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We report the case of DT, a man in his fifties who suffered a large right fronto-parietal hemorrhage, resulting in a dense left weakness and a persistent anosognosia for the resultant hemiplegia. DT engaged in several video-feedback sessions. Video feedback therapy has been successful in previously reported cases of anosognosia, resulting in immediate and lasting resolution. In the current case, similarly dramatic effects were observed, but with a subsequent recurrence of the anosognosic ideation. The present case sheds light on the interplay of neurologic and psychological mechanisms involved in anosognosia and on some of the pragmatic emotional considerations of engaging in this form of rehabilitative therapy.

Background: Vestibular dysfunction causing imbalance affects c. 80% of acute hospitalized traumatic brain injury (TBI) cases. Poor balance recovery is linked to worse return-to-work rates and reduced longevity. We previously showed that white matter network disruption, particularly of right inferior longitudinal fasciculus, mediates the overlap between imbalance and impaired vestibular perception of self-motion (i.e., vestibular agnosia) in acute hospitalized TBI. However, there are no prior reports tracking the acute-longitudinal trajectory of objectively measured vestibular function for hospitalized TBI patients. We hypothesized that recovery of vestibular agnosia and imbalance is linked and mediated by overlapping brain networks., Methods: We screened 918 acute major trauma in-patients, assessed 146, recruited 39 acutely, and retested 34 at 6 months. Inclusion criteria were 18-65-year-old adults hospitalized for TBI with laboratory-confirmed preserved peripheral vestibular function. Benign paroxysmal positional vertigo and migraine were treated prior to testing. Vestibular agnosia was quantified by participants' ability to perceive whole-body yaw plane rotations via an automated rotating-chair algorithm. Subjective symptoms of imbalance (via questionnaires) and objective imbalance (via posturography) were also assessed., Results: Acute vestibular agnosia predicted poor balance recovery at 6 months. Recovery of vestibular agnosia and linked imbalance was mediated by bihemispheric fronto-posterior cortical circuits. Recovery of subjective symptoms of imbalance and objective imbalance were not correlated., Conclusion: Vestibular agnosia mediates balance recovery post-TBI. The link between subjective dizziness and brain injury recovery, although important, is unclear. Therapeutic trials of vestibular recovery post-TBI should target enhancing bi-hemispheric connectivity and linked objective clinical measures (e.g., posturography)., Competing Interests: Declarations. Conflict of interest: The authors report no competing interests., (© 2025. The Author(s).)

Importance: GAAB Syndrome was recently discovered and coined by Rodríguez, Azariah, Ritter, et al.. (2024). It is characterized by bifrontal brain injury, visual pathway damage involving bilateral enucleation, generalized and visual anosognosia and lack of emotional processing with visual anosognosia being more prominent in the clinical presentation of the patient given the context of bilateral enucleation. The syndrome was not explained by delirium nor by amnesia, not either by multiple shunt adjustments or psychological denial., Objective: To describe the clinical presentation and syndrome length of the patient one year after injury. Results show that most of the syndrome symptoms are resolved after nine months, with just visual anosognosia not resolving completely. The patient improved cognitively as shown by the same tests one year later.

Background: Unawareness or anosognosia of memory impairment is a common phenomenon in patients with Alzheimer's disease (AD). Different findings have been reported regarding its presentation, assessment procedure, and cognitive correlates., Objective: To assess memory awareness of early AD patients predictively (before memory testing) and online (immediately after performing a memory test)., Methods: All participants were outpatients of a memory clinic. AD patients were compared with participants having mild cognitive impairment (MCI) and a group with normal cognitive aging (NC). We used a performance-based assessment procedure to measure self-perceived memory abilities. An anosognosia ratio was calculated by matching self-estimates with objective memory scores derived from the Consortium to Establish a Registry for Alzheimer's disease Neuropsychological battery., Results: Memory anosognosia in terms of self-overestimation was found in almost half of the AD sample, only rarely in MCI, and was not present in NC. Most AD patients had both, a low prediction and also a deficient online accuracy of self-estimation. Memory overestimators were older, less educated, and had significantly poorer neuropsychological scores. A stepwise linear regression analysis showed that memory anosognosia was predicted by age, everyday functional abilities and neuropsychological variables, including executive and mnestic abilities., Conclusions: Poor cognitive and memory functions combined with memory anosognosia are a hallmark of early AD. Further, mnemonic anosognosia as assessed by performance based measures separates AD patients already in the early disease stage from subjects with MCI or normal controls. Our findings highlight the importance of assessing memory self-appraisal in subjects attending a memory clinic, in addition to clinical and cognitive variables., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Data availabilityThe data of this study are available on reasonable request from the corresponding author.

La qualité des soins apportés aux personnes vivant avec la maladie d'Alzheimer (MA) dépend en partie de la capacité des professionnels à déterminer le degré de conscience de la maladie chez les patients. La présente recherche s'est intéressée aux représentations des soignants concernant la conscience des troubles chez les résidents d'établissements de soins de longue durée présentant un diagnostic de MA. Le pouvoir prédicteur de l'anosognosie sur le fardeau soignant a également été examiné. L'anosognosie des troubles de la construction (r = 0,40, p = 0,0164) et de l'initiation (r = 0,32, p = 0,052) était corrélée au fardeau soignant. Les professionnels se représentaient les résidents comme ayant une conscience altérée de leurs capacités, même en l'absence d'anosognosie. Les scores réels d'anosognosie ne prédisaient pas les estimations soignantes, hormis le score global sous forme de tendance (χ 2 = 3,38, p = 0,066). Les soignants surestimaient pourtant les performances cognitives des résidents, telles que mesurées au moyen du protocole Misawareness (prédictions aidants/performances réelles : DC = 12,32, p < 0,0001).

This case report presents the story of Mr. S, a professional orchestral musician with declining musical sight-reading ability, followed by progressive visuospatial and language deficits. Our novel musical assessment battery revealed deficits in music-reading (musical alexia) and music-writing (musical agraphia), with spared auditory perception and expression. Taken with neuropsychological testing, clinical history, and imaging, we conclude that his symptoms evolved from musical alexia to a multidomain, neurodegenerative process centered in the dominant inferior parietal lobe and temporoparietal junction. We suspect a primary TDP-opathy with comorbid preclinical Alzheimer's disease. Mr. S's case highlights musical symptoms as meaningful, early indicators of neurodegeneration.

Anosognosia for visual deficits - cases where significant visual deficits go unnoticed - challenges the view that our own conscious experiences are what we know best. We review these widespread and striking failures of awareness. Anosognosia can occur with total blindness, visual abnormalities induced by brain lesions, and eye diseases. We show that anosognosia for visual deficits is surprisingly widespread. Building on previous accounts, we introduce a framework showing how apparently disparate forms of anosognosia fit together. The central idea is that, to notice a deficit, individuals need to form expectations about normal vision, compare expectations and visual input, and judge any mismatch at the metacognitive level. A failure in any of these three steps may lead to unawareness of visual deficits., Competing Interests: Declaration of interests No interests are declared., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Alzheimer's disease (AD) encompasses a long continuum from a preclinical phase, characterized by neuropathological alterations albeit normal cognition, to a symptomatic phase, marked by its clinical manifestations. Yet, the neural mechanisms responsible for cognitive decline in AD patients remain poorly understood. Here, we posit that anosognosia, emerging from an error-monitoring failure due to early amyloid-β deposits in the posterior cingulate cortex, plays a causal role in the clinical progression of AD by preventing patients from being aware of their deficits and implementing strategies to cope with their difficulties, thus fostering a vicious circle of cognitive decline., (© 2024. The Author(s).)

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., Competing Interests: Declaration of competing interest The author declares that he has no conflict of interest., (Copyright © 2024 The Author. Published by Elsevier Ltd.. All rights reserved.)

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., (© 2024. The Author(s).)

Objective: Two studies were performed to validate a brief measure of cognitive insight and compare it to an empirical model - the Cognitive Awareness Model (CAM)., Method: A pilot study included 31 (52% male; Mage = 69.42) patients from an outpatient neuropsychological assessment clinic. Seven patients were diagnosed with likely Alzheimer's dementia (AD), 15 mild cognitive impairment (MCI), and 9 no diagnosis (i.e., cognitively normal; CN). The Cognitive Coding Form (CCF) and several other measures were administered. Study 2 entailed archival data extraction of 240 patients (80 CN, 80 MCI, and 80 AD; 53.3% female; Mage = 72.8) to examine whether the CCF predicts memory (Wechsler Memory Scale - IV) and executive functioning (Trail-Making Test B)., Results: The pilot study found preliminary evidence of convergent and discriminant validity for the 8-item CCF. Study 2 confirmed that both patient-reported cognitive concerns (F(2,237) = 10.40, p < .001, ω2 = .07, power = .99) and, more strongly, CCF informant-patient discrepancy scores (F(2,237) = 24.52, p < .001, ω2 = .16, power = .99) can distinguish CNs from those with MCI and AD. A regression indicated that depression (5.5%; β = -.38, p < .001) and TMT-B (13%; β = -.43, p < .001), together accounted for 18.5% of the variance in insight (R2 = .19, F(2,219) = 26.10, p < .001), supporting the CAM., Conclusions: These studies establish an efficient measure of insight with high clinical utility and inform the literature on the role of insight in predicting performance in those with Alzheimer's pathology., (© The Author(s) 2024. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

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., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Visual anosognosia, associated with confabulations and cortical blindness in the context of occipital lobe injury, is known as Anton syndrome. Patients with this syndrome strongly deny their vision loss and confabulate to compensate for both visual loss and memory impairments. In this article, we present a case of a patient with some similarities to Anton syndrome, however, with several differences in clinical presentation. Bifrontal brain injury, bilateral enucleation, affective indifference (anosodiaphoria), generalized anosognosia, and the conviction that vision will resume mark clear clinical differences with Anton syndrome. Differentiating these findings from Anton syndrome will help occupational therapists, neuropsychologists, speech-language pathologists, physical therapists, and physicians when assessing frontal lobe brain injury with total and partial visual loss. This case demonstrates that visual anosognosia and confabulations can occur without occipital lobe dysfunction or cortical blindness., (© 2024. Fondazione Società Italiana di Neurologia.)

Introduction: Anosognosia, defined as reduced awareness of one's deficit or symptom, is common in Huntington's disease (HD) and detectable at each disease stage. The impact of anosognosia on self-reporting in HD populations is critical to understand given growing use of patient-reported outcomes in HD clinical care and research. We aimed to determine the influence of anosognosia on patient-reported outcome measures assessing psychiatric symptoms and quality of life in HD., Methods: We enrolled HD patients to complete a battery of patient-reported and rater-administered measures, including the Anosognosia Scale, at baseline and 6 months later. Patient-reported outcome measures included NeuroQoL short forms for depression, anxiety, satisfaction with social roles and activities, and positive affect and well-being and Patient-Reported Outcomes Measurement Information System short forms for emotional distress-anger and sleep-related impairment. Anosognosia Scale-Difference Score indexed patient-clinician agreement on patient motor, cognitive, and behavioral abilities. We conducted multivariable linear regression analyses to quantify the association of baseline anosognosia with 6-month patient-reported outcomes., Results: Of 79 patients with complete Anosognosia Scale data at baseline, 25 (31.6 %) met the scale's criterion for anosognosia. In the regression analyses, baseline Difference Score improved prediction of 6-month patient-reported outcomes for depression, anxiety, anger, and positive affect and well-being (χ 2 (1) value range for likelihood ratio tests contrasting models with and without Difference Score: 13.1-20.9, p-values <0.001). Patients with more anosognosia self-reported less severe psychiatric symptoms and more positive affect and well-being., Conclusion: Study results suggest that anosognosia influences patient-reported outcomes for psychiatric symptoms and quality of life in HD populations., Competing Interests: Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: David A. Isaacs reports financial support was provided by Teva Branded Pharmaceutical Products, R&D, Inc. David A. Isaacs reports financial support was provided by National Institute of Neurological Disorders and Stroke. Jessie S. Gibson reports financial support was provided by the National Institute of Nursing Research and the iTHRIV Scholar Program at the University of Virginia. If there are other authors, they declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024. Published by Elsevier Ltd.)

Introduction: Anosognosia, a neurological condition, involves a lack of awareness of one's neurological or psychiatric deficits. Anton Syndrome (AS), an unusual form of anosognosia, manifests as bilateral vision loss coupled with denial of blindness. This systematic review delves into 64 studies encompassing 72 AS cases to explore demographics, clinical presentations, treatments, and outcomes., Materials and Methods: The study rigorously followed PRISMA guidelines, screening PubMed, Google Scholar, and Scopus databases without timeframe limitations. Only English human studies providing full text were included. Data underwent thorough assessment, examining patient demographics, etiological variables, and treatment modalities., Results: Sixty-four studies met the stringent inclusion criteria. Examining 72 AS cases showed a median age of 55 (6 to 96 years) with no gender preference. Hypertension (34.7 %) and visual anosognosia (90.3 %) were prevalent. Stroke (40.3 %) topped causes. Management included supportive (30.6 %) and causal approaches (30.6 %). Improvement was seen in 45.8 %, unchanged in 22.2 %, and deterioration in 11.1 %. Anticoagulation correlated with higher mortality (p < 0.05)., Discussion: AS, an unusual manifestation of blindness, stems mainly from occipital lobe damage, often due to cerebrovascular incidents. The syndrome shares features with Dide-Botcazo syndrome and dates back to Roman times. Its causes range from strokes to rare conditions like multiple sclerosis exacerbation. Accurate diagnosis involves considering clinical presentations and imaging studies, distinguishing AS from similar conditions., Conclusion: This comprehensive review sheds light on AS's complex landscape, emphasizing diverse etiologies, clinical features, and treatment options. Tailored treatments aligned with individual causes are crucial. The study's findings caution against blanket anticoagulation therapy, suggesting a nuanced approach. Further research is pivotal to refine diagnostics and optimize care for AS individuals., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Background: Anosognosia, or unawareness of symptoms, is common in Huntington's disease (HD), but the neuroanatomical basis of this is unknown., Objective: To identify neuroanatomical correlates of HD anosognosia using structural MRI data., Methods: We leveraged a pre-processed dataset of 570 HD participants across the well-characterized PREDICT-HD and TRACK-HD cohort studies. Anosognosia index was operationalized as the score discrepancies between HD participants and their caregivers on the Frontal Systems Behavior Scale (FrSBe)., Results: Univariate correlation analyses identified volumes of globus pallidus, putamen, caudate, basal forebrain, substantia nigra, angular gyrus, and cingulate cortex as significant correlates of anosognosia after correction for multiple comparisons. A multivariable model constructed with stepwise regression that included volumetric data showed globus pallidus volume alone explained more variance in anosognosia severity than motor impairment or CAP score alone., Conclusions: Anosognosia appears to be related to degeneration affecting both cortical and subcortical areas. Globus pallidus neurodegeneration in particular appears to be a key process of importance.

Background: Neurological disorders can present with a vast array of visual disturbances. The constellation of symptoms and findings in this patient prompted workup for unusual causes of both stroke and neurodegenerative disorder., Case Presentation: A woman in her sixties presented with visual disturbances, followed by weakness in her right arm and aphasia three days later. Her close acquaintances had suspected progressive cognitive decline during the previous year. CT and MRI showed an occluded left posterior cerebral artery with a subacute occipito-temporal infarction. The finding of extensive white matter lesions and segmental arterial vasoconstriction necessitated further workup of vasculitis and hereditary small vessel disease, which were ruled out. The stroke aetiology was considered to be atherosclerotic intracranial large vessel disease. FDG-PET scan revealed decreased metabolism in the left hemisphere, and cerebrospinal biomarkers had slightly decreased beta-amyloid. The findings were suggestive of early Alzheimer's disease or primary progressive aphasia, but currently inconclusive., Interpretation: Based on clinical-anatomical correlation, the patient's visual disturbances, in this case right hemianopsia and object agnosia, were solely related to the stroke and not to a neurodegenerative disorder. Knowledge and interpretation of visual agnosias can in many cases be clinically valuable.

A 76-year-old male patient was admitted to our hospital for the treatment of acute cerebral infarction in the right temporal stem, right lateral thalamus, and right pulvinar regions. Although his overall cognitive function was almost normal, he exhibited reduced visual sensitivity in the homonymous lower left quadrant of the visual field, left unilateral spatial neglect (USN), and simultanagnosia. Left USN improved 4 months after the onset of infarction; however, simultanagnosia persisted. To the best of our knowledge, this is the first case of simultanagnosia caused by cerebral infarction in the right temporal stem, right lateral thalamus, and right pulvinar regions.

Visual imagery has a close overlapping relationship with visual perception. Posterior cortical atrophy (PCA) is a neurodegenerative syndrome marked by early impairments in visuospatial processing and visual object recognition. We asked whether PCA would therefore also be marked by deficits in visual imagery, tested using objective forced-choice questionnaires, and whether imagery deficits would be selective for certain properties. We recruited four patients with PCA and a patient with integrative visual agnosia due to bilateral occipitotemporal strokes for comparison. We administered a test battery probing imagery for object shape, size, colour lightness, hue, upper-case letters, lower-case letters, word shape, letter construction, and faces. All subjects showed significant impairments in visual imagery, with imagery for lower-case letters most likely to be spared. We conclude that PCA subjects can show severe deficits in visual imagery. Further work is needed to establish how frequently this occurs and how early it can be found.

Introduction: A precise understanding of the neural substrates underlying tactually-related cognitive impairments such as bilateral tactile agnosia, bilateral agraphesthesia, kinesthetic alexia and kinesthetic reading difficulty is currently incomplete. In particular, recent data have implicated a role for the lateral occipital tactile visual region, or LOtv, in tactile object naming (Amedi et al. Cerebral Cortex 2002). Thus, this study set out to examine the degree to which the LOtv may be involved in tactually-related cognitive impairments by examining two unique cases., Methods: To assess whether LOtv or the visual word form area (VWFA) is involved in tactually-related cognitive impairments, the average activation point of LOtv and that of VWFA were placed on the single-photon emission computed tomography (SPECT) cerebral blood flow images of two patients: one with bilateral associative tactile agnosia, bilateral agraphesthesia, and ineffective kinesthetic reading, and the other with kinesthetic reading difficulty., Results: The average LOtv coordinate was involved in the area of hypoperfusion in both patients, whereas that of VWFA was not included in any of the hypoperfused areas., Conclusions: The results support the view that interruption of LOtv or disconnection to LOtv and to VWFA may cause these tactually-related cognitive impairments. Further, bilateral associative tactile agnosia and bilateral agraphesthesia are attributable toward the damage of the occipital lobe, whereas unilateral or predominantly one-sided associative tactile agnosia and agraphesthesia are attributable toward the damage of the parietal lobe., (© 2022. The Author(s) under exclusive licence to Belgian Neurological Society.)

Alzheimer's disease leads to an alteration of decision-making abilities which may increase risk-taking behaviours, particularly associated anosognosia. Anticipating the progression of the disease raises a number of questions, particularly in relation to aging in place. Our qualitative study aimed to identify the arguments used by older patients with Alzheimer's disease when choosing a place to age. The study included 22 older adults, living at home, and diagnosed as mild dementia. The patients' arguments in favour of ageing in place were based mainly on the preservation of internal security, through the familiarity of places and relations as well as the maintenance of their independence and their lifestyle habits, allowing stability in their daily lives. Despite the identification of memory loss, the associated risks were minimized or hidden from the reflection on the choice of the place to age.

Impaired awareness increases dependency of patients suffering from Alzheimer's Disease (AD) and caregivers' burden but remains insufficiently evaluated in clinical practice. The numerous conceptualisations of this symptomatology (anosognosia, denial, insight…) have only a slight impact on the three main assessment methodologies which are: the patient-caregiver discrepancy; the clinician rating of patients' awareness of illness; and the prediction of performance discrepancy methods. Nevertheless, most of evaluating tools are not validated yet, in particular regarding the clinician rating, leading to contrasted results. Most of recent studies reported positive correlations with apathy and AD severity, and negative relationships with depressive symptoms. Therefore, impaired awareness seems to be mainly influenced by patient's depression and apathy. We discuss these correlates and shared aspects of apathy and impaired awareness from neuroanatomical, clinical and conceptual viewpoints. We also highlight the relevance and limits of quantitative and qualitative assessment methods, in particular phenomenological.

Introduction: Individuals with Huntington's disease (HD) commonly experience anosognosia, a lack of awareness of deficits. Thus, it is important to examine the accuracy of patient vs caregiver ratings on the basis of objective performance-based measures., Methods: The Anosognosia Scale (AS) was given to 33 patients with manifest HD and their caregivers. The AS consists of 8 items in which individuals rate their global abilities relative to same-aged peers. Scores range from very impaired to excellent. Caregiver and patient ratings were then correlated with objective measures., Results: Caregivers' evaluations of patients' cognitive and motoric abilities were more highly correlated with objective measures than patients' ratings. Specifically, caregivers' AS item scores were highly correlated with objective measures of walking (Unified Huntington Disease Rating Scale (UHDRS) tandem walking score [r = 0.57, p = .001] vs. patient [r = 0.39, p = .031]); dexterity (UHDRS pronation supination score [r = 0.55, p = .011] vs. patient [r = 0.18, p = .393]); speech (UHDRS dysarthria score [r = 0.55, p = .004] vs. patient [r = 0.03, p = .854]); memory (MoCA score [r = -.45, p = .048] vs. patient [r = -.11, p = .963]); attention (Trails Making Test A score [r = 0.58, p = .004] vs. patient [r = 0.08, p = .686]); and word retrieval (category fluency ([r = -.58, p = .004] vs. patient [r = -.02, p = 1.00]). Moreover, both the UHDRS total motor score (TMS) (F(1,29) = 7.50, p = .010) and the Mini Mental Status Exam (MMSE) (F(1,31) = 5.40, p = .027) were significant predictors of patient levels of anosognosia., Conclusions: Our findings indicate that caregivers may be better able to rate HD patients' cognitive and motor abilities than patients themselves. Both cognitive and motor severity are significant predictors of levels of anosognosia in HD., Competing Interests: Declaration of competing interest None of the authors have any interests related to this study., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: Though not originally developed for this purpose, the Healthy Aging Brain Care Monitor (HABC-M) seems a valuable instrument for assessing anosognosia in Alzheimer's disease (AD)., Objectives: Our study aimed at 1) investigating the validity of the HABC-M (31 items), and its cognitive, psychological, and functional subscales, in discriminating AD patients from controls; 2) exploring whether the HABC-M discrepancy scores between the self-reports of patients/controls in these different domains and the respective ratings provided by their caregivers/informants correlate with an online measure of self-awareness; 3) determining whether the caregiver burden level, also derived from the HABC-M, could add additional support for detecting anosognosia., Methods: The HABC-M was administered to 30 AD patients and 30 healthy controls, and to their caregivers/informants. A measure of online awareness was established from subjects' estimation of their performances in a computerized experiment., Results: The HABC-M discrepancy scores distinguished AD patients from controls. The cognitive subscale discriminated the two groups from the prodromal AD stage, with an AUC of 0.88 [95% CI: 0.78;0.97]. Adding the caregiver burden level raised it to 0.94 [0.86;0.99]. Significant correlations between the HABC-M and online discrepancy scores were observed in the patients group, providing convergent validity of these methods., Conclusions: The cognitive HABC-M (six items) can detect anosognosia across the AD spectrum. The caregiver burden (four items) may corroborate the suspicion of anosognosia. The short-hybrid scale, built from these 10 items instead of the usual 31, showed the highest sensitivity for detecting anosognosia from the prodromal AD stage, which may further help with timely diagnosis.

Objective: The aim of this study is to verify the functional activation in a patient with anosognosia for hemiplegia following left brain lesions., Methods: We report a case of a 53-year-old right-handed female patient. She came to our rehabilitative unit with a diagnosis of an ischemic major stroke in the left internal carotid artery and important hemiplegia to the right side. She underwent functional magnetic resonance imaging (fMRI), during which she performed a motor imagery task., Results: The fMRI assessment showed an ischemic lesion in the frontotemporal and insular left areas. In the fMRI experiment, we revealed activation of the residual neural patterns of both hemispheres., Conclusion: We underlined an interest in the compensation mechanism that involved neural networks near brain lesions and some areas of the contro-lesional hemisphere, suggesting that the synaptic plasticity permitted an intra and inter-hemispheric reorganization of the cerebral system., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

A 66-year-old left-handed male was admitted to our acute inpatient rehabilitation (AIR) unit following a resection of the right occipito-parietal glioblastoma. He presented with symptoms of horizontal oculomotor apraxia, contralateral optic ataxia and left homonymous hemianopsia. We diagnosed this patient with partial Bálint's syndrome (BS)- oculomotor apraxia, optic ataxia but not simultanagnosia. BS is typically caused by bilateral posterior parietal lesions, but we here describe a unique case due toresection of a right intracranial tumor. A short AIR stay allowed our patient to learn how to compensate for visuomotor and visuospatial deficits, and improved his quality of life significantly.

Method: A comprehensive search of peer-reviewed articles was conducted from September to November 2020 using Google Scholar and PubMed databases. Key terms included "anosognosia," "self-awareness," "traumatic brain injury," and variants thereof. Our search was restricted to articles published in English within the last 25 years, although a few historical articles were included due to scientific merit. Articles were chosen based on methodological quality, inclusion of solely or predominantly msevTBI sample, and relevance to the current topic., Conclusions: Anosognosia is a multifaceted and domain-specific construct that affects the majority of those with msevTBI. It is related to TBI severity, injuries in right-hemispheric and cortical midline regions, specific aspects of executive function, psychological function, and cultural factors. We offer pragmatic advice for clinicians working with this population and discuss implications for the field regarding "best practices" of anosognosia assessment and intervention.

Background: Auditory agnosia refers to the impairments in sound recognition despite intact hearing and written language abilities. When auditory agnosia is specific to spoken language, it can be indicated as pure word deafness (PWD), which is characterized by the isolated difficulty in understanding spoken language, despite preserved reading comprehension, recognition of nonverbal sounds, and production of written and spoken language., Case: A middle-aged man with a high level of education developed a progressive speech disorder initially characterized by isolated phonemic errors during spontaneous speech and later enriched by difficulties in comprehending long sentences. The patient's past medical history was unremarkable except for hypertension. The neuropsychological picture was suggestive of PWD, while cerebrospinal fluid (CSF) analyses lead to a biomarker-based diagnosis of Alzheimer's disease (AD). PWD remained the prevalent cognitive deficit over the subsequent 4 years., Conclusions: This case report shows that the presence of isolated auditory agnosia or PWD should prompt consideration of a diagnosis of AD. It also suggests that the spectrum of atypical presentations of early-onset AD may be larger than what we currently think., (© 2022. Fondazione Società Italiana di Neurologia.)

Objective: To study awareness of problems with one's own Activities of Daily Living (ADL) following stroke by means of a novel instrument-the Visual-Analogue Test for Anosognosia for Activities of Daily Living (VATA-ADL)., Methods: The new test overcomes some of the methodological problems of traditional structured interviews and self-rating questionnaires. In particular, to account for possible verbal communication difficulties, each question is illustrated by a drawing and a 4-point visual-analogue Likert scale. The patient's self-rating is compared with that given by informants (personal or professional caregiver) to acquire a measure of metacognition of one's own problems in performing everyday tasks., Results: The VATA-ADL was validated in 61 dyads of older people and their informants. A group of 80 post-acute stroke patients and their informants then completed the test. Informant ratings correlated highly with traditional ADL scales, the questionnaire items showed high internal consistency (α = .95) and loaded onto one factor. By comparison to informants' assessments, the patients showed a generally poor appreciation of their functional disabilities. Thirty-nine patients overestimated their abilities (anosognosia) whereas nine showed underestimation of their abilities., Conclusions: Anosognosia (overestimation of abilities) for ADL is frequent, even in post-acute stages post-stroke. Some other patients underestimated their abilities, indicating that poor metacognition of one's own abilities in brain damaged patients is bi-directional. Both types of misestimation may have clinical consequences worth considering for the wellbeing of patients and their carers., (© The Author(s) 2022. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

In recent decades, the research traditions of (first-person) embodied cognition and of (third-person) social cognition have approached the study of self-awareness with relative independence. However, neurological disorders of self-awareness offer a unifying perspective to empirically investigate the contribution of embodiment and social cognition to self-awareness. This study focused on a neuropsychological disorder of bodily self-awareness following right-hemisphere damage, namely anosognosia for hemiplegia (AHP). A previous neuropsychological study has shown AHP patients, relative to neurological controls, to have a specific deficit in third-person perspective taking and allocentric stance (the other unrelated to the self) in higher order mentalizing tasks. However, no study has tested if verbal awareness of motor deficits is influenced by perspective-taking and centrism and identified the related anatomical correlates. Accordingly, two novel experiments were conducted with right-hemisphere stroke patients with (n = 17) and without AHP (n = 17) that targeted either their own (egocentric, experiment 1) or another stooge patients (allocentric, experiment 2) motor abilities from a first-or-third person perspective. In both experiments, neurological controls showed no significant difference in perspective-taking, suggesting that social cognition is not a necessary consequence of right-hemisphere damage. More specifically, experiment 1 found AHP patients more aware of their own motor paralysis (egocentric stance) when asked from a third compared to a first-person perspective, using both group level and individual level analysis. In experiment 2, AHP patients were less accurate than controls in making allocentric judgements about the stooge patient, but with only a trend towards significance and with no difference between perspectives. As predicted, deficits in egocentric and allocentric third-person perspective taking were associated with lesions in the middle frontal gyrus, superior temporal and supramarginal gyri, and white matter disconnections were more prominent with deficits in allocentricity. Behavioural and neuroimaging results demonstrate the intersecting relationship between bodily self-awareness and self-and-other-directed metacognition or mentalisation., (Copyright © 2022. Published by Elsevier Ltd.)

Dementia is a neurodegenerative condition with progressive decline in cognitive faculties and associated with different clinical phenomena. Mirror phenomenon in terms of both mirror agnosia and mirror image agnosia wherein there is difficulty in processing and perception of reflected images is not uncommonly seen, and understanding the same can contribute to early diagnosis and prognostication. We report two elderly women with Alzheimer's dementia and frontotemporal dementia, respectively, presenting with features of abnormalities in mirror image processing. The former had features of both mirror agnosia and mirror image agnosia and the latter had predominantly features of mirror image agnosia with preoccupation with her mirror image. Understanding neuroanatomical networks underlying these phenomena can help early identification and subtyping dementia. Clinically differentiating these phenomena from psychotic symptoms of dementia can help in initiating appropriate non-pharmacological measures rather than resorting to use of psychotropics, the use of which may be counterproductive.

Background: Disorders of higher visual processing often impact patients with acquired brain injury. Even with treatment, these vision conditions can cause chronic challenges for patients. Understanding these conditions and their management can help improve functional independence and quality of life., Objectives: To discuss the various disorders of higher visual processing that result from acquired brain injury. Discussion to include classification, evaluation, and treatment techniques available to clinicians., Methods: Peer reviewed journal articles were searched, primarily through PubMed. Articles spanning several decades were included in the review for historical context of these conditions, however an emphasis was placed on more recent publications for purposes of a discussion regarding clinical management of these conditions., Results: Peer-reviewed articles and clinical trials from across several disciplines were included to frame a discussion of this varied group of conditions., Conclusion: Visual processing disorders have debilitating impacts on both the rehabilitation process as well as functional independence. Varied approaches are utilized in the treatment of these conditions with limited success. Understanding the benefits and limitations of both restorative and compensatory treatments will better help clinicians manage patients with these conditions.

From a cohort of 36 patients presenting apperceptive tactile agnosia after first cortical ischemic stroke, 14 showed temporary impairment at admission. A previous multi-voxel analysis of the cortical lesions, using as explanatory variable the course of tactile object recognition performance over the recovery period of 9 months, partitioned the cohort into three subgroups. Of the 14 patients constituting two of the subgroups, 7 recovered from their impairment whereas 7 did not. These two subgroups could not be distinguished at admission. The primary aim of the present study is to present two assessments that can do so. The first assessment comprises a pattern of behavioral measures, determined via principal component analysis, encoded in three tests: picking small objects, macrogeometrical discrimination and tactile object recognition. The receiver operating characteristic curve derived from permutation of the behavioral test scores yielded an 80% probability of correct identification of the patient subgroup and an 8% probability for false identification. As done with the permuted scores, the pattern could predict the persistence of affliction of new stroke patients with tactile agnosia. The second predictive assessment extends our previous evaluation of cortical MRI lesion maps to include subcortical regions. Confirming our previous study, the lesions of the persistently impaired subgroup disrupted significantly the anterior arcuatus fasciculus and associated superior longitudinal fasciculus III in the ipsilesional hemisphere, impeding reciprocal information transfer between supramarginal gyrus and both the ventral premotor cortex and Brodmann area 44. Due to the importance of interhemispheric information transfer in tactile agnosia, we performed a supplementary analysis of tactile object recognition scores. It showed that haptic information transfer from the non-affected to the affected hands in the persistent cases partly restored function during the nine months, possibly following restoration of functional interhemispheric haptic information transfer at the border of posterior corpus callosum and splenium. In conclusion, the combined findings of the cortical lesion at subarea PFt of the inferior parietal lobule and the associated subcortical tract lesions permit almost perfect prediction of persistent impairment of tactile object recognition. The study substantiates the need for combined analysis of both cortical lesions and white matter tract disconnections., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Purpose of Review: Up to 80% of survivors of right brain stroke leave acute care without being diagnosed with a major invisible disability. Studies indicate that a generic cognitive neurologic evaluation does not reliably detect spatial neglect, nor does it identify unawareness of deficit after right brain stroke; this article reviews the symptoms, clinical presentation, and management of these two cognitive disorders occurring after right brain stroke., Recent Findings: Stroke and occupational therapy practice guidelines stress a quality standard for spatial neglect assessment and treatment to reduce adverse outcomes for patients, their families, and society. Neurologists may attribute poor outcomes associated with spatial neglect to stroke severity. However, people with spatial neglect are half as likely to return to home and community, have one-third the community mobility, and require 3 times as much caregiver supervision compared with similar stroke survivors. Multiple randomized trials support a feasible first-line rehabilitation approach for spatial neglect: prism adaptation therapy; more than 20 studies reported that this treatment improves daily life independence. Evidence-based treatment of anosognosia is not as developed; however, treatment for this problem is also available., Summary: This article guides neurologists' assessment of right brain cognitive disorders and describes how to efficiently assemble and direct a treatment team to address spatial neglect and unawareness of deficit., (Copyright © 2021 American Academy of Neurology.)

Abstract: 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., Competing Interests: Financial disclosure statements have been obtained, and no conflicts of interest have been reported by the authors or by any individuals in control of the content of this article., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Reports an error in "Anosognosia for prospective and retrospective memory deficits: Assessment and theoretical considerations" by Silvia Chapman, Nicoletta Beschin, Stephanie Cosentino, Mitchell S. V. Elkind, Sergio Della Sala and Gianna Cocchini ( Neuropsychology , 2019[Oct], Vol 33[7], 1020-1031). In the article (http://dx.doi.org/10.1037/neu0000568), the racial and ethnic description of the participants was missing. The following text has been added to the first paragraph under the "Participants" heading in the "Method" section: "The racial and ethnic distribution of the participants was 76.5% (n = 39) White, 13.7% (n = 7) Black, 5.9% (n = 3) Hispanic, and 3.9% (n = 2) Asian." The online version of this article has been corrected. (The following abstract of the original article appeared in record 2019-33671-001.) Objective: Patients who suffer from memory loss after an Acquired Brain Injury (ABI) may also suffer from anosognosia, or unawareness of their memory loss. How we define and measure anosognosia can have critical implications for its study and clinical assessment. Commonly used measures often lack standardization and reliability checks for responses. Moreover, these methods rely heavily on cognitive abilities (e.g., language abilities) that are often affected after brain injury. The aim of this study is to elucidate how to best conceptualize and detect anosognosia for memory loss by introducing a new method of assessment, the Visual-Analogue Test for Anosognosia for memory impairment (VATAmem). Method: A total of 51 patients ( M = 61 years, M = 13 years of education) with memory difficulties after ABI were recruited from outpatient clinics. A total of 73 informants were also recruited ( M = 51 years old, M = 13 years of education). Both patients and informants evaluated the severity of patients' everyday memory mistakes on the VATAmem, for prospective and retrospective memory deficits by using visual analogue scales, vignettes, and check questions to ensure reliability. Results and Conclusion: A total of 30% of the patients were deemed unaware of their memory deficits. Patients were less aware of their prospective (29%) than their retrospective memory difficulties (18%). The new method of assessment provided by the VATAmem reduced possible false positives and enhanced reliability. We conclude that careful consideration of methodology is a key step to interpreting anosognosia findings within a theoretical framework. (PsycInfo Database Record (c) 2021 APA, all rights reserved).

Background: Amyotrophic lateral sclerosis (ALS) has long been considered a pure motor neurodegenerative disease. However, now, extra-motor manifestations such as cognitive-behavioral disorders are considered not rare and are even a severity factor of the disease. Experiencing anosognosia (i.e., the inability to recognize neurological symptoms) might affect care and treatment compliance in ALS. Regardless, this pivotal feature has been little investigated., Objectives: By comparing patients' and caregivers' reports, we analysed whether patients with ALS would experience a lack of awareness about their executive disorders and their apathy symptoms., Methods: From the ALS reference center in Paris, we included 85 patients (47 men, mean [SD] age 60.5 [12] years and ALS-Functional Rating Scale-revised score 8 to 46) and their primary family caregivers who all completed the Dysexecutive Questionnaire (DEX) and the Apathy Evaluation Scale (AES). Overall scores and answers were compared by agreement/disagreement statistical methods., Results: Caregivers reported higher levels of cognitive-behavioral disorders than did patients, but reports matched when cognitive-behavioral disorders were absent or mild. With published DEX and AES cutoffs, 32% and 51% of patients had executive disorders and apathy, respectively. In these patients with significant impairment, Bland-Altman plots (i.e., visual display agreement that represents the difference between the patient's and caregiver's scores as a function of their average) showed a strong discrepancy between joint reports: patients underestimated their symptoms by a mean bias of -6.81 DEX points (95% confidence interval -11.88, -1.75) and -8.85 AES points (95% confidence interval -11.72, -5.98). We found no clear relationship between bulbar or spinal ALS subtypes and anosognosia., Conclusions: ALS patients with a cognitive-behavioral phenotype show anosognosia by a mismatch between self and proxy reports, which warrants further investigation in neuroimaging. Systematic longitudinal screening of anosognosia is needed to propose targeted psychoeducation in patient-caregiver dyads showing disagreement., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Purpose: Visuospatial disorders (VSDs) are common in Parkinson's disease (PD). VSDs may involve cerebellar vermis, but evidence from functional connectivity (FC) studies is lacking. Here we compared FC between cerebellar vermis and the entire brain between PD patients with or without VSD, and between patients and healthy controls., Methods: Resting-state 3.0-T functional magnetic resonance imaging was performed on 19 controls, 31 PD patients with VSD and 12 PD patients without VSD. Correlations in brain network were calculated between eight regions of interest in the cerebellar vermis (I-VIII) and other voxels in the brain, and voxel-based FC was analyzed. Patients were assessed in terms of cognitive function as well as motor and non-motor symptoms., Results: In both types of patients, cerebellar vermis VIII, IX and X showed positive FC with the default-mode network (DMN), executive control network and sensorimotor network. Cerebellar vermis I and II showed positive FC with the visual network and DMN in controls, but negative FC in PD patients without VSD. Cerebellar vermis X showed negative FC with lobules VIII and IX of the left cerebellar hemisphere in controls, but positive FC in PD patients with VSD., Conclusion: Positive FC connecting the cerebellar vermis VIII and X with associated brain networks in PD patients with VSD may be compensatory activation. PD may involve disruption of functional coupling between the cerebellar vermis and cerebral cortex., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Objectives: Anosognosia is the inability to recognize one's own symptoms. Although dementia with Lewy bodies (DLB) is the second most common degenerative dementia, there is little evidence of memory deficit awareness in this condition. The objectives of this research were to compare anosognosia between individuals with DLB and dementia due to Alzheimer's disease (AD) and to evaluate whether medial temporal atrophy, a marker of AD pathology, could help to explain different rates of anosognosia in DLB and dementia due to AD., Methods/design: This is a cross-sectional study that took place at the Memory Clinic of D'Or Institute for Research and Education (IDOR). Twenty individuals with DLB and 20 with dementia due to AD were included in this study. We assessed anosognosia for memory using an index derived from subjective memory complaints (using the Memory Complaint Questionnaire) and from the performance in memory neuropsychological testing (Rey Auditory Verbal Learning Test). Thirty-one participants also underwent brain Magnetic Resonance Imaging to evaluate hippocampal atrophy with a visual scale (MTA-score [medial temporal atrophy score])., Results: There was no significant difference between groups regarding age, years of education, sex or time of disease. Individuals with DLB had a higher index of anosognosia than dementia due to AD (2.92 and 1.87; p = 0.024), meaning worse awareness of memory deficits. MTA-score was slightly higher in dementia due to AD than in DLB, albeit without statistical significance., Conclusion: Our study was the first to demonstrate that anosognosia for memory is worse in DLB than in dementia due to AD. This finding supports the hypothesis that anosognosia in DLB is a heterogeneous phenomenon., (© 2021 John Wiley & Sons Ltd.)

Alzheimer's continuum biological profiles (A + T - N - , A + T + N - , A + T - N + , and A + T + N + ) were established in the 2018 National Institute on Aging and Alzheimer's Association research framework for Alzheimer's disease (AD). We aim to assess the relation between AT(N) biomarker profiles and brain functional connectivity (FC) and assess the neural correlates of anosognosia. We assessed local functional coupling and between-network connectivity through between-group intrinsic local correlation and independent component analyses. The neural correlates of anosognosia were assessed via voxel-wise linear regression analysis in prodromal AD. Statistical significance for the FC analysis was set at p ≤ 0.05 false discovery rate (FDR)-corrected for cluster size. One hundred and twenty-one and 73 participants were included in the FC and the anosognosia analysis, respectively. The FC in the default mode network is greater in prodromal AD than AD with dementia (i.e., local correlation: T = 8.26, p-FDR < 0.001, k = 1179; independent component analysis: cerebellar network, T = 4.01, p-FDR = 0.0012, k = 493). The default mode network is persistently affected in the early stages of Alzheimer's biological continuum. The anterior cingulate cortex (T = 2.52, p-FDR = 0.043, k = 704) is associated with anosognosia in prodromal AD., (Copyright © 2021 The Author(s). Published by Elsevier Inc. All rights reserved.)

Deficits in olfaction and taste are among the most frequent non-motor manifestations in Parkinson's disease (PD) that start very early and frequently precede the PD motor symptoms. The limited data available suggest that the basis of the olfactory and gustatory dysfunction related to PD are likely multifactorial and may include the same determinants responsible for other non-motor symptoms of PD. This review describes the most relevant molecular and genetic factors involved in the PD-related smell and taste impairments, and their associations with the microbiota, which also may represent risk factors associated with the disease.

Background: In central auditory disorders caused by damage of the cerebral hemispheres, there are cortical deafness and auditory agnosia. Although clinical cases of cortical deafness have been reported, little is known about the hearing problems and localized lesions associated with cortical deafness., Aims/objectives: The aims of our research are to elucidate lesion sites associated with cortical deafness and to clarify why patients with cerebral lesions are not aware of any sound at all., Materials and Methods: Three patients diagnosed as having total loss of hearing participated in this study. We conducted pure-tone audiometry, speech audiometry, distortion product otoacoustic emission (DPOAE), auditory brainstem response (ABR), and brain magnetic resonance imaging (MRI) to diagnose cortical deafness with aphasia tests of these patients., Results: Our studies showed that waves VI and VII as well as waves I to V have normal peak latencies in ABRs in all three patients. In brain MRI, we found complete damage of proximal parts of bilateral auditory radiations in the three patients., Conclusions: We propose 'subcortical deafness' as a subtype of auditory agnosia.

A 57-year-old man presented with a bilateral posterior cerebral artery attack and was visually impaired. He had a hard time identifying familiar faces and shades. He also felt that the familiar building looked different, and complained that it was not possible to tell from visual information alone whether the food was cooked or the kimono fabric was soft.We assessed the patient's visual function using real materials and material images and was diagnosed with visual texture agnosia. There are few reports of visual texture agnosia, detailed evaluation is considered important because perceiving texture is important for activities of daily living.

Vestibular dysfunction, causing dizziness and imbalance, is a common yet poorly understood feature in patients with TBI. Damage to the inner ear, nerve, brainstem, cerebellum and cerebral hemispheres may all affect vestibular functioning, hence, a multi-level assessment-from reflex to perception-is required. In a previous report, postural instability was the commonest neurological feature in ambulating acute patients with TBI. During ward assessment, we also frequently observe a loss of vertigo sensation in patients with acute TBI, common inner ear conditions and a related vigorous vestibular-ocular reflex nystagmus, suggesting a 'vestibular agnosia'. Patients with vestibular agnosia were also more unbalanced; however, the link between vestibular agnosia and imbalance was confounded by the presence of inner ear conditions. We investigated the brain mechanisms of imbalance in acute TBI, its link with vestibular agnosia, and potential clinical impact, by prospective laboratory assessment of vestibular function, from reflex to perception, in patients with preserved peripheral vestibular function. Assessment included: vestibular reflex function, vestibular perception by participants' report of their passive yaw rotations in the dark, objective balance via posturography, subjective symptoms via questionnaires, and structural neuroimaging. We prospectively screened 918 acute admissions, assessed 146 and recruited 37. Compared to 37 matched controls, patients showed elevated vestibular-perceptual thresholds (patients 12.92°/s versus 3.87°/s) but normal vestibular-ocular reflex thresholds (patients 2.52°/s versus 1.78°/s). Patients with elevated vestibular-perceptual thresholds [3 standard deviations (SD) above controls' average], were designated as having vestibular agnosia, and displayed worse posturography than non-vestibular-agnosia patients, despite no difference in vestibular symptom scores. Only in patients with impaired postural control (3 SD above controls' mean), whole brain diffusion tensor voxel-wise analysis showed elevated mean diffusivity (and trend lower fractional anisotropy) in the inferior longitudinal fasciculus in the right temporal lobe that correlated with vestibular agnosia severity. Thus, impaired balance and vestibular agnosia are co-localized to the inferior longitudinal fasciculus in the right temporal lobe. Finally, a clinical audit showed a sevenfold reduction in clinician recognition of a common peripheral vestibular condition (benign paroxysmal positional vertigo) in acute patients with clinically apparent vestibular agnosia. That vestibular agnosia patients show worse balance, but without increased dizziness symptoms, explains why clinicians may miss treatable vestibular diagnoses in these patients. In conclusion, vestibular agnosia mediates imbalance in traumatic brain injury both directly via white matter tract damage in the right temporal lobe, and indirectly via reduced clinical recognition of common, treatable vestibular diagnoses., (© The Author(s) (2020). Published by Oxford University Press on behalf of the Guarantors of Brain.)