Nathalie Ehrlé

Dissociation of emotional processes in response to visual and olfactory stimuli following frontotemporal damage.

Contemporary neuropsychological studies have stressed the widely distributed and multicomponential nature of human affective processes. Here, we examined facial electromyographic (EMG) (zygomaticus and corrugator muscle activity), autonomic (skin conductance and heart rate) and subjective measures of affective valence and arousal in patient TG, a 30 year-old man with left anterior mediotemporal and left orbitofrontal lesions resulting from a traumatic brain injury. Both TG and a normal control group were exposed to hedonically valenced visual and olfactory stimuli. In contrast with control subjects, facial EMG and electrodermal activity in TG did not differentiate among pleasant, unpleasant and neutral pictures. In addition, the controls reacted spontaneously with larger corrugator EMG activity and higher skin conductance to unpleasant odors. By contrast, the subjective feeling states (pleasure and arousal ratings) remained preserved in TG. The covariation between facial and self-report measures of negative valence was also a function of the nature of the olfactory task in the patient only. Taken together, the data suggest a functional dissociation between brain substrates supporting generation of emotion and those supporting representation of emotion. We thank TG and the subjects of the control group for their participation in this research.

Recognition of facial and musical emotions in Parkinson's disease

Patients with amygdala lesions were found to be impaired in recognizing the fear emotion both from face and from music. In patients with Parkinsons disease (PD), impairment in recognition of emotions from facial expressions was reported for disgust, fear, sadness and anger, but no studies had yet investigated this population for the recognition of emotions from both face and music.

Juvenile Frontotemporal Dementia with Parkinsonism Associated with Tau Mutation G389R

Frontotemporal lobe degeneration includes a large spectrum of neurodegenerative disorders. Patients with frontotemporal dementia with parkinsonism linked to chromosome 17 exhibit heterogeneity in both clinical and neuropathological features. Here, we report the case of a young patient with a G389R mutation. This teenager girl was 17 years old when she progressively developed severe behavioral disturbances. First, she was considered to be suffering from atypical depression. After 2 years, she was referred to the department of neurology. By this time, the patient exhibited typical frontotemporal dementia with mild extrapyramidal disorders. The main behavioral features included apathy and reduced speech output. MRI and SPECT showed a frontotemporal atrophy and hypofixation, respectively. She died 7 years after onset. Three relatives on her father side had also died after early onset dementia. Genetic testing revealed a heterozygous guanine to cytosine mutation at the first base of codon 389 (Exon 13) of MAPT, the tau gene, resulting in a glycine to arginine substitution, in the patient and her non-affected father. Postmortem neuropathological and biochemical data indicate a Pick-like tau pathology but with phosphoserine 262-positive immunoreactivity. This case is remarkable because of the extremely early onset of the disease.

Présentation d’une batterie d’évaluation des fonctions sociocognitives chez des patients atteints d’affections neurologiques Application dans la démence frontale

This paper presents a French battery designed to assess emotional and sociocognitive abilities in neurological patients in clinical practice. The first part of this battery includes subtests assessing emotions: a recognition task of primary facial emotions, a discrimination task of facial emotions, a task of expressive intensity judgment, a task of gender identification, a recognition task of musical emotions. The second part intends to assess some sociocognitive abilities, that is mainly theory of mind (attribution tasks of mental states to others: false believe tasks of first and second order, faux-pas task) and social norms (moral/conventional distinction task, social situations task) but also abstract language and humour. We present a general description of the battery with special attention to specific methodological constraints for the assessment of neurological patients. After a brief introduction to moral and conventional judgments (definition and current theoretical basis), the French version of the social norm task from RJR Blair (Blair and Cipolotti, 2000) is developed. The relevance of these tasks in frontal variant of frontotemporal dementia (fvFTD is illustrated by the report of the results of a study conducted in 18 patients by the Cambridge group and by the personal study of a patient with early stage of vfFTD. The relevance of the diagnostic of sociocognitive impairment in neurological patients is discussed.

Vieillissement normal : vers une dégradation des représentations structurales, auditives et visuelles, des objets ?

Previous data collected in healthy elderly participants by our laboratory suggested impairment of visual and auditory object processing in normal aging. This impairment seemed not to be related to simple perceptual deficits. The aim of the present study was to identify the mechanism of this disorder according to serial models of object recognition, by dissociating the recognition abilities using naming tasks from a visual and from auditory stimuli, perceptual representations (visual and auditory), and semantic knowledge on the same 62 objects. Fifty three healthy participants were divided in three groups according to age (20-30 years, n = 17; 40-50 years, n = 14; 60-95 years, n = 22). They performed a battery assessing visual and auditory naming, judgement on semantic knowledge and perceptual visual properties, and matching of perceptual auditory properties of the same objects. The aged participants had lower performance on the naming tasks in both modalities, and worse perceptual properties performances again for both modalities than the other two groups. However, no significantly statistical difference was found on the semantic task. A significant correlation was found between age and the scores on each of the four tasks on which aged participants had lower scores than the youngest ones. No statistical difference was found between the two younger groups, but a trend was shown for the perceptual properties. Thus a degradation of perceptual representations of objects seems to be present in normal aging, but the nature of this degradation has to be specified.

[Semantic memory training in Alzheimer's disease].

The purpose of this study was to assess the effects of semantic stimulation of Alzheimers patients on semantic memory comparatively to psychological support. We conducted semantic training with two target categories (musical instruments and human actions), because these concepts were massively failed in previous data collected in Alzheimers disease. Ten patients (57-78 year old, MMSE scores from 17 to 26) were divided in an experimental and a control group where patients received psychological support instead of semantic cognitive training. Semantic abilities were significantly improved in patients from the experimental group, but only after semantic stimulation involving examples from musical instrument category. However, further analysis failed to show an item-specific improvement, suggesting that our results could be explained by a general increase of semantic retrieval. Results could also be explained by some motivational effect caused by more attractive material. Implications for future research and clinical applications are discussed.

Test de compréhension écrite complexe de Chapman-Cook : meilleures performances pour les participants âgés en comparaison des plus jeunes pour les niveaux d’études inférieurs au baccalauréat

We studied the comprehension abilities of healthy participants with a French version of the Chapman-Cook Speed of Reading Test. The objective was to assess the effect of gender, age and educational level on chronometric performances and errors. In this test, the task is to cross out an inappropriate word within short passages. In the original version, the participant is told to perform as quickly as possible during 150 seconds. The score is usually the number of passages correctly completed within this time limit. In the present study, we measured the time to achieve the first 10 passages, the first 14 passages corresponding to the first page and the total (29 passages) corresponding to the two pages. The number of errors was also considered. The normative sample included 150 participants (63 males; 87 females) with three educational level (47: superior to baccalaureate; 21: baccalaureate and 78: inferior to baccalaureate). Age was between 20 and 69 years old, divided in 5 age groups, without neurological or psychiatric disease, or cognitive abnormal development. All were French native speaking and have been schooling in France. For time completion, no effect of gender was found, but a significant and unexpected effect of age was shown according to educational level. Whereas the age groups obtained similar times for educational levels superior to baccalaureate, an age effect was demonstrated for the educational level inferior to baccalaureate. Participants over 40 years of age were faster than younger participants with the same educational level and similar than all age groups of higher educational level. On the contrary, young participants were slower compared to those with high educational levels and all older participants without baccalaureate. This surprising result is discussed.