Influence of anosognosia on patient-reported outcomes for psychiatric symptoms and quality of life in Huntington's disease.

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 (χ2(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.

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.

[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.

Shadowing Behavior May Be Associated with an Inability to Recognize the External World: A Case Report of Shadowing in a Patient with Posterior Cortical Atrophy.

Although shadowing behavior- when one individual closely follows another- is routinely documented among patients with dementia, its mechanisms have yet to be elucidated. In particular, there have been no detailed descriptions of patients with shadowing behavior. To propose its potential backgrounds, we describe a patient with posterior cortical atrophy who exhibited prominent shadowing behavior. He also experienced severe difficulties recognizing external stimuli, including visuospatial dysfunction, several types of agnosia, difficulties in verbal comprehension, disorientation, and its associated depression. This shadowing behavior may be adaptive relative to his extreme difficulty with recognizing the world around him.

[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.

Tactually-related cognitive impairments: sharing of neural substrates across associative tactile agnosia, agraphesthesia, and kinesthetic reading difficulty.

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.

Distinguishing transient from persistent tactile agnosia after partial anterior circulation infarcts - Behavioral and neuroimaging evidence for white matter disconnection.

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.

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.

Awareness is in the eye of the observer: Preserved third-person awareness of deficit in anosognosia for hemiplegia.

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.

Disorders of higher visual processing in patients with acquired brain injury.

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.

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.

Partial Balint's syndrome and left homonymous hemianopsia presenting after resection of a right occipito-parietal glioblastoma.

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.