Physiological, behavioral and subjective sadness reactivity in frontotemporal dementia subtypes.

Frontotemporal dementia (FTD), a neurodegenerative disease broadly characterized by socioemotional impairments, includes three clinical subtypes: behavioral variant FTD (bvFTD), semantic variant primary progressive aphasia (svPPA) and non-fluent variant primary progressive aphasia (nfvPPA). Emerging evidence has shown emotional reactivity impairments in bvFTD and svPPA, whereas emotional reactivity in nfvPPA is far less studied. In 105 patients with FTD (49 bvFTD, 31 svPPA and 25 nfvPPA) and 27 healthy controls, we examined three aspects of emotional reactivity (physiology, facial behavior and subjective experience) in response to a sad film. In a subset of the sample, we also examined the neural correlates of diminished aspects of reactivity using voxel-based morphometry. Results indicated that all three subtypes of FTD showed diminished physiological responding in respiration rate and diastolic blood pressure; patients with bvFTD and svPPA also showed diminished subjective experience, and no subtypes showed diminished facial behavior. Moreover, there were differences among the clinical subtypes in brain regions where smaller volumes were associated with diminished sadness reactivity. These results show that emotion impairments extend to sadness reactivity in FTD and underscore the importance of considering different aspects of sadness reactivity in multiple clinical subtypes for characterizing emotional deficits and associated neurodegeneration in FTD

Crossed Aphasia in a Patient with Anaplastic Astrocytoma of the Non-Dominant Hemisphere

Aphasia describes a spectrum of speech impairments due to damage in the language centers of the brain. Insult to the inferior frontal gyrus of the dominant cerebral hemisphere results in Broca\u27s aphasia - the inability to produce fluent speech. The left cerebral hemisphere has historically been considered the dominant side, a characteristic long presumed to be related to a person\u27s handedness . However, recent studies utilizing fMRI have shown that right hemispheric dominance occurs more frequently than previously proposed and despite a person\u27s handedness. Here we present a case of a right-handed patient with Broca\u27s aphasia caused by a right-sided brain tumor. This is significant not only because the occurrence of aphasia in right-handed-individuals with right hemispheric brain damage (so-called crossed aphasia ) is unusual but also because such findings support dissociation between hemispheric linguistic dominance and handedness. © 2017, EduRad. All rights reserved

Using the Oxford cognitive screen to detect cognitive impairment in stroke patients. A comparison with the Mini-Mental State Examination

Background: The Oxford Cognitive Screen (OCS) was recently developed with the aim of describing the cognitive de cits after stroke. The scale consists of 10 tasks encom- passing ve cognitive domains: attention and executive function, language, memory, number processing, and praxis. OCS was devised to be inclusive and un-confounded by aphasia and neglect. As such, it may have a greater potential to be informative on stroke cognitive de cits of widely used instruments, such as the Mini-Mental State Examination (MMSE) or the Montreal Cognitive Assessment, which were originally devised for demented patients. Objective: The present study compared the OCS with the MMSE with regards to their ability to detect cognitive impairments post-stroke. We further aimed to examine perfor- mance on the OCS as a function of subtypes of cerebral infarction and clinical severity. Methods: 325 rst stroke patients were consecutively enrolled in the study over a 9-month period. The OCS and MMSE, as well as the Bamford classi cation and NIHSS, were given according to standard procedures. results: About a third of patients (35.3%) had a performance lower than the cutoff (<22) on the MMSE, whereas 91.6% were impaired in at least one OCS domain, indicating higher incidences of impairment for the OCS. More than 80% of patients showed an impairment in two or more cognitive domains of the OCS. Using the MMSE as a standard of clinical practice, the comparative sensitivity of OCS was 100%. Out of the 208 patients with normal MMSE performance 180 showed impaired performance in at least one domain of the OCS. The discrepancy between OCS and MMSE was particularly strong for patients with milder strokes. As for subtypes of cerebral infarction, fewer patients demonstrated widespread impairments in the OCS in the Posterior Circulation Infarcts category than in the other categories. conclusion: Overall, the results showed a much higher incidence of cognitive impairment with the OCS than with the MMSE and demonstrated no false negatives for OCS vs MMSE. It is concluded that OCS is a sensitive screen tool for cognitive de cits after stroke. In particular, the OCS detects high incidences of stroke-specific cognitive impairments, not detected by the MMSE, demonstrating the importance of cognitive pro ling.Background: The Oxford Cognitive Screen (OCS) was recently developed with the aim of describing the cognitive deficits after stroke. The scale consists of 10 tasks encompassing five cognitive domains: attention and executive function, language, memory, number processing, and praxis. OCS was devised to be inclusive and un-confounded by aphasia and neglect. As such, it may have a greater potential to be informative on stroke cognitive deficits of widely used instruments, such as the Mini-Mental State Examination (MMSE) or the Montreal Cognitive Assessment, which were originally devised for demented patients. Objective: The present study compared the OCS with the MMSE with regards to their ability to detect cognitive impairments post-stroke. We further aimed to examine performance on the OCS as a function of subtypes of cerebral infarction and clinical severity. Methods: 325 first stroke patients were consecutively enrolled in the study over a 9-month period. The OCS and MMSE, as well as the Bamford classification and NIHSS, were given according to standard procedures. Results: About a third of patients (35.3%) had a performance lower than the cutoff(< 22) on the MMSE, whereas 91.6% were impaired in at least one OCS domain, indicating higher incidences of impairment for the OCS. More than 80% of patients showed an impairment in two or more cognitive domains of the OCS. Using the MMSE as a standard of clinical practice, the comparative sensitivity of OCS was 100%. Out of the 208 patients with normal MMSE performance 180 showed impaired performance in at least one domain of the OCS. The discrepancy between OCS and MMSE was particularly strong for patients with milder strokes. As for subtypes of cerebral infarction, fewer patients demonstrated widespread impairments in the OCS in the Posterior Circulation Infarcts category than in the other categories. Conclusion: Overall, the results showed a much higher incidence of cognitive impairment with the OCS than with the MMSE and demonstrated no false negatives for OCS vs MMSE. It is concluded that OCS is a sensitive screen tool for cognitive deficits after stroke. In particular, the OCS detects high incidences of stroke-specific cognitive impairments, not detected by the MMSE, demonstrating the importance of cognitive profiling. © 2018 Mancuso, Demeyere, Abbruzzese, Damora, Varalta, Pirrotta, Antonucci, Matano, Caputo, Caruso, Pontiggia, Coccia, Ciancarelli, Zoccolotti and The Italian OCS Grou

“Looks familiar, but I do not know who she is”:The role of the anterior right temporal lobe in famous face recognition

Processing a famous face involves a cascade of steps including detecting the presence of a face, recognizing it as familiar, accessing semantic/biographical information about the person, and finally, if required, production of the proper name. Decades of neuropsychological and neuroimaging studies have identified a network of occipital and temporal brain regions ostensibly comprising the 'core' system for face processing. Recent research has also begun to elucidate upon an 'extended' network, including anterior temporal and frontal regions. However, there is disagreement about which brain areas are involved in each step, as many aspects of face processing occur automatically in healthy individuals and rarely dissociate in patients. Moreover, some common phenomena are not easily induced in an experimental setting, such as having a sense of familiarity without being able to recall who the person is. Patients with the semantic variant of Primary Progressive Aphasia (svPPA) often recognize a famous face as familiar, even when they cannot specifically recall the proper name or biographical details. In this study, we analyzed data from a large sample of 105 patients with neurodegenerative disorders, including 43 svPPA, to identify the neuroanatomical substrates of three different steps of famous face processing. Using voxel-based morphometry, we correlated whole-brain grey matter volumes with scores on three experimental tasks that targeted familiarity judgment, semantic/biographical information retrieval, and naming. Performance in naming and semantic association significantly correlates with grey matter volume in the left anterior temporal lobe, whereas familiarity judgment with integrity of the right anterior middle temporal gyrus. These findings shed light on the neuroanatomical substrates of key components of overt face processing, addressing issues of functional lateralization, and deepening our understanding of neural substrates of semantic knowledge

A Systematic Survey of Cognitive-Communicative Evaluations

abstract: Dementia is a syndrome resulting from an acquired brain disease that affects many domains of cognitive impairment. The progressive disorder generally affects memory, attention, executive functions, communication, and other cognitive domains that significantly alter everyday function (Quinn, 2014). The purpose of this research was to gather a systematic review of cognitive-communication assessments and screeners used in assessing dementia to assist in early prognosis. From this review, there is potential in developing a new test to address the areas that people with dementia often have deficits in 1) Memory, 2) Attention, 3) Executive Functions, 4) Language, and 5) Visuospatial Skills. In the field of speech-language pathology, or medicine in general, there is no one assessment that can diagnose dementia. Additionally, this review will explore identifying speech and language characteristics of dementia through speech analytics to theoretically help clinicians identify early signs of dementia.Dissertation/ThesisMasters Thesis Communication Disorders 201

The Nature of Working Memory in Aphasia

Thesis (PhD) - Indiana University, Speech and Hearing, 2007It is well known that many adults with aphasia demonstrate concomitant deficits in higher-level cognitive functions, including attention, executive function, and short-term and working memory. This has led to two premises: (a) the domain-specific hypothesis, in which aphasia is associated with additional cognitive deficits only to the extent that these are dependent upon language; and (b) the domain-general hypothesis, in which aphasia is associated with nonlinguistic cognitive impairments as a consequence of either overlapping anatomy or widespread cortical changes post-insult. The purpose of this research was to disentangle these competing hypotheses with regards to working memory (WM) in adults with aphasia. Like other categories of cognitive impairment in this patient group, past research has identified but failed to elucidate WM impairments in aphasic language processing. Toward this end, 15 adults with left-hemisphere damage and aphasia (LHD) and 12 non-brain-damaged controls (NBD) completed a parametric WM task with systematic variation of psycholinguistic complexity (high-frequency, low-frequency, or non-nameable stimuli) and WM load (0-, 1-, and 2-back). Data were analyzed with respect to the differential impact of these variables within and across subjects and groups. Whereas expected effects of word frequency were elicited in stimulus confrontation naming, LHD subjects were affected only minimally by frequency manipulations during the n-back task. Instead, these subjects demonstrated a significant performance decrement relative to controls with increasing WM load. Moreover, aphasia severity was moderately correlated with WM for non-nameable (i.e., more difficult) but not nameable stimuli. At the theoretical level, these results support a resource-based processing model in aphasia; at the neurobiological level, these findings are consistent with the proposition of widespread cortical connectivity changes irrespective of type or location of brain damage. A secondary purpose of this study was to investigate the reliability of LHD performance on the n-back task, given the known performance variability associated with aphasia and the general dearth of reliability data for higher-level tasks. Results demonstrated that the n-back task is a reliable WM indicator over time for this population

Advances and controversies in frontotemporal dementia: diagnosis, biomarkers, and therapeutic considerations

Frontotemporal dementia comprises a group of clinical syndromes that are characterised by progressive changes in behaviour, executive function, or language. The term frontotemporal lobar degeneration encompasses the neurodegenerative diseases that give rise to these clinical syndromes and involve proteinopathies associated with frontotemporal network dysfunction. Improvements in clinical, genetic, and molecular characterisation have provided new insights into frontotemporal dementia and frontotemporal lobar degeneration, with a much broader range of signs and symptoms at presentation than has been previously considered. Accurate and early diagnosis of frontotemporal dementia is now a possibility due to development of neuropsychological measures with a special focus on social cognition. Advances in plasma and CSF biomarkers, and innovations in structural and functional imaging, will prove useful for future clinical trials in people with frontotemporal dementia

Physiology and neuroanatomy of emotional reactivity in frontotemporal dementia

ABSTRACT AND SUMMARY OF EXPERIMENTAL FINDINGS The frontotemporal dementias (FTD) are a heterogeneous group of neurodegenerative diseases that cause variable profiles of fronto-insulo-temporal network disintegration. Loss of empathy and dysfunctional social interaction are a leading features of FTD and major determinants of care burden, but remain poorly understood and difficult to measure with conventional neuropsychological instruments. Building on a large body of work in the healthy brain showing that embodied responses are important components of emotional responses and empathy, I performed a series of experiments to examine the extent to which the induction and decoding of somatic physiological responses to the emotions of others are degraded in FTD, and to define the underlying neuroanatomical changes responsible for these deficits. I systematically studied a range of modalities across the entire syndromic spectrum of FTD, including daily life emotional sensitivity, the cognitive categorisation of emotions, interoceptive accuracy, automatic facial mimicry, autonomic responses, and structural and functional neuroanatomy to deconstruct aberrant emotional reactivity in these diseases. My results provide proof of principle for the utility of physiological measures in deconstructing complex socioemotional symptoms and suggest that these warrant further investigation as clinical biomarkers in FTD. Chapter 3: Using a heartbeat counting task, I found that interoceptive accuracy is impaired in semantic variant primary progressive aphasia, but correlates with sensitivity to the emotions of others across FTD syndromes. Voxel based morphometry demonstrated that impaired interoceptive accuracy correlates with grey matter volume in anterior cingulate, insula and amygdala. Chapter 4: Using facial electromyography to index automatic imitation, I showed that mimicry of emotional facial expressions is impaired in the behavioural and right temporal variants of FTD. Automatic imitation predicted correct identification of facial emotions in healthy controls and syndromes focussed on the frontal lobes and insula, but not in syndromes focussed on the temporal lobes, suggesting that automatic imitation aids emotion recognition only when social concepts and semantic stores are intact. Voxel based morphometry replicated previously identified neuroanatomical correlates of emotion identification ability, while automatic imitation was associated with grey matter volume in a visuomotor network including primary visual and motor cortices, visual motion area (MT/V5) and supplementary motor cortex. Chapter 5: By recording heart rate during viewing of facial emotions, I showed that the normal cardiac reactivity to emotion is impaired in FTD syndromes with fronto-insular atrophy (behavioural variant FTD and nonfluent variant primary progressive aphasia) but not in syndromes focussed on the temporal lobes (right temporal variant FTD and semantic variant primary progressive aphasia). Unlike automatic imitation, cardiac reactivity dissociated from emotion identification ability. Voxel based morphometry revealed grey matter correlates of cardiac reactivity in anterior cingulate, insula and orbitofrontal cortex. Chapter 6: Subjects viewed videos of facial emotions during fMRI scanning, with concomitant recording of heart rate and pupil size. I identified syndromic profiles of reduced activity in posterior face responsive regions including posterior superior temporal sulcus and fusiform face area. Emotion identification ability was predicted by activity in more anterior areas including anterior cingulate, insula, inferior frontal gyrus and temporal pole. Autonomic reactivity related to activity in both components of the central autonomic control network and regions responsible for processing the sensory properties of the stimuli

Slowing down facial movements and vocal sounds enhances facial expression recognition and facial-vocal imitation in children with autism

International audienceThis study examined the effects of slowing down presentation of facial expressions and their corresponding vocal sounds on facial expression recognition and facial and/or vocal imitation in children with autism. Twelve autistic children and twenty-four normal control children were presented with emotional and non-emotional facial expressions on CD-Rom, under audio or silent conditions, and under dynamic visual conditions (slowly, very slowly, at normal speed) plus a static control. Overall, children with autism showed lower performance in expression recognition and more facial-vocal imitation than controls. Facial expression recognition and facial-vocal imitation were significantly enhanced in slow conditions. Findings may give new perspectives for understanding and intervention for verbal and emotional perceptive and communicative impairments in autistic populations