Au sein des degenerescences lobaires fronto-temporales, il existe actuellement un debat entre deux entites proches : l’aphasie progressive primaire fluente (APPf) et la demence semantique (DS). La DS est caracterisee par une degradation progressive de la memoire semantique et se manifeste par des troubles du langage, une anomie et un trouble de la comprehension des mots isoles, sans atteinte de la phonologie et de la syntaxe, et par des troubles de l’identification visuelle. L’APPf se traduit par des troubles du langage similaires, mais non accompagnes de troubles de l’identification visuelle. Pour certains auteurs, ces deux syndromes cliniques ne seraient en fait qu’une seule et meme entite, l’APPf etant une DS debutant sur le versant verbal. Dans les deux cas, les troubles du langage seraient d’origine semantique. Nous presenterons ici l’etude de cas d’un patient, HD, atteint d’une APPf et nous proposerons une hypothese alternative pour expliquer ses troubles du langage, la degradation progressive de son lexique phonologique.

Aphasia is common in elderly patients in the context of vascular or neurodegenerative disorders. In some cases, aphasia is an isolated symptom, occurring suddenly after a stroke, or developing progressively as a primary progressive aphasia. The diagnosis and treatment are then very similar in older and younger patients. Therapy may be more complicated because of the high prevalence, in older patients, of associated non linguistic symptoms (attentional and dysexecutive symptoms, behavioral and psychological symptoms or sensorial deficits), fatigability, and comprehension deficits. It may then become very difficult to recognize aphasia among all these disorders and to appreciate the physiopathology. A complete evaluation of language, cognitive functions, psychopathology, and behavior is very helpful, as are neuroimaging techniques (MRI is the most relevant). A good knowledge of classical aphasic pictures associated with stroke, Alzheimer disease or related disorders, is highly recommended. Rehabilitation must be proposed even for older patients, so far as aphasia alters the communication abilities. It must be kept in mind that associated symptoms may limit considerably the therapy.

La démence sémantique se définit comme une perte progressive des connaissances sémantiques, responsable d'une difficulté à identifier les objets (et/ou personnes) à partir de leur image et/ou de leur nom. La mémoire au jour le jour est normale et les aspects non sémantiques du langage sont théoriquement préservés. Cependant, les travaux qui ont évalué chez les patients DS l'intégrité des représentations lexicales pré-sémantiques au moyen de la tâche de décision lexicale donnent lieu à des résultats divergents, allant d'une préservation totale à un déficit sévère. L'hypothèse la plus couramment avancée consiste à mettre en relation la dégradation des performances en décision lexicale avec la sévérité des troubles sémantiques. Afin de tester cette hypothèse, nous avons évalué de façon systématique les performances dans cette tâche de 6 patients DS. 6 patients présentant une DS selon les critères de Neary et al (1998) ont participé à l'étude. 2 patients présentent une forme verbale pure de DS et 4 présentent une forme multimodale associant des troubles du langage à des déficits de l'identification visuelle des objets et des personnes. La sévérité des troubles sémantiques était attestée par la performance au Pyramid et Palm Trees test, dans sa forme verbale. La tâche de décision lexicale comportait des mots de haute et basse fréquence et des non mots légaux, appariés en longueur et fréquence des graphèmes, présentés oralement. Les performances en décision lexicale sont variables et vont d'une préservation totale à des performances très déficitaires. Il n'existe aucune corrélation avec la sévérité des troubles sémantiques mesurés par la performance au PPTT verbal. En revanche, la performance est corrélée avec le profil des troubles : la décision lexicale est très déficitaire chez les 2 patients présentant une forme atypique (verbale pure) selon les critères de Moreaud et al (2008), alors qu'elle est préservée dans les DS typiques (multimodales), qui présentent pourtant des déficits sémantiques plus sévères. Ce résultat suggère que la forme verbale pure et la forme multimodale de démence sémantique pourraient représenter deux entités cliniques distinctes sous-tendues par des déficits fonctionnels différents.

Semantic dementia (SD) is a syndrome of progressive loss of semantic knowledge for objects and people. International criteria propose that SD be included in the frontotemporal lobar degeneration syndromes, with progressive non-fluent aphasia and frontotemporal dementia (FTD). However, several related syndromes have been defined that clinically and conceptually share both similarities and differences with SD: fluent progressive aphasia, progressive prosopagnosia, temporal variant of FTD. In order to establish a French consensus for the diagnosis and modalities of evaluation and follow-up of SD, a working group, composed of neurologists, neuropsychologists and speech-therapists, was established by the Groupe de reflexion sur les evaluations cognitives (GRECO). New criteria were elaborated, based on clinical, neuropsychological, and imaging data. They define typical and atypical forms of SD. A diagnosis of typical SD relies on an isolated and progressive loss of semantic knowledge, attested by a deficit of word comprehension and a deficit of objects and/or people identification, with imaging showing temporal atrophy and/or hypometabolism. SD is atypical if the deficit of semantic knowledge is present only within a single modality (verbal versus visual), or if non-semantic deficits (mild and not present at onset) and/or neurological signs, are associated with the semantic loss. (C) 2008 Elsevier Masson SAS. Tous droits reserves.

We report a case of massive associative visual agnosia. In the light of current theories of identification and semantic knowledge organization, a deficit involving both levels of structural description system and visual semantics must be assumed to explain the case. We suggest, in line with a previous case study [1], an alternative account in the framework of (non abstractive) episodic models of memory [4].

Primary progressive aphasia (PPA), initially described by Mesulam, is a syndrome of progressive deterioration of language, occurring in the presenium. Several classifications have been proposed, but the most useful one distinguishes non fluent and fluent forms of PPA. Both begin by anomia. In non fluent PPA, there is a progressive reduction of language, sometimes with aggramatism and articulatory impairment, but without impairment of comprehension. Fluent PPA is characterized by preserved fluency with severe impairment of single word comprehension. It is frequently confounded with semantic dementia (SD), but some observations can be differentiated from SD. After several years, all patients become mute, and most of them develop dementia, usually of frontal lobe type. The syndrome of PPA is due to neurodegenerative brain pathology affecting mostly the perisylvian regions of the left hemisphere. In most cases, no Alzheimer type pathology was found in the brain, but tauopathy (mainly in non fluent PPA) or motor neuron disease type pathology - tau negative ubiquitin inclusions (mainly in fluent PPA). However, Alzheimer type pathology was found in a substantial number of cases.

The aim of this study was to evaluate neurologists' reliability in recognizing retrospectively a diagnosis of psychogenic status and status epilepticus (SE) based solely on clinical semiology, as reported in medical charts.This is a retrospective analysis of medical records of patients with suspected SE, diagnosed with psychogenic status and SE, proven by video-electroencephalography (EEG) monitoring, over a two-year period, from January 1st 2012 to December 31st 2013. Eight additional patients outside this time frame were included in this series because they had video-EEG proven psychogenic status, and they met all the inclusion criteria. The group with SE was divided into symptomatic SE (SSE) if a precipitating factor was identified, and undetermined SE (USE) if none were identified. Twenty-two neurologists from the CHU de Grenoble-Alpes were asked to fill out a survey where they were asked to score, for each patient, their agreement, using Likert scales, for the respective diagnoses of psychogenic status and SE. Their opinions were based on a provided written sheet summarizing the clinical description of the event and patients' clinical context. Neurologists were blinded to video-EEG monitoring results and final diagnosis. The level of agreement, disagreement, and the homogeneity of neurologist's responses according to the final diagnosis were then calculated. Finally, clinical data, as provided in the event's clinical description and context, considered as highly relevant by neurologists to establish an accurate diagnosis were gathered.Eighteen neurologists completed the survey for 48 patients, including 11 diagnosed with psychogenic status and 37 with SE (30 with SSE and 7 with USE). For patients diagnosed with SE, the presence of a precipitating factor increased the likelihood and the homogeneity among neurologists of a diagnosis of SE (77%), with a specificity (Sp) of 96% and a positive predictive value of 95%. The lack of a precipitating factor significantly decreased the diagnosis likelihood of SE (55%) with a predictive value of 82%. For patients diagnosed with psychogenic status, most of neurologists agreed with the diagnosis of psychogenic status (69%) with a predictive value of 82%, although heterogeneity in the diagnosis was found. According to neurologists participating in this study, most significant terms, found in the medical charts, helping to distinguish SE from psychogenic status were "stereotypical movements", "limb myoclonus", "epilepsy", and "vigilance alteration". To differentiate psychogenic status from SE, most relevant terms used by neurologists were "resistance to eyes opening", "anarchic movements", "prolonged motor manifestations", "limb tremor" and "opisthotonus". However, analysis of the distribution of the terms among the different groups (SSE, USE, and psychogenic status) showed no significant difference.This study is in line with previous literature highlighting the difficulty in retrospectively differentiating SE from psychogenic status based on clinical events description recorded in the medical chart.

We report the case of a 71 year-old female patient (DC) with a left parietal lesion resulting in a peripheral dysgraphia essentially characterized by difficulties in letter sequences writing. The aim of our experiments was to analyze the influence of motor difficulties on the visual perception of both writing and reaching movements. Results showed a strong link between motor and perceptual performance. For reaching movements, performances in both production and perception tasks conform to the motor principles identified in healthy subjects (Fitts' law and motor anticipation). By contrast, for handwriting movements, DC's productions do not follow the motor principles usually observed in normal subjects (isochrony principle, motor anticipation) and in perception the same results were observed. The motor references used by DC in the visual perception of writing movement were not the laws of movement but rather her own way of writing. Taken together these data strongly suggest that motor competences is involved in the visual perception of human movements. They are discussed in the general framework of the simulation theory.

While many with schizophrenia spectrum disorders experience difficulties understanding the feelings of others, little is known about the psychological antecedents of these deficits. To explore these issues we examined whether deficits in mental state decoding, mental state reasoning and metacognitive capacity predict performance on an emotion recognition task. Participants were 115 adults with a schizophrenia spectrum disorder and 58 adults with substance use disorders but no history of a diagnosis of psychosis who completed the Eyes and Hinting Test. Metacognitive capacity was assessed using the Metacognitive Assessment Scale Abbreviated and emotion recognition was assessed using the Bell Lysaker Emotion Recognition Test. Results revealed that the schizophrenia patients performed more poorly than controls on tests of emotion recognition, mental state decoding, mental state reasoning and metacognition. Lesser capacities for mental state decoding, mental state reasoning and metacognition were all uniquely related emotion recognition within the schizophrenia group even after controlling for neurocognition and symptoms in a stepwise multiple regression. Results suggest that deficits in emotion recognition in schizophrenia may partly result from a combination of impairments in the ability to judge the cognitive and affective states of others and difficulties forming complex representations of self and others.

The present study describes two Frenchteenagers with developmental reading andwriting impairments whose performance wascompared to that of chronological age andreading age matched non-dyslexic participants.Laurent conforms to the pattern of phonologicaldyslexia: he exhibits a poor performance inpseudo-word reading and spelling, producesphonologically inaccurate misspellings butreads most exception words accurately. Nicolas,in contrast, is poor in reading and spelling ofexception words but is quite good atpseudo-word spelling, suggesting that hesuffers from surface dyslexia and dysgraphia.The two participants were submitted to anextensive battery of metaphonological tasks andto two visual attentional tasks. Laurentdemonstrated poor phonemic awareness skills butgood visual processing abilities, while Nicolasshowed the reverse pattern with severedifficulties in the visual attentional tasksbut good phonemic awareness. The presentresults suggest that a visual attentionaldisorder might be found to be associated withthe pattern of developmental surface dyslexia.The present findings further show thatphonological and visual processing deficits candissociate in developmental dyslexia.

There is considerable debate on the extent to which reading and spelling rely on shared versus distinct cognitive processes (Tainturier & Rapp, 2001). Studies of brain-damaged individuals with impaired access to orthography have supported both views. Of particular interest are cases of "surface dyslexia," a disorder characterised by a dissociation between impaired reading of irregular words and relatively preserved reading of regular words and nonwords. Most errors are regularisations (e.g., CHEF--> "tchef"). Surface dyslexia is believed to result from a deficit at the level of the orthographic lexicon, a memory store of the spelling of familiar words. The accurate reading of regular words and nonwords is thought to reflect the use of a spared non-lexical route to reading. Attempting to read irregular words through this route will result in regularisation errors because the sublexical process maps graphemes to their most common pronunciation (i.e., CH pronounced "tch" rather than "sh"). Advocates of shared orthographic lexicons (e.g., Behrmann & Bub, 1992) have stressed the fact that patients with surface dyslexia usually also suffer from "surface dysgraphia," a disorder affecting words with ambiguous/irregular spellings and manifesting itself by the production of phonologically plausible errors such as "knowledge" --> NOLIGE. However, advocates of distinct reading/spelling lexicons (e.g., Caramazza, 1988) have pointed out that some patients have surface dysgraphia without surface dyslexia. This might suggest impaired access to an orthographic output lexicon used in spelling with preserved access to an orthographic input lexicon used in reading and written word recognition. We report the first case of a patient who presents with the opposite dissociation, that is, prototypical surface dyslexia with entirely preserved spelling. (C) 2002 Elsevier Science (USA). All rights reserved.

Two main hypotheses have been proposed regarding the role of phonology in written word production. According to the phonological mediation hypothesis, the retrieval of the lexical phonological representation of a word is an obligatory prerequisite to the retrieval of its spelling. Therefore, deficits to the phonological lexicon should affect both spoken and written picture naming, In contrast, the orthographic autonomy hypothesis posits that the lexical orthographic representations of words can be accessed without any necessary phonological mediation. In support of this view, cases of preserved written naming despite impaired lexical phonology have been reported following brain damage. In this report, we replicate this basic pattern of performance in case YP, a 60-year-old woman with a pattern of frontotemporal dementia, As her disease progressed, YP's ability to write down the names of pictures remained very good despite a severe decline in oral naming. Further testing indicated that this deficit was not primarily due to an articulatory or post-lexical phonological deficit. YP's case provides strong additional support for the orthographic autonomy hypothesis. The significance of this case with respect to the characterization of dementia syndromes is discussed.

Patients with Alzheimer's disease (AD) produce a high rate of semantic errors when naming to confrontation. This is considered to be one of the many consequences of their semantic memory deficit. However, it has been shown, in aphasic patients with focal lesions, that semantic errors could arise from impairment to any one of the levels in the naming process. To check this hypothesis in AD, we assessed in 15 patients the capacity to name and access semantic knowledge (by multiple-choice probe questions) about 14 objects presented successively in the visual, tactile, auditory, and verbal modalities. In the visual naming task, 33 errors were recorded: 26 (78.8%) were semantic and 7 (21.2%) were unrelated errors. Of the 26 semantic errors, 8 were related to a deficit of the semantic knowledge related to the item and 17 to a deficit in the retrieval of the phonological form of the word. One was associated with a deficit of access to semantic knowledge in the visual modality. The 7 unrelated errors were associated with a loss of semantic knowledge for 4 and deficit of access to the phonological form for 3. In conclusion, this study shows that semantic errors do not systematically reflect a deficit of semantic knowledge in Alzheimer's disease. It also seems that unrelated errors are more frequently related to semantic deficits than semantic errors in this population.