Atypical crossmodal emotional integration in patients with gliomas.

The relevance of emotional perception in interpersonal relationships and social cognition has been well documented. Although brain diseases might impair emotional processing, studies concerning emotional recognition in patients with brain tumours are relatively rare. The aim of this study was to explore emotional recognition in patients with gliomas in three conditions (visual, auditory and crossmodal) and to analyse how tumour-related variables (notably, tumour localisation) and patient-related variables influence emotion recognition. Twenty six patients with gliomas and 26 matched healthy controls were instructed to identify 5 basic emotions and a neutral expression, which were displayed through visual, auditory and crossmodal stimuli. Relative to the controls, recognition was weakly impaired in the patient group under both visual and auditory conditions, but the performances were comparable in the crossmodal condition. Additional analyses using the 'race model' suggest differences in multisensory emotional integration abilities across the groups, which were potentially correlated with the executive disorders observed in the patients. These observations support the view of compensatory mechanisms in the case of gliomas that might preserve the quality of life and help maintain the normal social and professional lives often observed in these patients.

Strengths and weaknesses of multimodal processing in a group of adults with gliomas.

The present study aimed to analyze the multimodal skills that would be spared, altered, or impaired by gliomas that slowly infiltrate various and diversely localized areas in the cerebral hemispheres. Ten patients and 60 healthy controls were evaluated using four multimodal processing paradigms across 11 tasks. Our objectives were as follows: (a) to describe the strengths and weaknesses of the glioma patients' multimodal processing performance after accounting for task specificity and their individual performances compared to those of the control group; (b) to determine the correlation between lesion localization and impairments; and (c) to identify the tasks that were most sensitive to tumor infiltration and plasticity limits. Our results show that patients as a whole were efficient at most tasks; however, the patients exhibited difficulties in the productive picture-naming task, the receptive verbal judgment task, and the visual/graphic portion of the dual-attention task. The individual case reports show that the difficulties were distributed across the patients and did not correlate with lesion localization and tumor type.

Psychiatric and cognitive phenotype of childhood myotonic dystrophy type 1.

AIM: To investigate the psychiatric and cognitive phenotype in young individuals with the childhood form of myotonic dystrophy type 1 (DM1). METHOD: Twenty-eight individuals (15 females, 13 males) with childhood DM1 (mean age 17y, SD 4.6, range 7-24y) were assessed using standardized instruments and cognitive testing of general intelligence, visual attention, and visual-spatial construction abilities. RESULTS: Nineteen patients had repeated a school grade. The mean (SD) Full-scale IQ was 73.6 (17.5) and mean Verbal IQ was significantly higher than the mean Performance IQ: 80.2 (19.22) versus 72.95 (15.58), p=0.01. Fifteen patients had one or more diagnoses on the DSM-IV axis 1, including internalizing disorders (phobia, n=7; mood disorder, n=6; other anxiety disorders, n=5) and attention-deficit-hyperactivity disorder, inattentive subtype (n=8). Twelve out of 22 patients had alexithymia (inability to express feelings with words and to recognize and share emotional states). Cognitive testing found severe impairments in visual attention and visual-spatial construction abilities in four out of 18, and 14 out of 24 patients respectively. No diagnosis was correlated with the transmitting parent's sex or with cytosine-thymine-guanine (CTG) repeat numbers. Patients with severe visual-spatial construction disabilities had a significantly longer CTG expansion size than those with normal visual-spatial abilities (p=0.04). INTERPRETATION: Children and adolescents with childhood DM1 have frequent diagnoses on DSM-IV axis 1, with internalizing disorders being the most common type of disorder. They also have borderline low intelligence and frequent impairments in attention and visual-spatial construction abilities.

Cerebrotendinous xanthomatosis presenting with severe externalized disorder: improvement after one year of treatment with chenodeoxycholic Acid.

Cerebrotendinous xanthomatosis (CTX) is a rare inborn disorder of sterol storage with autosomal recessive inheritance and a variable clinical presentation. We describe two siblings with an early psychiatric presentation of CTX-associated attention-deficit/hyperactivity disorder and oppositional defiant disorder, also associated with a mild intellectual disability and major behavioral impairments. In both cases, treatment with chenodeoxycholic acid improved externalized symptoms and a partial recovery of cognitive impairments was observed. This suggests that CTX is potentially reversible, demonstrating the need for early diagnosis and treatment of this disorder before irreversible neurological lesions can occur.

Speaking without Broca's area after tumor resection.

We present the case of a right-handed patient who received surgical treatment for a left frontal WHO grade II glioma invading the left inferior and middle frontal gyri, the head of the caudate nucleus, the anterior limb of the internal capsule and the anterior insula, in direct contact also with the anterior-superior part of the lentiform nucleus. The tumor resection was guided by direct electrical stimulation on brain areas, while the patient was awake. Adding a narrative production task to the neuropsychological assessment, we compared pre-, peri- and post-surgical language skills in order to analyze the effects of the tumor infiltration and the consequences of the left IFG resection, an area known to be involved in various language and cognitive processes. We showed that the tumor infiltration and its resection did not lead to the severe impairments predicted by the localization models assigning a significant role in language processing to the left frontal lobe, notably Broca's area. We showed that slow tumor evolution - the patient had been symptom-free for a long time - enabled compensatory mechanisms to process most language functions endangered by the tumor infiltration. However, a subtle fragility was observed in two language devices, i.e., reported speech and relative clauses, related to minor working memory deficits. This case study of a patient speaking without Broca's area illustrates the efficiency of brain plasticity, and shows the necessity to broaden pre-, peri-, post-surgery language and cognitive assessments.

Naming performance in two bilinguals with frontal vs. temporal glioma.

Two bilingual patients had World Health Organization Grade II Gliomas removed from a language area, one in the left mesiofronto-cingular region and one in the left postero-temporal region. They performed a picture naming task in their two languages before their surgery and afterwards. Both patients showed slowness in naming in their first language but different patterns of naming performance across their first and second language. Their patterns depended upon the site of their lesion and their language experience. These data, from brain-damaged, bilingual adult patients, contribute to the neuropsychological literature on brain organization and plasticity, and highlight the importance of assessing naming speed to obtain a better understanding of impairment and recovery mechanisms.

A discrete area within the left dorsolateral prefrontal cortex involved in visual-verbal incongruence judgment.

The role of the frontal lobe in cross-modal visual-auditory processing has been documented in experiments using incongruent/congruent paradigms. In this study, 4 patients with left frontal World Health Organization Grade II glioma were assessed during pre-, intra-, and postoperative sessions with picture-naming and verbal-visual task requiring judgment of congruence between pictures and words. During awake brain surgery, the naming and cross-modal tasks were coupled with electrical stimulation inactivating restricted specific regions. For all patients, focal brain stimulation in the dorsolateral prefrontal cortex elicited picture-word matching disturbances but no naming impairment, and the elicited errors exclusively appeared in incongruent and not congruent conditions. The dissociation observed between correct picture naming and disturbed cross-modal judgment shows that electrical stimulation of a discrete cortical area within the left dorsolateral prefrontal cortex can inhibit the simultaneous processing of visual-verbal information without disturbing larger networks involved in the naming process.

Psychiatric and cognitive phenotype in children and adolescents with myotonic dystrophy.

Myotonic dystrophy type 1 (DM1) is the most frequent inherited neuromuscular disorder. The juvenile form has been associated with cognitive and psychiatric dysfunction, but the phenotype remains unclear. We reviewed the literature to examine the psychiatric phenotype of juvenile DM1 and performed an admixture analysis of the IQ distribution of our own patients, as we hypothesised a bimodal distribution. Two-thirds of the patients had at least one DSM-IV diagnosis, mainly attention deficit/hyperactivity disorder and anxiety disorder. Two-thirds had learning disabilities comorbid with mental retardation on one hand, but also attention deficit, low cognitive speed and visual spatial impairment on the other. IQ showed a bi-modal distribution and was associated with parental transmission. The psychiatric phenotype in juvenile DM1 is complex. We distinguished two different phenotypic subtypes: one group characterised by mental retardation, severe developmental delay and maternal transmission; and another group characterised by borderline full scale IQ, subnormal development and paternal transmission.

Brief report: visual-spatial deficit in a 16-year-old girl with maternally derived duplication of proximal 15q.

Duplications of chromosome 15 may be one of the most common single genetic causes of autism spectrum disorders (ASD), aside from fragile X. Most of the cases are associated with maternally derived interstitial duplication involving 15q11-13. This case report describes a female proband with a maternally derived interstitial duplication of proximal 15q. She did not exhibit any symptoms of ASD apart from some developmental delay. By adolescence, she showed mild dysmorphism, a discrepant profile on the Wechsler Intelligence Scale for Children (Verbal IQ = 87; Performance IQ = 65) and a major deficit in visual-spatial abilities affecting fine motor skills, mathematical reasoning, visual memory and some global reading tasks. This is one of the first reports of a child with a maternal duplication who exhibits a visual-spatial deficit without ASD.

Individual cognitive training of reading disability improves word identification and sentence comprehension in adults with mild mental retardation.

Reading therapy has been shown to be effective in treating reading disabilities (RD) in dyslexic children, but little is known of its use in subjects with mild mental retardation (MR). Twenty adult volunteers, with both RD and mild MR, underwent 60 consecutive weeks in a cognitive remediation program, and were compared with 32 untreated control subjects. The experimental group showed a significant improvement in word identification, as measured by oral production (p=0.0004) or silent reading (p=0.023), and sentence comprehension (p=0.0002). Adults with MR appear to benefit from new approaches in the field of RD.

Compensating for age limits through emotional crossmodal integration.

Social interactions in daily life necessitate the integration of social signals from different sensory modalities. In the aging literature, it is well established that the recognition of emotion in facial expressions declines with advancing age, and this also occurs with vocal expressions. By contrast, crossmodal integration processing in healthy aging individuals is less documented. Here, we investigated the age-related effects on emotion recognition when faces and voices were presented alone or simultaneously, allowing for crossmodal integration. In this study, 31 young adults (M = 25.8 years) and 31 older adults (M = 67.2 years) were instructed to identify several basic emotions (happiness, sadness, anger, fear, disgust) and a neutral expression, which were displayed as visual (facial expressions), auditory (non-verbal affective vocalizations) or crossmodal (simultaneous, congruent facial and vocal affective expressions) stimuli. The results showed that older adults performed slower and worse than younger adults at recognizing negative emotions from isolated faces and voices. In the crossmodal condition, although slower, older adults were as accurate as younger except for anger. Importantly, additional analyses using the "race model" demonstrate that older adults benefited to the same extent as younger adults from the combination of facial and vocal emotional stimuli. These results help explain some conflicting results in the literature and may clarify emotional abilities related to daily life that are partially spared among older adults.

[Altered identification with relative preservation of emotional prosody production in patients with Alzheimer's disease]. / Altération de l'identification de la prosodie émotionnelle avec relative préservation de la production chez des patients atteints de la maladie d'Alzheimer.

Patients with Alzheimer's disease (AD) show cognitive and behavioral disorders, which they and their caregivers have difficulties to cope with in daily life. Psychological symptoms seem to be increased by impaired emotion processing in patients, this ability being linked to social cognition and thus essential to maintain good interpersonal relationships. Non-verbal emotion processing is a genuine way to communicate, especially so for patients whose language may be rapidly impaired. Many studies focus on emotion identification in AD patients, mostly by means of facial expressions rather than emotional prosody; even fewer consider emotional prosody production, despite its playing a key role in interpersonal exchanges. The literature on this subject is scarce with contradictory results. The present study compares the performances of 14 AD patients (88.4±4.9 yrs; MMSE: 19.9±2.7) to those of 14 control subjects (87.5±5.1 yrs; MMSE: 28.1±1.4) in tasks of emotion identification through faces and voices (non linguistic vocal emotion or emotional prosody) and in a task of emotional prosody production (12 sentences were to be pronounced in a neutral, positive, or negative tone, after a context was read). The Alzheimer's disease patients showed weaker performances than control subjects in all emotional recognition tasks and particularly when identifying emotional prosody. A negative relation between the identification scores and the NPI (professional caregivers) scores was found which underlines their link to psychological and behavioral disorders. The production of emotional prosody seems relatively preserved in a mild to moderate stage of the disease: we found subtle differences regarding acoustic parameters but in a qualitative way judges established that the patients' productions were as good as those of control subjects. These results suggest interesting new directions for improving patients' care.

The contribution of phonological awareness and visual attention in early reading and spelling.

We examined the development of phonological processing, naming speed, and visual attention in kindergarten and addressed the question of their contribution to reading and spelling in grade 1. Seventy five French-speaking children were administered seven tasks at the two phases of the study, and reading and spelling were assessed in grade 1. The major findings revealed that syllable awareness and visual attention were the most important predictors of early reading and spelling, and confirm the influence of naming speed and phoneme awareness on specific skills. These observations strongly suggest that written language acquisition relies on linguistic, perceptual and cognitive cross-modal skills and highlight the need for diversifying written language measures and analyzing their specific predictors.

Influence of auditory-verbal, visual-verbal, visual, and visual-visual processing speed on reading and spelling at the end of Grade 1.

This study examined cognitive processing speed through four modalities (auditory-verbal, visual-verbal, visual, and visual-visual) at the end of Grade 1 and how it influences reading and spelling. The subjects were 124 French-speaking children, selected for their contrasting performance on reading and spelling tasks. The children in the first group (N=69) were average readers; the second group of children (N=55) performed worse or much worse on all reading and spelling tasks. The experimental design consisted of a set of 10 tasks administered in two sessions. The major findings reveal that: (1) the children with reading difficulties displayed low and slow performance on most cognitive tasks, whatever the modality; (2) auditory-verbal and visual-verbal processing speed significantly predicted written language, which was not the case with the visual modalities; and (3) that visual problems did not appear to be a potential cause of reading problems in most delayed readers. The findings also confirm the independence of phonological and naming-speed skills in reading development and reading impairment.

Predictive influence of phonological processing, morphological/syntactic skill, and naming speed on spelling performance.

This paper focuses on the predictive influence of phonological awareness, morphological/syntactic skill, and naming speed on spelling. The retrospective study correlated spelling performance in a group of 199 French-speaking children at the end of grade 2 with earlier capacities for phonemic manipulation, morphological/syntactic correction, and naming speed, assessed at the end of grade 1. The results are consistent with an integrative model that challenges the unitary phonological disorder hypothesis and confirmed that in French, as in other languages, naming speed is an independent predictor of reading performance.

The interaction between phonological processing, syntactic awareness, and naming speed in the reading and spelling performance of first-grade children.

The performance of 267 first-grade children was examined on tasks assessing phonological processing, syntactic awareness, and naming speed. The children were also given several measures of word and pseudoword reading, reading comprehension, and pseudoword and dictation spelling. A series of hierarchical analyses indicated that three variables (phonological awareness, syntactic awareness, and naming speed) were still predictors of reading and spelling performance after variance in the others had been controlled for. The results, which confirm that syntactic awareness can account for variance in written language after phonological ability had been controlled for, support the hypothesis concerning the relationships between naming-speed processes and written language, and challenge the unitary phonological theory of reading difficulty.

Oral language deficits in dyslexic children: weaknesses in working memory and verbal planning.

This paper focuses on the relationships among language processing (word- and sentence-level), working memory, and verbal/nonverbal linguistic output. The study examined oral language abilities in a group of 26 French-speaking dyslexic children, compared to two control groups (26 normally developing age-matched children and 26 normally developing younger children). The experimental procedure consisted of tasks involving auditory memory skills (digit span, unfamiliar word repetition, sentence repetition), word retrieval (with semantic, phonological and grammatical criteria), and sentence processing (with verbal and act-out production). The major findings reveal that (a) compared with their age-mates, the dyslexic children exhibited a significant deficit affecting all tasks; and (b) the dyslexic children and the younger controls performed similarly on several tasks. The results are consistent with the processing limitation hypothesis and suggest that the core deficit is the formulation of cognitive plans from auditory input to verbal output.