Preserved emotional awareness of pain in a patient with extensive bilateral damage to the insula, anterior cingulate, and amygdala

Functional neuroimaging investigations of pain have discovered a reliable pattern of activation within limbic regions of a putative "pain matrix" that has been theorized to reflect the affective dimension of pain. To test this theory, we evaluated the experience of pain in a rare neurological patient with extensive bilateral lesions encompassing core limbic structures of the pain matrix, including the insula, anterior cingulate, and amygdala. Despite widespread damage to these regions, the patient's expression and experience of pain was intact, and at times excessive in nature. This finding was consistent across multiple pain measures including self-report, facial expression, vocalization, withdrawal reaction, and autonomic response. These results challenge the notion of a "pain matrix" and provide direct evidence that the insula, anterior cingulate, and amygdala are not necessary for feeling the suffering inherent to pain. The patient's heightened degree of pain affect further suggests that these regions may be more important for the regulation of pain rather than providing the decisive substrate for pain's conscious experience

The epidemiology of mild cognitive impairment (MCI) and Alzheimer’s disease (AD) in community-living seniors: protocol of the MemoVie cohort study, Luxembourg

BACKGROUND: Cognitive impairment and Alzheimer’s disease (AD) are increasingly considered a major public health problem. The MemoVie cohort study aims to investigate the living conditions or risk factors under which the normal cognitive capacities of the senior population in Luxembourg (≥ 65 year-old) evolve (1) to mild cognitive impairment (MCI) – transitory non-clinical stage – and (2) to AD. Identifying MCI and AD predictors undeniably constitutes a challenge in public health in that it would allow interventions which could protect or delay the occurrence of cognitive disorders in elderly people. In addition, the MemoVie study sets out to generate hitherto unavailable data, and a comprehensive view of the elderly population in the country. METHODS/DESIGN: The study has been designed with a view to highlighting the prevalence in Luxembourg of MCI and AD in the first step of the survey, conducted among participants selected from a random sample of the general population. A prospective cohort is consequently set up in the second step, and appropriate follow-up of the non-demented participants allows improving the knowledge of the preclinical stage of MCI. Case-control designs are used for cross-sectional or retrospective comparisons between outcomes and biological or clinical factors. To ensure maximal reliability of the information collected, we decided to opt for structured face to face interviews. Besides health status, medical and family history, demographic and socio-cultural information are explored, as well as education, habitat network, social behavior, leisure and physical activities. As multilingualism is expected to challenge the cognitive alterations associated with pathological ageing, it is additionally investigated. Data relative to motor function, including balance, walk, limits of stability, history of falls and accidents are further detailed. Finally, biological examinations, including ApoE genetic polymorphism are carried out. In addition to standard blood parameters, the lipid status of the participants is subsequently determined from the fatty acid profiles in their red blood cells. The study obtained the legal and ethical authorizations. DISCUSSION: By means of the multidisciplinary MemoVie study, new insights into the onset of cognitive impairment during aging should be put forward, much to the benefit of intervention strategies as a whole

Relations between C9orf72 expansion size in blood, age at onset, age at collection and transmission across generations in patients and presymptomatic carriers

A (GGGGCC) n repeat expansion in C9orf72 gene is the major cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). The relations between the repeats size and the age at disease onset (AO) or the clinical phenotype (FTD vs. ALS) were investigated in 125 FTD, ALS, and presymptomatic carriers. Positive correlations were found between repeats number and the AO (p < 10 e−4 ) but our results suggested that the association was mainly driven by age at collection (p < 10 e−4 ). A weaker association was observed with clinical presentation (p = 0.02), which became nonsignificant after adjustment for the age at collection in each group. Importantly, repeats number variably expanded or contracted over time in carriers with multiple blood samples, as well as through generations in parent-offspring pairs, conversely to what occurs in several expansion diseases with anticipation at the molecular level. Finally, this study establishes that measure of repeats number in lymphocytes is not a reliable biomarker predictive of the AO or disease outcome in C9orf72 long expansion carriers

Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study.

We aimed to accurately estimate the frequency of a hexanucleotide repeat expansion in C9orf72 that has been associated with a large proportion of cases of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD)

Assessing capacity to consent for research in cognitively impaired older patients

International audienceBackground: The number of clinical trials including older patients, and particularly patients with cognitive impairment, is increasing. While statutory provisions exist to make sure that the capacity to consent is assessed systematically for each patient, many gray areas remain with regard to how this assessment is made or should be made in the routine practice of clinical research. Objectives: The aim of this review was to draw up an inventory of assessment tools evaluating older patients' capacity to consent specifically applicable to clinical research, which could be used in routine practice. Methods: Two authors independently searched PubMed, Cochrane, and Google Scholar databases between November 2015 and January 2016. The search was actualized in April 2017. We used keywords (MeSH terms and text words) referring to informed consent, capacity to consent, consent for research, research ethics, cognitive impairment, vulnerable older patients, and assessment tools. Existing reviews were also considered. Results: Among the numerous existing tools for assessing capacity to consent, 14 seemed potentially suited for clinical research and six were evaluated in older patients. The MacArthur Competence Assessment Tool for Clinical Research (MacCAT-CR) was the most frequently cited. Conclusion: The MacCAT-CR is currently the most used and the best validated questionnaire. However, it appears difficult to use and time-consuming. A more recent tool, the University of California Brief Assessment of Capacity to Consent (UBACC), seems interesting for routine practice because of its simplicity, relevance, and applicability in older patients

J ai modifié mon Empathie (Expertise neuropsychologique (Attention et Emotion) du cas NG)

Dans le champ de la psychopathologie, de nombreuses études s intéressent aux complications psychiatriques observées dans l épilepsie partielle pharmaco-résistante temporale. On estime à 31% la prévalence de ces troubles psychiatriques au décours d une lobectomie temporale antérieure. La plupart du temps, il est rapporté des troubles de l humeur et des troubles anxieux. Récemment, quelques études se sont intéressées à l aspect neuropsychologique comportemental dans l épilepsie temporale notamment la reconnaissance des expressions faciales, la cognition sociale et plus particulièrement la théorie de l esprit. Cependant, aucune étude n a porté sur l évaluation de l empathie. Le fil directeur de ce travail de thèse a reposé sur l écoute des plaintes d une patiente (NG) épileptique ayant subi une amygdalo-hippocampectomie droite. Très rapidement après la chirurgie elle a ressenti des modifications de ses émotions et surtout de son empathie. Cette plainte reste présente et inchangée après 13 ans de suivi post-opératoire. Pour expertiser de cette situation singulière, la patiente a réalisé outre un bilan neuropsychologique extensif, des épreuves classiques de psychopathologie cognitive, des épreuves adaptées de la littérature ou des épreuves construites au fil de la recherche clinique guidées par les données recueillies. D autre part, nous nous sommes intéressés à l influence des émotions sur l attention. Ainsi, nous avons étudié la capture attentionnelle de NG pour des stimuli émotionnels. Dans notre étude, nous avons confirmé que les performances de NG dans les tâches d empathie et de théorie de l esprit émotionnelle sont excellentes. De plus nous avons démontré une capture attentionnelle globale pour NG envers les stimuli émotionnels. En évaluant la pertinence de ces stimuli (mots et images), nous avons rapporté un effet majeur de celle-ci sur les ressources attentionnelles de la patiente. Notre travail est le premier, en l état actuel de la littérature, à décrire une amplification spectaculaire de l empathie après une amygdalo-hippocampectomie droite. Cette patiente présente une capacité plus développée à décoder les états mentaux d autrui, notamment leurs émotions. Nous rapprochons l amplification d empathie pour NG avec une dysconnexion fronto-amygdalienne droite. Nous démontrons également que cette révélation d un nouveau comportement peut relever d un biais de capture attentionnelle pour les stimuli émotionnels, traduisant un traitement perceptif différent, plus opérationnel ou sélectif.ST ETIENNE-BU Médecine (422182102) / SudocSudocFranceF

Beauty and Paintings: Aesthetic Experience in Patients with Behavioral Variant Frontotemporal Dementia When Viewing Abstract and Concrete Paintings

We assessed the aesthetic experience of patients with behavioral variant frontotemporal dementia (bvFTD) to understand their ability to experience feelings of the sublime and to be moved when viewing paintings. We exposed patients with bvFTD and control participants to concrete and abstract paintings and asked them how moved they were by these paintings and whether the latter were beautiful or ugly. Patients with bvFTD declared being less moved than control participants by both abstract and concrete paintings. No significant differences were observed between abstract and concrete paintings in both patients with bvFTD and control participants. Patients with bvFTD provided fewer “beautiful” and more “ugly” responses than controls for both abstract and concrete paintings. No significant differences in terms of “beautiful” and “ugly” responses were observed between abstract and concrete paintings in both patients with bvFTD and control participants. These findings suggest disturbances in the basic affective experience of patients with bvFTD when they are exposed to paintings, as well as a bias in their ability to judge the aesthetic quality of paintings

Long Term Neuropsychological Follow-Up in Patients With Herpes Simplex Encephalitis and Predominantly Left-Sided Lesions

Five patients with predominantly dominant cerebral hemisphere lesions due to herpes simplex encephalitis are described. Verbal amnesia was the main deficit but amnesic aphasia sometimes associated with impairment of remote memory also occurred. Semantic and episodic memory deficits were also explored in one case and the role of the right cerebral hemisphere in facilitating recovery of learning is discussed