, she faced challenges at the execution stage, such as naming, constructing sentences, and copying. We diagnosed cerebral embolism because of atrial fibrillation and initiated direct oral anticoagulant therapy. Subsequently, we initiated a rehabilitation treatment comprising visual agnosia training (attribute learning training), verbalization learning training (writing training), and semantic memory training (copying training) to address visual agnosia. Conclusion: Associative visual perception challenges arising from hippocampal infarcts are rare and can be improved with early intervention through a rehabilitation program for visual agnosia.

True or false? Alzheimer's disease is type 3 diabetes: Evidences from bench to bedside.

Globally, Alzheimer's disease (AD) is the most widespread chronic neurodegenerative disorder, leading to cognitive impairment, such as aphasia and agnosia, as well as mental symptoms, like behavioral abnormalities, that place a heavy psychological and financial burden on the families of the afflicted. Unfortunately, no particular medications exist to treat AD, as the current treatments only impede its progression.The link between AD and type 2 diabetes (T2D) has been increasingly revealed by research; the danger of developing both AD and T2D rises exponentially with age, with T2D being especially prone to AD. This has propelled researchers to investigate the mechanism(s) underlying this connection. A critical review of the relationship between insulin resistance, Aß, oxidative stress, mitochondrial hypothesis, abnormal phosphorylation of Tau protein, inflammatory response, high blood glucose levels, neurotransmitters and signaling pathways, vascular issues in AD and diabetes, and the similarities between the two diseases, is presented in this review. Grasping the essential mechanisms behind this detrimental interaction may offer chances to devise successful therapeutic strategies.

Gerstmann Syndrome: What is the Possible Role of Deep Brain Stimulation?

Gerstmann syndrome, characterized by a tetrad of symptoms, which are agraphia, acalculia, left-right disorientation, and finger agnosia, presents challenges in both understanding its pathophysiology and establishing effective treatment modalities. Neuroanatomical studies have highlighted the involvement of the dominant parietal lobe, particularly the inferior parietal lobule, in the development of Gerstmann syndrome. Although current treatment options are largely supportive, recent research suggests a potential role for deep brain stimulation (DBS) in managing this condition. DBS, known for its efficacy in various neurological disorders, has been hypothesized to modulate neuronal pathways associated with Gerstmann syndrome. However, clinical evidence supporting DBS in Gerstmann syndrome remains scarce, posing challenges in patient selection and ethical considerations. Future research should prioritize investigating the efficacy and safety of DBS in Gerstmann syndrome to improve patient outcomes and quality of life.

Electrophysiological markers of vestibular-mediated self-motion perception - A pilot study.

Peripheral vestibular activation results in multi-level responses, from brainstem-mediated reflexes (e.g. vestibular ocular reflex - VOR) to perception of self-motion. While VOR responses indicate preserved vestibular peripheral and brainstem functioning, there are no automated measures of vestibular perception of self-motion - important since some patients with brain disconnection syndromes manifest a vestibular agnosia (intact VOR but impaired self-motion perception). Electroencephalography ('EEG') - may provide a surrogate marker of vestibular perception of self-motion. A related objective is obtaining an EEG marker of vestibular sensory signal processing, distinct from vestibular-motion perception. We performed a pilot study comparing EEG responses in the dark when healthy participants sat in a vibrationless computer-controlled motorised rotating chair moving at near threshold of self-motion perception, versus a second situation in which subjects sat in the chair at rest in the dark who could be induced (or not) into falsely perceiving self-motion. In both conditions subjects could perceive self-motion perception, but in the second there was no bottom-up reflex-brainstem activation. Time-frequency analyses showed: (i) alpha frequency band activity is linked to vestibular sensory-signal activation; and (ii) theta band activity is a marker of vestibular-mediated self-motion perception. Consistent with emerging animal data, our findings support the role of theta activity in the processing of self-motion perception.

Undoubtedly unaware of homonymous hemianopia: The contribution of overconfidence to anosognosia of hemianopia.

A new functional deficit caused by a stroke can be understood as a situation of uncertainty that has to prompt deficit discovery and subsequent incorporation into an altered self-perception. Anosognosia for visual field deficits is frequent after stroke. For hemiplegia, patients' performance in a riddle test provided evidence that the inability to generate and adjust beliefs in face of uncertainty contributes to anosognosia for hemiplegia. In this prospective study, the same riddles are used in patients with homonymous hemianopia due to a first-ever stroke in the posterior cerebral artery territory and in an age-matched control cohort. The riddles create a situation of uncertainty that is resolved with five successive clues which progressively delimit the target word. After each clue, patients have to guess the target word and rate their confidence in the answer's correctness. Patients were tested once during the hospital stay. According to the Bisiach score for anosognosia, 12 out of 29 patients were unaware of their visual field deficits. All patients with anosognosia for hemianopia had right hemisphere lesions. Patients with and without anosognosia did not differ significantly in global cognitive impairment, mental flexibility or memory function. Importantly, patients with anosognosia showed higher confidence ratings than patients without anosognosia and controls in the first two clues (situations of uncertainty). This was demonstrated by a significant interaction effect in a mixed ANOVA with the factors group (anosognosia, nosognosia, controls) and riddle clues. An exploratory lesion subtraction analysis showed a high proportion of deficit unawareness in patients with lesions in the right fusiform and (para)hippocampal gyri. Our findings suggest that overconfidence in situations of uncertainty might contribute to the appearance of anosognosia for hemianopia. Because this has been demonstrated before in anosognosia for hemiplegia, we suggest that overconfidence is a supra-modal contributor to deficit unawareness.

Clinical and structural disconnectome evaluation in a case of optic aphasia.

Optic Aphasia (OA) and Associative Visual Agnosia (AVA) are neuropsychological disorders characterized by impaired naming on visual presentation. From a cognitive point of view, while stimulus identification is largely unimpaired in OA (where access to semantic knowledge is still possible), in AVA it is not. OA has been linked with right hemianopia and disconnection of the occipital right-hemisphere (RH) visual processing from the left hemisphere (LH) language areas.In this paper, we describe the case of AA, an 81-year-old housewife suffering from a deficit in naming visually presented stimuli after left occipital lesion and damage to the interhemispheric splenial pathway. AA has been tested through a set of tasks assessing different levels of visual object processing. We discuss behavioral performance as well as the pattern of lesion and disconnection in relation to a neurocognitive model adapted from Luzzatti and colleagues (1998). Despite the complexity of the neuropsychological picture, behavioral data suggest that semantic access from visual input is possible, while a lesion-based structural disconnectome investigation demonstrated the splenial involvement.Altogether, neuropsychological and neuroanatomical findings support the assumption of visuo-verbal callosal disconnection compatible with a diagnosis of OA.

Do I Have Symptoms of Dementia: A Discursive Study of Awareness and Shame Among People With Advanced Dementia.

BACKGROUND AND OBJECTIVES: This study explores the narrated experiences of individuals with advanced stages of late-onset dementia, focusing on their diagnosis awareness. Such framing is motivated by 2 reasons. Firstly, there is a lack of consensus regarding the prevalence of anosognosia among people with dementia. Secondly, research on anosognosia often neglects to address the important issues of shame and stigma associated with receiving a dementia diagnosis. RESEARCH DESIGN AND METHODS: For this qualitative study, a total of 27 participants ranging in age from 66 to 94 were involved. The data collected were analyzed using textual-oriented discourse analysis. RESULTS: Our findings indicated that individuals with dementia struggled to comprehend the medical terminology used to describe their experiences within biomedical standards. The interviewees utilized 5 negative discourses on dementia, which shaped their attitudes toward the condition and people diagnosed with it. These discourses depicted dementia as an illness, negative aging, a devaluation, a burden, and a life tragedy. Moreover, study participants did not outright reject the diagnosis but rather negotiated its acceptance within the context of shame associated with dementia. DISCUSSION AND IMPLICATIONS: The concept of anosognosia can serve as a mechanism of social control and stigmatization of people with dementia within the dominant biomedical discourse.

Visual dysfunction in dementia with Lewy bodies.

PURPOSE OF REVIEW: To review the literature on visual dysfunction in dementia with Lewy bodies (DLB), including its mechanisms and clinical implications. RECENT FINDINGS: Recent studies have explored novel aspects of visual dysfunction in DLB, including visual texture agnosia, mental rotation of 3-dimensional drawn objects, and reading fragmented letters. Recent studies have shown parietal and occipital hypoperfusion correlating with impaired visuoconstruction performance. While visual dysfunction in clinically manifest DLB is well recognized, recent work has focused on prodromal or mild cognitive impairment (MCI) due to Lewy body pathology with mixed results. Advances in retinal imaging have recently led to the identification of abnormalities such as parafoveal thinning in DLB. Patients with DLB experience impairment in color perception, form and object identification, space and motion perception, visuoconstruction tasks, and illusions in association with visual cortex and network dysfunction. These symptoms are associated with visual hallucinations, driving impairment, falls, and other negative outcomes.