Alouette-R normative data for French-speaking school-aged children living in Quebec.

BACKGROUND: The Alouette-R (2005) by Lefavrais is one of the most widely used tools to assess reading skills in French. However, this instrument does not have normative data specific to the French-speaking population of Quebec, Canada. AIMS: The validity of an assessment being strongly compromised when using inappropriate norms, the first objective of this study was to establish local norms for the Alouette-R. The second objective was to provide sensitivity and specificity data for each Alouette-R measure in the French-speaking Quebec population. The third objective was to compare Quebec and French normative data and their sensitivity to better understand the applicability and effectiveness of the Alouette-R test at the regional level. METHODS & PROCEDURES: A total of 347 fluent readers and 48 children with dyslexia from 3rd to 6th grades were recruited from different regions in Quebec. Participants had to read aloud the 265-word text of the Alouette-R in a maximum of 3 min. OUTCOMES & RESULTS: Norms (means, standard deviations and percentiles) by school grades were created for each test measure: reading time, number of words read, number of errors, number of words correctly read, reading accuracy index and reading fluency index. The sensitivity (i.e., the ability to correctly identify children with dyslexia) and specificity (i.e., the ability to correctly identify children without dyslexia) of these measurements were also documented. The norms and their sensitivity were then compared with those of the original French study by Lefavrais in 2005. CONCLUSIONS & IMPLICATIONS: The presence of differences between European and Quebec norms supports the importance of using local norms when assessing language skills. The reading accuracy and fluency indexes are the measurements that best discriminated children with dyslexia from those without a reading disorder in our study. This study will allow clinicians working in Quebec to have a better interpretation of the Alouette-R measurements and ultimately avoid erroneous conclusions resulting from the use of foreign normative data. WHAT THIS PAPER ADDS: What is already known on this subject The Alouette-R is a reading test validated and standardized in France to screen for dyslexia in children. The validity of existing norms with the Quebec population in Canada is questionable due to socio-linguistic differences with the population of France. What this study adds to existing knowledge This study provides for the first time normative and sensitivity/specificity data of the Alouette-R for French-speaking school-aged children living in Quebec. Differences were noted with the normative data from France, which supports the importance of using local normative data when administering reading tests in Quebec. What are the potential or actual clinical implications of this work? When administering the Alouette-R, clinicians in Quebec will now be able to use normative data adapted to the local population, which will limit erroneous conclusions resulting from the use of foreign normative data. In addition, the sensitivity and specificity values reported in the article will allow these clinicians to better interpret their results when screening for a developmental reading disorder.

Sex Differences in Youth with Chronic Tic Disorder and Tourette Syndrome: Evaluation of Tic Severity, Psychological Profiles, and Quality of Life.

Background: Tourette syndrome (TS) and Chronic Tic Disorder (CT) are neurodevelopmental conditions involving motor and/or phonic tics. Youth with tics may encounter feelings of isolation, diminished self-esteem and quality of life, and academic difficulties. A growing body of scientific literature suggests sex differences in youth with tics, but findings have been mixed so far. Because symptom severity peaks around puberty, understanding sex differences in tic manifestations and associated symptoms during this critical period is essential. Therefore, we aimed to assess sex differences related to tic symptoms, action planning styles, quality of life, and externalizing/internalizing symptoms in youth with tics. Methods: Our sample consisted of 66 youths with tics (19 girls) aged 7-14 (mean = 10 years). Youths were assessed with clinical interviews, as well as self- and parent-reported inventories evaluating tic symptoms, psychological profiles, and quality of life. Results: While no differences in tic symptoms were found, girls exhibited lower functional inflexibility, reduced overall functional planning effectiveness, and higher impairment in the psychological well-being subscale than boys. Additionally, girls had reduced general life satisfaction and social self-esteem. Boys reported more explosive outbursts, higher levels of hyperactivity, and more difficulties with self-concept. Conclusions: Our analyses suggested differences in several manifestations associated with tics. This introduces new perspectives that refine our understanding of sex differences. A better understanding of sex differences in tic disorders may eventually improve outcomes for all individuals living with these conditions.

Creativity in the Predominantly Inattentive and Combined Presentations of ADHD in Adults.

Objective: ADHD and its associated inhibition deficits might promote creativity. However, results in the literature are conflicting, possibly due to the heterogeneity of ADHD. To control for this heterogeneity, creativity, and inhibition were investigated in the predominantly inattentive (ADHD-I) and combined (ADHD-C) presentations. Method: Participants were males/females aged 18 to 51, diagnosed with ADHD-I (n = 21), ADHD-C (n = 19), or without ADHD (n = 43). Self-rated Kaufman Domains of Creativity Scale and evaluator-rated figural Torrance Test of Creative Thinking (TTCT) were used for measuring creativity, Stroop task for inhibition, and Conners' Adult ADHD Rating Scales for ADHD symptoms. Results: The ADHD-C group reported higher self-rated creativity than other groups and made more original drawings paired to more abstract titles in the figural TTCT than controls. Conclusion: ADHD-C participants were the most creative. This result was more importantly associated with higher degrees of ADHD symptoms rather than poorer inhibition.

Capturing Subtle Neurocognitive Differences in Children with and without Tourette Syndrome through a Fine-Grained Analysis of Design Fluency Profiles.

BACKGROUND: Tourette syndrome (TS) can be accompanied by neurocognitive impairment. Only a few studies have focused on executive function assessment in TS using design fluency, providing preliminary results. This study aimed to characterize the detailed design fluency profile of children with TS compared with neurotypical children, while addressing the central concern of frequent comorbidities in studies on TS by considering tic severity and attention-deficit/hyperactivity disorder (ADHD) symptoms and diagnosis. METHODS: Sixty-one children aged between 6 and 15 years participated and were divided into a TS group (n = 28 (with ADHD n = 15)) and a control group (n = 33). Our objective was addressed by examining a wide range of measures of the Five-Point-Test, presumably sensitive to frontostriatal dysfunction. The total number of designs, repetitions, repetition ratio, unique designs, and numerical, spatial, and total strategies were examined for the total duration of the test (global measures) and at five equal time intervals (process measures). RESULTS: The TS group produced significantly fewer numerical strategies. Groups did not differ in other global or process measures. ADHD did not affect performance. CONCLUSIONS: Children with TS do not inherently show general executive dysfunction but may present with subtle neurocognitive characteristics here revealed by comprehensive design fluency profiles.

A connectionist study on the role of pitch in infant-directed speech.

Infant-directed speech (IDS) is believed to facilitate language learning. However, the benefit may be either due to clearer acoustic correlates to linguistic structures, or simply increased attention from infants induced by IDS exaggerated prosody. This study investigated the pure effect of IDS pitch on lexical tone learning, with attentional/affective factors removed by using artificial neural networks. Following training with the pitch of Mandarin tones in IDS versus adult-directed speech, the networks yielded equal tonal categorization for both registers. IDS pitch produced no additional linguistic support. IDS pitch appears to strictly play the non-linguistic role of attention/affect, which may indirectly benefit learning.

Neuropsychology supervision: A survey of practices in Quebec and a cross-cultural comparison.

OBJECTIVE: Supervisors in neuropsychology have an ethical responsibility to continuously improve their ability to supervise. Despite a growing interest in the field, there exist little data on the actual practice and few guidelines to help the practitioner through the process of neuropsychology supervision. This study aims to characterize neuropsychology supervisors and their practices in Quebec, Canada and compare these with supervisory practices of supervisors in the United States, with the ultimate aim of offering recommendations to supervisors. METHOD: Seventy-nine neuropsychology supervisors responded to the 20-question online survey of supervisory experience, education, practices, and familiarity with and use of supervision models that was inspired by Shultz and colleagues. RESULTS: Experience in clinical supervision ranged from 0.12 to 35 years and from having supervised 1-150 supervisees. About half of respondents reported having received continuing education in supervision and about two thirds were familiar with at least one type of supervision model. Some supervisory practices were associated with experience, but not with familiarity and utilization of supervision models. Supervisors from Quebec and the U.S. reported a similar frequency of addressing most of the various supervisory competencies with their supervisees. CONCLUSIONS: Based on the competency-based approach we offer a portrait of neuropsychology supervision in Quebec while highlighting some cultural differences with the U.S. Recommendations include focusing more on certain foundational (e.g. reflective practice) and functional competencies (supervision most notably). Neuropsychology supervisors are also encouraged to devote more time to continuing education opportunities in supervision in order to ensure supervisee development and quality care.

Design Fluency in Children with ADHD and Comorbid Disorders.

BACKGROUND: Attention deficit/hyperactivity disorder (ADHD) is often associated with frontal executive impairment in children. Oppositional defiant disorder (ODD) and anxiety disorders (AD) frequently accompany ADHD, but the impact of these comorbid disorders on cognition remains elusive. The five-point test (FPT), a design fluency task, has been shown to be sensitive to neurological damage, specifically to frontal lobe lesions in patients with brain injuries. The purpose of this study was to compare the performances of neurotypical children with that of children with ADHD, ADHD-ODD, and ADHD-AD on the FPT in order to examine whether these groups could be distinguished from one another based on their cognitive profile. METHODS: A total of 111 children aged 8 to 11 years old participated in the study. Six measures from the FPT were used to characterize their performance. RESULTS: Statistically significant differences between groups were observed for five of the six FPT measures. Essentially, children with ADHD-ODD made more repeated designs than the three other groups (control p > 0.001, ADHD p = 0.008, ADHD-AD p = 0.008), while children with ADHD-AD produced fewer total and correct designs than the control and ADHD groups (p = 0.009). CONCLUSIONS: This suggests that comorbidities have an additive impact on the cognitive profile of children with ADHD. Design fluency may be a sensitive measure for capturing the subtle cognitive deficits that are likely to be involved in these disorders.

Kinematic analysis of fast pen strokes in children with ADHD.

In this study, we aimed to determine whether a new measure of fine motor skills, the Pen Stroke Test (PST), can discriminate between children with and without attention-deficit/hyperactivity disorder (ADHD). Twelve children with ADHD and 12 controls age 8-11 were asked to produce handwriting strokes on a digitizer. The sigma-lognormal model derived from the Kinematic Theory of rapid human movements was used to analyze the strokes. Standard measurements of fine motor skills and handwriting were also obtained. Children with ADHD demonstrated poorer motor planning (t0, D) and execution (nbLog) and greater variability in motor control (SNR/nbLog) than did controls. Parameters extracted from the PST were significantly correlated with performance on other motor and handwriting measures. This study provides preliminary evidence that the PST may be useful as a tool for rapidly detecting motor skill problems in the context of ADHD.

Learning phonetic categories by tracking movements.

We explore in this study how infants may derive phonetic categories from adult input that are highly variable. Neural networks in the form of self-organizing maps (SOMs; ) were used to simulate unsupervised learning of Mandarin tones. In Simulation 1, we trained the SOMs with syllable-sized continuous F(0) contours, produced by multiple speakers in connected speech, and with the corresponding velocity profiles (D1). No attempt was made to reduce the large amount of variability in the input or to add to the input any abstract features such as height and slope of the F(0) contours. In the testing phase, reasonably high categorization rate was achieved with F(0) profiles, but D1 profiles yielded almost perfect categorization of the four tones. Close inspection of the learned prototypical D1 profile clusters revealed that they had effectively eliminated surface variability and directly reflected articulatory movements toward the underlying targets of the four tones as proposed by . Additional simulations indicated that a further learning step was possible through which D1 prototypes with one-to-one correspondence to the tones were derived from the prototype clusters learned in Simulation 1. Implications of these findings for theories of language acquisition, speech perception and speech production are discussed.

A Review of the Neuropsychological Dimensions of Tourette Syndrome.

Neurocognitive functioning in Tourette syndrome (TS) has been the subject of intensive research in the past 30 years. A variety of impairments, presumably related to frontal and frontostriatal dysfunctions, have been observed. These impairments were found in various domains, such as attention, memory, executive functions, language, motor and visuomotor functions, among others. In line with contemporary research, other neurocognitive domains have recently been explored in TS, bringing evidence of altered social reasoning, for instance. Therefore, the aims of this review are to give an overview of the neuropsychological dimensions of TS, to report how neuropsychological functions evolve from childhood to adulthood, and to explain how various confounding factors can affect TS patients' performance in neuropsychological tasks. Finally, an important contribution of this review is to show how recent research has confirmed or changed our beliefs about neuropsychological functioning in TS.

Exploring the dynamics of design fluency in children with and without ADHD using artificial neural networks.

The neuropsychology of attention deficit/hyperactivity disorder (ADHD) has been extensively studied, with a general focus on global performance measures of executive function. In this study, we compared how global (i.e., endpoint) versus process (i.e., dynamic) measures of performance may help characterize children with and without ADHD using a design fluency task as a case study. The secondary goal was to compare the sensitivity of standard versus connectionist statistical models to group differences in cognitive data. Thirty-four children diagnosed with ADHD and 37 children without ADHD aged 8-11 years old were tested on the Five-Point Test. The continuous process measure of performance, indexed as the number of produced designs at each consecutive 1 minute interval during 5 minutes, was analyzed against the discrete process measure, that is, the number of designs between first and last intervals and the standard global performance measure of total number of produced designs. Results show that the continuous process measure distinguished the two groups better than the two other measures. The detailed observation of production patterns revealed a decreasing linear trajectory in children without ADHD that contrasts with the flat, but fluctuating productivity pattern of children with ADHD. With regards to the second goal, results show that the connectionist and standard methods are equally sensitive to group differences for the three types of measures. This illustrates the utility of quantitative process measures together with the connectionist method in neuropsychological research and suggests great potential for a dynamical approach to cognition.

A single complete ultrasound investigation of the venous network for the diagnostic management of patients with a clinically suspected first episode of deep venous thrombosis of the lower limbs.

In patients clinically suspected of deep-vein thrombosis (DVT) of the lower limbs, it is safe to withhold anticoagulant therapy after a negative ultrasound (US) limited to the popliteal and the femoral veins, provided that this can either be repeated or combined with other diagnostic procedures. To assess the safety of withholding anticoagulants after a single negative complete US, we performed a multicenter, prospective, cohort study including consecutive ambulatory outpatients from institutional and private practice settings, with a clinically suspected first episode of DVT. Patients fulfilling the inclusion criteria were enrolled after careful clinical assessment. A complete US examination of the proximal and the distal veins was performed according to a standardized and detailed protocol. Anticoagulant therapy was administered in patients with proximal or isolated distal DVT and withheld in those with negative results. The main outcome measure was the occurrence of objectively documented clinical thromboembolic events during a three-month follow-up after a negative US. Out of 623 patients, 401 (64.4%) had a baseline negative US, were not anticoagulated and could be followed-up for three months. Two patients presented a calf DVT within three months. The incidence of venous thromboembolic events, including distal DVT, was 0.5% [95% confidence interval: 0.1-1.8]. No proximal DVT, or non-fatal or fatal pulmonary embolism occurred (incidence: 0.0% [95% confidence interval: 0.0-0.9]). In conclusion, it is safe to withhold anticoagulant therapy in patients with clinically suspected DVT after a single, negative, complete US. Integrating this method within diagnostic strategies for DVT could improve management and be more acceptable for patients and physicians.

Non-contact biomedical photoacoustic and ultrasound imaging.

The detection of ultrasound in photoacoustic tomography (PAT) usually relies on ultrasonic transducers in contact with the biological tissue through a coupling medium. This is a major drawback for important potential applications such as surgery. Here we report the use of a remote optical method, derived from industrial laser-ultrasonics, to detect ultrasound in tissues. This approach enables non-contact PAT (NCPAT) without exceeding laser exposure safety limits. The sensitivity of the method is based on the use of suitably shaped detection laser pulses and a confocal Fabry-Perot interferometer in differential configuration. Reliable image reconstruction is obtained by measuring remotely the surface profile of the tissue with an optical coherence tomography system. The proposed method also allows non-contact ultrasound imaging (US) by applying a second reconstruction algorithm to the data acquired for NCPAT. Endogenous and exogenous inclusions exhibiting optical and acoustic contrasts were detected ex vivo in chicken breast and calf brain specimens. Inclusions down to 0.3 mm in size were detected at depths exceeding 1 cm. The method could expand the scope of photoacoustic and US to in-vivo biomedical applications where contact is impractical.

Intravascular optical coherence tomography on a beating heart model.

The advantages and limitations of using a beating heart model in the development of intravascular optical coherence tomography are discussed. The model fills the gap between bench experiments, performed on phantoms and excised arteries, and whole animal in-vivo preparations. The beating heart model is stable for many hours, allowing for extended measurement times and multiple imaging sessions under in-vivo conditions without the complications of maintaining whole-animal preparation. The perfusate supplying the heart with nutrients can be switched between light scattering blood to a nonscattering perfusate to allow the optical system to be optimized without the need of an efficient blood displacement strategy. Direct access to the coronary vessels means that there is no need for x-ray fluoroscopic guidance of the catheter to the heart, as is the case in whole animal preparation. The model proves to be a valuable asset in the development of our intravascular optical coherence tomography technology.

Simulating the acquisition of lexical tones from continuous dynamic input.

Infants develop phonetic categories by simply being exposed to adult speech. It remains unclear, however, how they handle the extensive variability inherent to speech, and how they process multiple linguistic functions that share the same acoustic parameters. Across four neural network simulations of lexical tone acquisition, self-organizing maps were trained with continuous speech input of increasing variability. Robust tonal categorization was achieved by tracking the velocity profiles of fundamental frequency contours. This result suggests that continuous speech signal carries sufficient categorical information that can be directly processed, and that dynamic acoustic information can be used for resolving the variability problem.

A novel 196Leu to Pro substitution in the beta3 subunit of the alphaIIbbeta3 integrin in a patient with a variant form of Glanzmann thrombasthenia.

Glanzmann thrombasthenia (GT) is an inherited disorder where an absence of platelet aggregation is associated with quantitative or qualitative abnormalities of the alphaIIbbeta3 integrin. In rare patients, amino acid substitutions have provided information on the functional significance of specific domains within alphaIIb or beta3. We now report an elderly male GT patient (R.M.) from the south west of France whose platelets possess a small residual expression of alphaIIbbeta3. Furthermore, the integrin failed to undergo the necessary conformational changes following platelet activation to permit the binding of fibrinogen or activation-dependent monoclonal antibodies despite the presence of an RGD-binding site. Screening of the alphaIIb and beta3 genes by PCR-SSCP revealed a heterozygous mutation at position 685 in exon 5 of the beta3 gene leading to a 196Leu to Pro substitution. 196Leu is a highly conserved amino acid of beta3. The other beta3 allele appeared to be silent. This mutation, inherited from his mother and present in other family members with intermediate levels of alphaIIbbeta3, was close to the MIDAS-like domain of beta3, a fact that appears to explain its effect on alphaIIbbeta3 activation and fibrinogen binding.

Increased soluble and platelet-associated CD40 ligand in essential thrombocythemia and reactive thrombocytosis.

CD40 ligand (CD40L) is expressed on activated CD4(+) T lymphocytes and at the activated platelet surface. A circulating soluble form of CD40L (sCD40L) is generated by the way of a proteolytic cleavage. We measured sCD40L in the plasma of either healthy subjects; patients with inflammatory disorders and low, normal, or high platelet count (reactive thrombocytosis); or patients with essential thrombocythemia (ET). A tight correlation was found between the platelet count and plasma sCD40L. ET patients had the highest levels of sCD40L. Platelet-associated CD40L was increased in ET and reactive thrombocytosis, conditions associated with increased platelet regeneration. Platelet-associated CD40L was released upon platelet activation. In conclusion, platelets appear as a reservoir of CD40L that may be a major contributor to circulating sCD40L. Platelet-associated CD40L may be a potential marker of platelet regeneration.