Generalized anosognosia, anosodiaphoria, and visual hallucinations with bilateral enucleation after severe bifrontal brain injury: a case report describing similarities with and differences from Anton syndrome.

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.

Insights into Anton Syndrome: When the brain denies blindness.

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.

[Willingness to age in place and anosognosia of risks in Alzheimer's disease]. / Volonté de rester à domicile et anosognosie des risques associés à la maladie neurodégénérative de type Alzheimer.

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.

En kvinne i 60-årene som ikke lenger gjenkjente det hun så.

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.

[Simultanagnosia caused by cerebral infarction in the right temporal stem, right lateral thalamus, and right pulvinar regions].

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.

Detecting Anosognosia from the Prodromal Stage of Alzheimer's Disease.

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.

Can a failure in the error-monitoring system explain unawareness of memory deficits in Alzheimer's disease?

Unawareness of memory deficits is an early manifestation in patients with Alzheimer's disease (AD), which often delays diagnosis. This intriguing behavior constitutes a form of anosognosia, whose neural mechanisms remain largely unknown. We hypothesized that anosognosia may depend on a critical synaptic failure in the error-monitoring system, which would prevent AD patients from being aware of their own memory impairment. To investigate, we measured event-related potentials (ERPs) evoked by erroneous responses during a word memory recognition task in two groups of amyloid positive individuals with only subjective memory complaints at study entry: those who progressed to AD within the five-year study period (PROG group), and those who remained cognitively normal (CTRL group). A significant reduction in the amplitude of the positivity error (Pe), an ERP related to error awareness, was observed in the PROG group at the time of AD diagnosis (vs study entry) in intra-group analysis, as well as when compared with the CTRL group in inter-group analysis, based on the last EEG acquisition for all subjects. Importantly, at the time of AD diagnosis, the PROG group exhibited clinical signs of anosognosia, overestimating their cognitive abilities, as evidenced by the discrepancy scores obtained from caregiver/informant vs participant reports on the cognitive subscale of the Healthy Aging Brain Care Monitor. To our knowledge, this is the first study to reveal the emergence of a failure in the error-monitoring system during a word memory recognition task at the early stages of AD. This finding, along with the decline of awareness for cognitive impairment observed in the PROG group, strongly suggests that a synaptic dysfunction in the error-monitoring system may be the critical neural mechanism at the origin of unawareness of deficits in AD.

[Updates on anosognosia in Alzheimer's disease]. / Actualités sur l'anosognosie dans la maladie d'Alzheimer.

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.

Anosognosia in HD: Comparison of self-report and caregiver ratings with objective performance measures.

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.

Anosognosia in hoarding disorder is predicted by alterations in cognitive and inhibitory control.

Insight impairment contributes significantly to morbidity in psychiatric disorders. The neurologic concept of anosognosia, reflecting deficits in metacognitive awareness of illness, is increasingly understood as relevant to psychopathology, but has been little explored in psychiatric disorders other than schizophrenia. We explored anosognosia as an aspect of insight impairment in n = 71 individuals with DSM-5 hoarding disorder. We used a standardized clutter severity measure to assess whether individuals with hoarding disorder underreport home clutter levels relative to independent examiners. We then explored whether underreporting, as a proxy for anosognosia, is predicted by clinical or neurocognitive behavioral measures. We found that individuals with hoarding disorder underreport their clutter, and that underreporting is predicted by objective severity of clutter. In an n = 53 subset of participants, we found that underreporting is predicted by altered performance on tests of cognitive control and inhibition, specifically Go/No-Go and Stroop tests. The relation of underreporting to objective clutter, the cardinal symptom of hoarding disorder, suggests that anosognosia may reflect core pathophysiology of the disorder. The neurocognitive predictors of clutter underreporting suggest that anosognosia in hoarding disorder shares a neural basis with metacognitive awareness deficits in other neuropsychiatric disorders and that executive anosognosia may be a transdiagnostic manifestation of psychopathology.

Motor awareness: a model based on neurological syndromes.

Motor awareness is a complex, multifaceted construct involving the awareness of both (i) one's motor state while executing a movement or remaining still and (ii) one's motor abilities. The analysis of neurological syndromes associated with motor disorders suggests the existence of various different components which are, however, integrated into a model of motor awareness. These components are: (i) motor intention, namely, a conscious desire to perform an action; (ii) motor monitoring and error recognition, that is, the capacity to check the execution of the action and identify motor errors; and (iii) a general awareness of one's own motor abilities and deficits, that is, the capacity to recognize the general state of one's motor abilities about the performance of specific actions and the potential consequences of motor impairment. Neuroanatomical correlates involving the parietal and insular cortices, the medial and lateral frontal regions, and subcortical structures (basal ganglia and limbic system) support this multi-component model. Specific damage (or disconnections) to these structures results in a number of different disorders in motor awareness, such as anosognosia for hemiplegia and apraxia, and a number of symptoms which are specific to motor intention disorders (e.g., the Anarchic Hand Syndrome and Tourette's Syndrome) or motor monitoring (e.g., Parkinson's and Huntington's diseases). All of these clinical conditions are discussed in the light of a motor awareness model.

The making of a syndrome: Gerstmann's patients before Gerstmann syndrome.

When Gerstmann published the case report which later became known as the first case of Gerstmann syndrome, he did not claim the discovery of a new syndrome. It was only a few years later, after reporting on another two similar cases, that he isolated the famous tetrad of symptoms (finger agnosia, right-left disorientation, agraphia and acalculia) as a meaningful cluster with both localising and functional value. In this article, we provide the translation of key-excerpts of the second of Gerstmann's reports (Gerstmann, 1927) and a synoptic description of the symptoms as reported in the three original cases, which were later identified as cases of Gerstmann syndrome. The descriptions appear highly consistent across cases. Among symptoms, finger agnosia stands out for its pervasiveness, which may explain why Gerstmann considered this as the core symptom and speculated it could subtend all symptoms. However, no common functional denominator emerges from the original descriptions.

Pure word deafness: a case report of an atypical manifestation of Alzheimer's disease.

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.

Reflected image processing abnormalities in dementia - a case series and review.

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.

VATA-ADL: The Visual Analogue Test for Anosognosia for Activities of Daily Living.

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.

Anosognosia and Memory Encoding in Huntington Disease.

BACKGROUND: Anosognosia can manifest as an unawareness of neurobehavioral symptoms in individuals with Huntington disease (HD). Measurement of anosognosia is challenging, but the Anosognosia Scale (AS) represents a brief option with promising findings in small samples. OBJECTIVE: To replicate application of the AS in a larger HD sample than previous studies in order to assess psychometrics and demographic correlates and to investigate the genetic, motor, and neuropsychological correlates of the AS in individuals with HD. METHOD: We retrospectively reviewed the AS ratings of 74 genetically confirmed Huntington gene carriers, nearly all early motor manifest, who had been referred for clinical neuropsychological assessment. Concurrent clinical neurologic examination and neuropsychometric assessment data were compiled, where available (ns = 35-74). The severity of the anosognosia per AS ratings was characterized for the HD sample. RESULTS: The AS ratings did not correlate with demographic variables, genetic markers, or motor dysfunction severity. Correlation analyses revealed that higher AS ratings correlated with worse recognition-discrimination memory performance (r = 0.38, P < 0.05) but not cognitive control on executive functioning performance or on collateral-reported frontal-behavioral symptoms. Higher AS ratings also correlated with fewer patient-reported depressive symptoms (r = -0.38, P < 0.01) and diurnal hypersomnia symptoms (r = -0.44, P < 0.01). CONCLUSION: Anosognosia (per AS) is associated with recognition-discrimination deficits and fewer self-reported neuropsychiatric symptoms in individuals with pre-to-early manifest HD, though not with HD severity per genetic or motor markers, nor to executive dysfunction or collateral-reported frontal-behavioral symptoms.

Spatial Neglect and Anosognosia After Right Brain Stroke.

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.

Benson's Disease or Posterior Cortical Atrophy, Revisited.

BACKGROUND: D. Frank Benson and colleagues first described the clinical and neuropathological features of posterior cortical atrophy (PCA) from patients in the UCLA Neurobehavior Program. OBJECTIVE: We reviewed the Program's subsequent clinical experience with PCA, and its potential for clarifying this relatively rare syndrome in comparison to the accumulated literature on PCA. METHODS: Using the original criteria derived from this clinic, 65 patients with neuroimaging-supported PCA were diagnosed between 1995 and 2020. RESULTS: On presentation, most had visual localization complaints and related visuospatial symptoms, but nearly half had memory complaints followed by symptoms of depression. Neurobehavioral testing showed predominant difficulty with visuospatial constructions, Gerstmann's syndrome, and Balint's syndrome, but also impaired memory and naming. On retrospective application of the current Consensus Criteria for PCA, 59 (91%) met PCA criteria with a modification allowing for "significantly greater visuospatial over memory and naming deficits." There were 37 deaths (56.9%) with the median overall survival of 10.3 years (95% CI: 9.6-13.6 years), consistent with a slow neurodegenerative disorder in most patients. CONCLUSION: Together, these findings recommend modifying the PCA criteria for "relatively spared" memory, language, and behavior to include secondary memory and naming difficulty and depression, with increased emphasis on the presence of Gerstmann's and Balint's syndromes.

"Anosognosia for prospective and retrospective memory deficits: Assessment and theoretical considerations": Correction to Chapman et al. (2019).

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).