Long-term outcomes of paediatric Guillain-Barré syndrome.

AIM: To study long-term sequelae in children with Guillain-Barré syndrome (GBS). METHOD: This was a prospective observational study with children from two French tertiary centres. Data were from clinical and several standardized scales or questionnaires. RESULTS: Fifty-one patients were included with a median follow-up of 6 years 4 months (range 3-20 years) after the acute phase. The sequelae rate was 67% (95% confidence interval [CI] 53-78) and did not vary with time. Most children had minor sequelae (Guillain-Barré Syndrome Disability Score [GBSDS] = 1); only one was unable to run (GBSDS = 2). The most frequent complaints were paraesthesia (43%), pain (35%), and fatigue (31%). The neurological examination was abnormal in 18% of children, autonomy was compromised in 14%, and symptoms of depression occurred in 34%. The factors associated with late-onset sequelae were correlated with severity during the initial phase (i.e. initial GBSDS >4, odds ratio 6.6, 95% CI 1.8-33; p = 0.009). The predictive factors of more severe late-onset conditions were initial severity (p = 0.002) and sex (female patients; p = 0.01). INTERPRETATION: Two-thirds of children with GBS had late-onset sequelae following an episode, often minor, but sometimes with continuing effects on their everyday lives. Particularly affected were those who had severe GBS during the acute phase and who lost the ability to walk. WHAT THIS PAPER ADDS: Two-thirds of children with Guillain-Barré syndrome (GBS) had persistent sequelae. Sequelae were often minor, but daily repercussions of them were sometimes serious. Sequelae were significantly associated with severe GBS during the acute phase.

Confirmatory validation of the french version of the Duchenne Muscular Dystrophy module of the pediatric quality of life inventory (PedsQLTM3.0DMDfv).

Duchenne Muscular Dystrophy (DMD) is a neuromuscular disease that inevitably leads to total loss of autonomy. The new therapeutic strategies aim to both improve survival and optimise quality of life. Evaluating quality of life is nevertheless a major challenge. No DMD-specific quality of life scale to exists in French. We therefore produced a French translation of the English Duchenne Muscular Dystrophy module of the Pediatric Quality of Life Inventory (PedsQLTMDMD) following international recommendations. The study objective was to carry out a confirmatory validation of the French version of the PedsQLTMDMD for paediatric patients with DMD, using French multicentre descriptive cross-sectional data. The sample consisted of 107 patients. Internal consistency was acceptable for proxy-assessments, with Cronbach's alpha coefficients above 0.70, except for the Treatment dimension. For self-assessments, internal consistency was acceptable only for the Daily Activities dimension. Our results showed poor metric qualities for the French version of the PedsQLTMDMD based on a sample of about 100 children, but these results remained consistent with those of the original validation. This confirms the interest of its use in clinical practice.

Psychomotor intervention to improve handwriting skills in children with ADHD: A single-case experimental design with direct inter-subject and systematic replications.

Dysgraphia is highly prevalent in children with attention deficit hyperactivity disorder (ADHD) and adversely affects academic and developmental trajectories. To date, no study has rigorously examined the effects of a training program on handwriting quality in this specific population. Our objective was thus to develop an innovative program - we entitled PRO-PEN - and to evaluate its effects. We planned a multiple-baseline design across participants from grade 3-5, with direct inter-subject and systematic replications. Children of Group 1 (n = 4) were diagnosed with ADHD. Systematic replication was conducted in a second group of participants (Group 2, n = 4) with a diagnosis of developmental coordination disorder in addition to ADHD. The primary assessment focused on quality of handwriting. Generalization measures evaluated diverse neuropsychological and behavioural domains. In Group 1, effect sizes regarding handwriting quality were large (Taus > .60). Improvement was also observed for children of Group 2 (Taus > .50). Importantly, the positive effects persisted three months after the end of the training. Generalization effects extended beyond handwriting sphere. Therefore, PRO-PEN can be considered a promising training program for improving handwriting quality in ADHD, with a possible impact on wide cerebral regulation loops underpinning both handwriting and other neuropsychological and behavioural domains.

Procedural learning and retention of audio-verbal temporal sequence is altered in children with developmental coordination disorder but cortical thickness matters.

Rhythmic abilities are impaired in developmental coordination disorder (DCD) but learning deficit of procedural skills implying temporal sequence is still unclear. Current contradictory results suggest that procedural learning deficits in DCD highly depend on learning conditions. The present study proposes to test the role of sensory modality of stimulations (visual or auditory) on synchronization, learning, and retention of temporal verbal sequences in children with and without DCD. We postulated a deficit in learning particularly with auditory stimulations, in association with atypical cortical thickness of three regions of interesting: sensorimotor, frontal and parietal regions. Thirty children with and without DCD (a) performed a synchronization task to a regular temporal sequence and (b) practiced and recalled a novel non-regular temporal sequences with auditory and visual modalities. They also had a magnetic resonance imaging to measure their cortical thickness. Results suggested that children with DCD presented a general deficit in synchronization of a regular temporal verbal sequence irrespective of the sensory modality, but a specific deficit in learning and retention of auditory non-regular verbal temporal sequence. Stability of audio-verbal synchronization during practice correlated with cortical thickness of the sensorimotor cortex. For the first time, our results suggest that synchronization deficits in DCD are not limited to manual tasks. This deficit persists despite repeated exposition and practice of an auditory temporal sequence, which suggests a possible alteration in audio-verbal coupling in DCD. On the contrary, control of temporal parameters with visual stimuli seems to be less affected, which opens perspectives for clinical practice.

Behavioral Parent Training for School-Aged Children With ADHD: A Systematic Review of Randomized Control Trials.

BACKGROUND: All guidelines for the treatment of ADHD in children include behavioral parent training in combination with other strategies. In the past, several systematic reviews have been carried out that were either outdated or not sufficiently specific to ADHD. We wanted to conduct a new review focusing on a specific ADHD population of school age (4-12 years) and on behavioral and cognitive programs. We aimed to test our hypothesis that behavioral parent training would improve parents' difficulties, children's symptomatology, and the quality of life of families with ADHD. METHODS: PUBMED, PsychInfo, Web of Science, ERIC, and Cochrane databases were searched for original articles on randomized control trials on behavioral parent training group for children with ADHD aged from 4 to 12 years until July 2023. RESULTS: A total of 20 studies were included in the systematic review. The results were divided into four categories: parent data, child data collected by parents, teachers, or researchers. A qualitative analysis revealed for parents, effects on parental stress, feelings of parental efficacy, and negative parental educational behavior. As regard children, only effects are noted for parental assessment, on ADHD symptomatology, externalized disorders, and social skills. CONCLUSION: Despite the heterogeneity or small number of studies in some categories, BPTs have positive effects on both parents and children. There are no convincing results to support the generalization of progress. This would seem to indicate that it remains essential to consider actions specific to each problematic environment for the child.

PENDAH program for parents with children with attention deficit hyperactivity disorder. French adaptation of a behavioral parent training group: pilot study.

Behavioral parent training (BPT) is recognized as an effective part of the care offered to children with attention deficit hyperactivity disorder (ADHD). The aim of this pilot study was to objectively examine the effect that this intervention may have on motor activity, in addition to the measures classically found in this type of study. Parents of 24 school-aged children (6-12 year) with ADHD who met eligibility criteria were enrolled in the study. Before, after and five months after the intervention, we used three-dimensional accelerometers over one-week periods to measure the children's motor activity, and questionnaires for parental stress, quality of life, ADHD symptoms, anxiety and sensory disorders. To measure motor activity, a control group of normotypic children matched for age, sex and socio-professional category was set up. The experimental group showed slight decreases in motor activity compared with the control group, particularly in the classroom. The intervention showed improvements for parents in average stress and quality of life, and for children in average intensity global ADHD symptom, inattention, opposition and aggression, in line with previous studies on the effectiveness of BPT. The trial is the first clinical study to assess the effects of BPT on motor activity in children with ADHD.

Is There a Deficit in Product and Process of Handwriting in Children with Attention Deficit Hyperactivity Disorder? A Systematic Review and Recommendations for Future Research.

Handwriting abnormalities in children with attention deficit hyperactivity disorder (ADHD) have sometimes been reported both (i) at the product level (i.e., quality/legibility of the written trace and speed of writing) and (ii) at the process level (i.e., dynamic and kinematic features, such as on-paper and in-air durations, pen pressure and velocity peaks, etc.). Conversely, other works have failed to reveal any differences between ADHD and typically developing children. The question of the presence and nature of handwriting deficits in ADHD remains open and merits an in-depth examination. The aim of this systematic review was, therefore, to identify studies that have investigated the product and/or process of handwriting in children with ADHD compared to typically developing individuals. This review was conducted and reported in accordance with the PRISMA statement. A literature search was carried out using three electronic databases. The methodological quality of the studies was systematically assessed using the Critical Appraisal Skills Program (CASP) criteria. Twenty-one articles were identified. Of these, 17 described handwriting quality/legibility, 12 focused on speed and 14 analyzed the handwriting process. All the studies (100%) with satisfactory methodology procedures reported an impaired product and process in children with ADHD, while 25% evidenced a difference in the speed of production. Most importantly, the studies differed widely in their methodological approaches. Substantial gaps remain, particularly with regard to ascertaining comorbidities, ADHD subtypes and the medical status of the included children. The lack of overall homogeneity in the samples calls for higher quality studies. We conclude with recommendations for further studies.

Reading Comprehension Impairment in Children With Neurofibromatosis Type 1 (NF1): The Need of Multimodal Assessment of Attention.

Attention span, which has been shown to have an impact on reading quality in many other conditions, is one of the main cognitive disorders of neurofibromatosis type 1 (NF1). The aim of this work is to observe the impact of attention on reading comprehension, in NF1 and non-NF1 children. A multicenter, cross-sectional study was conducted on 150 children (8-12 years old) with or without NF1 (75 NF1 vs 75 non-NF1; 72 female, 78 male), matched for age, sex, handedness, and reading level, thus forming a continuum from good to poor readers in both NF1 and non-NF1 groups. Children with intellectual deficiency or neurologic or psychiatric disorder were excluded. Attentional skills were assessed by combining a parent questionnaire (Child Behavior CheckList) and a performance-based assessment (Conner's Continuous Performance Test-Second Edition). Reading comprehension was assessed through a standardized reading comprehension test (ORLEC Lobrot). The performance-based attention scores were associated with text and sentence comprehension ability (P = .0235 and P = .0164, respectively), while indirect questionnaire attention scores were only associated with sentence comprehension (P = .0263). For both groups, the correlations between questionnaire and performance-based measures were low. We have shown that reading comprehension is greatly influenced by attention in NF1 and non-NF1, even if predictors of good reading comprehension also include IQ score and reading accuracy. Indirect observer-rated questionnaires and direct performance-based measures of attention do not assess the same variables, are linked to different components of reading skills, and are not interchangeable assessments of attention difficulties. Both assessments are complementary and must be used simultaneously, leading to recommendations that support multimodal assessment of attention.

Developmental coordination disorder.

Developmental coordination disorder (DCD) is a neurodevelopmental disorder that affects children's ability to execute coordinated motor actions, resulting in slow, clumsy, or inaccurate motor performances and learning difficulties (of new motor tasks or to adapt previously learned gestures to a modified or additional constraint). In the course of development, children with DCD exhibit a diversity of motor signs, including fine and gross motor problems with impaired postural control and balance, and sensorimotor coordination or motor learning difficulties. The prevalence ranges between 1.8% and 8%, depending on the diagnostic criteria used, based on the cutoff of motor scores from standardized scales. Four main hypotheses have been postulated to explain DCD in terms of deficits in visuospatial functions, procedural learning, internal modeling, or executive functions. Neuroimaging studies are scarce but have highlighted several brain regions, including the parietal, frontal, and cerebellar cortices. Meta-analyses have supported task-oriented approaches as effective therapies to improve motor performance in children with DCD.

Are morphological and structural MRI characteristics related to specific cognitive impairments in neurofibromatosis type 1 (NF1) children?

INTRODUCTION: NF1 children have cognitive disorders, especially in executive functions, visuospatial, and language domains, the pathophysiological mechanisms of which are still poorly understood. MATERIALS AND METHODS: A correlation study was performed from neuropsychological assessments and brain MRIs of 38 NF1 patients and 42 controls, all right-handed, aged 8-12 years and matched in age and gender. The most discriminating neuropsychological tests were selected to assess their visuospatial, metaphonological and visuospatial working memory abilities. The MRI analyses focused on the presence and location of Unidentified Bright Objects (UBOs) (1), volume analysis (2) and diffusion analysis (fractional anisotropy and mean diffusivity) (3) of the regions of interest including subcortical structures and posterior fossa, as well as shape analysis of subcortical structures (4). The level of attention, intelligence quotient, age and gender of the patients were taken into account in the statistical analysis. Then, we studied how diffusion and volumes parameters were associated with neuropsychological characteristics in NF1 children. RESULTS: NF1 children present different brain imaging characteristics compared to the control such as (1) UBOs in 68%, (2) enlarged total intracranial volume, involving all subcortical structures, especially thalamus, (3) increased MD and decreased FA in thalamus, corpus callosum and hippocampus. These alterations are diffuse, without shape involvement. In NF1 group, brain microstructure is all the more altered that volumes are enlarged. However, we fail to find a link between these brain characteristics and neurocognitive scores. CONCLUSION: While NF1 patients have obvious pathological brain characteristics, the neuronal substrates of their cognitive deficits are still not fully understood, perhaps due to complex and multiple pathophysiological mechanisms underlying this disorder, as suggested by the heterogeneity observed in our study. However, our results are compatible with an interpretation of NF1 as a diffuse white matter disease.

Sporadic and Familial Variants in NF1: An Explanation of the Wide Variability in Neurocognitive Phenotype?

Background: Cognitive impairment is the most common neurological manifestation in NF1 and occurs in 30-70% of NF1 cases. The onset and severity of each specific cognitive deficit varies greatly from child to child, with no apparent external causes. The wide variability of phenotype is the most complex aspect in terms of management and care. Despite multiple research, the mechanism underlying the high heterogeneity in NF1 has not yet been elucidated. While many studies have focused on the effects of specific and precise genetic mutations on the NF1 phenotype, little has been done on the impact of NF1 transmission (sporadic vs. familial cases). We used a complete neuropsychological evaluation designed to assess five large cognitive areas: general cognitive functions (WISC-IV and EVIP); reading skills ("L'Alouette," ODEDYS-2 and Lobrot French reading tests); phonological process (ODEDYS-2 test); visual perceptual skills (JLO, Thurstone and Corsi block tests) and attention (CPT-II), as well as psychosocial adjustments (CBCL) to explore the impact of NF1 transmission on cognitive disease manifestation in 96 children affected by NF1 [55 sporadic cases (29♀, 26♂); 41 familial cases (24♀, 17♂)]. Results: Familial and Sporadic form of NF1 only differ in IQ expression. The families' socioeconomic status (SES) impacts IQ performance but not differently between sporadic and familial variants. However, SES is lower in familial variants than in the sporadic variant of NF1. No other cognitive differences emerge between sporadic and familial NF1. Conclusions: Inheritance in NF1 failed to explain the phenotype variability in its entirety. IQ differences between groups seems in part linked to the environment where the child grows up. Children with NF1, and especially those that have early diagnoses (most often in inherited cases), must obtain careful monitoring from their early childhood, at home to strengthen investment in education and in school to early detect emerging academic problems and to quickly place them into care. Trial Registration: IDRCB, IDRCB2008-A01444-51. Registered 19 January 2009.

Can the Cognitive Phenotype in Neurofibromatosis Type 1 (NF1) Be Explained by Neuroimaging? A Review.

Neurofibromatosis type 1 (NF1) is one of the most frequent monogenetic disorders. It can be associated with cognitive dysfunctions in several domains such as executive functioning, language, visual perception, motor skills, social skills, memory and/or attention. Neuroimaging is becoming more and more important for a clearer understanding of the neural basis of these deficits. In recent years, several studies have used different imaging techniques to examine structural, morphological and functional alterations in NF1 disease. They have shown that NF1 patients have specific brain characteristics such as Unidentified Bright Objects (UBOs), macrocephaly, a higher volume of subcortical structures, microstructure integrity alterations, or connectivity alterations. In this review, which focuses on the studies published after the last 2 reviews of this topic (in 2010 and 2011), we report on recent structural, morphological and functional neuroimaging studies in NF1 subjects, with special focus on those that examine the neural basis of the NF1 cognitive phenotype. Although UBOs are one of the most obvious and visible elements in brain imaging, correlation studies have failed to establish a robust and reproducible link between major cognitive deficits in NF1 and their presence, number or localization. In the same vein, the results among structural studies are not consistent. Functional magnetic resonance imaging (fMRI) studies appear to be more sensitive, especially for understanding the executive function deficit that seems to be associated with a dysfunction in the right inferior frontal areas and the middle frontal areas. Similarly, fMRI studies have found that visuospatial deficits could be associated with a dysfunction in the visual cortex and especially in the magnocellular pathway involved in the processing of low spatial frequency and high temporal frequency. Connectivity studies have shown a reduction in anterior-posterior "long-range" connectivity and a deficit in deactivation in default mode network (DMN) during cognitive tasks. In conclusion, despite the contribution of new imaging techniques and despite relative advancement, the cognitive phenotype of NF1 patients is not totally understood.

Developmental coordination disorder and dysgraphia: signs and symptoms, diagnosis, and rehabilitation.

Developmental coordination disorder (DCD) is a common and well-recognized neurodevelopmental disorder affecting approximately 5 in every 100 individuals worldwide. It has long been included in standard national and international classifications of disorders (especially the Diagnostic and Statistical Manual of Mental Disorders). Children and adults with DCD may come to medical or paramedical attention because of poor motor skills, poor motor coordination, and/or impaired procedural learning affecting activities of daily living. Studies show DCD persistence of 30-70% in adulthood for individuals who were diagnosed with DCD as children, with direct consequences in the academic realm and even beyond. In particular, individuals with DCD are at increased risk of impaired handwriting skills. Medium-term and long-term prognosis depends on the timing of the diagnosis, (possible) comorbid disorders (and their diagnosis), the variability of signs and symptoms (number and intensity), and the nature and frequency of the interventions individuals receive. We therefore chose to investigate the signs and symptoms, diagnosis, and rehabilitation of both DCD and developmental dysgraphia, which continues to receive far too little attention in its own right from researchers and clinicians.

Neuropsychological status of French children with developmental dyslexia and/or developmental coordination disorder: Are both necessarily worse than one?

Developmental dyslexia (DD) and developmental coordination disorder (DCD) co-occur frequently, raising the underlying question of shared etiological bases. We investigated the cognitive profile of children with DD, children with DCD, and children with the dual association (DD + DCD) to determine the inherent characteristics of each disorder and explore the possible additional impact of co-morbidity on intellectual, attentional, and psychosocial functioning. The participants were 8- to 12-year-olds (20 DD, 22 DCD, and 23 DD + DCD). Cognitive abilities were assessed by the Wechsler Intelligence Scale for Children - Fourth Edition (WISC-IV) and the Continuous Performance Test - Second Edition (CPT-II) and behavioral impairments were evaluated by the Child Behavior Checklist (CBCL). No differences were found between the three groups on attention testing (CPT-II) or psychosocial characteristics (CBCL), but a higher percentage of DD + DCD children had pathological scores on psychosocial scales. Significant between-group differences were observed on Processing Speed Index scores and the block design and symbol search subtests, where DD children fared better than DCD children. No significant differences were evident between the co-morbid vs. the pure groups. Our results clearly show significant differences between children with DD only and children with DCD only. In particular, visuo-spatial disabilities and heterogeneity of intellectual profile seem to be good markers of DCD. However, it should be noted that despite these distinct and separate characteristics, a common cognitive profile (weaknesses and strengths) is likely shared by both neurodevelopmental disorders. Surprisingly, concerning co-morbidity, DD + DCD association is not associated with a decrease in intellectual or attentional capacities.

Lack of Motor Inhibition as a Marker of Learning Difficulties of Bimanual Coordination in Teenagers With Developmental Coordination Disorder.

This study tested the learning of a new bimanual coordination in teenagers with and without Developmental Coordination Disorder (DCD). Both groups improved accuracy of the new coordination. No difference was found on stability. But DCD teenagers exhibited an overall higher number of additional taps, suggesting a persistent lack of motor inhibition during learning. Moreover, teenagers with the lowest scores of motor abilities present the highest number of additional taps. All these results suggest that this number of additional taps (rather than traditional measures of accuracy and stability) could be a good marker of perceptual-motor learning deficit in DCD.

Neural changes associated to procedural learning and automatization process in Developmental Coordination Disorder and/or Developmental Dyslexia.

OBJECTIVE: Recent theories hypothesize that procedural learning may support the frequent overlap between neurodevelopmental disorders. The neural circuitry supporting procedural learning includes, among others, cortico-cerebellar and cortico-striatal loops. Alteration of these loops may account for the frequent comorbidity between Developmental Coordination Disorder (DCD) and Developmental Dyslexia (DD). The aim of our study was to investigate cerebral changes due to the learning and automatization of a sequence learning task in children with DD, or DCD, or both disorders. METHOD: fMRI on 48 children (aged 8-12) with DD, DCD or DD + DCD was used to explore their brain activity during procedural tasks, performed either after two weeks of training or in the early stage of learning. RESULTS: Firstly, our results indicate that all children were able to perform the task with the same level of automaticity, but recruit different brain processes to achieve the same performance. Secondly, our fMRI results do not appear to confirm Nicolson and Fawcett's model. The neural correlates recruited for procedural learning by the DD and the comorbid groups are very close, while the DCD group presents distinct characteristics. This provide a promising direction on the neural mechanisms associated with procedural learning in neurodevelopmental disorders and for understanding comorbidity.

Neural Signature of DCD: A Critical Review of MRI Neuroimaging Studies.

The most common neurodevelopmental disorders (e.g., developmental dyslexia (DD), autism, attention-deficit hyperactivity disorder (ADHD)) have been the subject of numerous neuroimaging studies, leading to certain brain regions being identified as neural correlates of these conditions, referring to a neural signature of disorders. Developmental coordination disorder (DCD), however, remains one of the least understood and studied neurodevelopmental disorders. Given the acknowledged link between motor difficulties and brain features, it is surprising that so few research studies have systematically explored the brains of children with DCD. The aim of the present review was to ascertain whether it is currently possible to identify a neural signature for DCD, based on the 14 magnetic resonance imaging neuroimaging studies that have been conducted in DCD to date. Our results indicate that several brain areas are unquestionably linked to DCD: cerebellum, basal ganglia, parietal lobe, and parts of the frontal lobe (medial orbitofrontal cortex and dorsolateral prefrontal cortex). However, research has been too sparse and studies have suffered from several limitations that constitute a serious obstacle to address the question of a well-established neural signature for DCD.

Procedural learning and automatization process in children with developmental coordination disorder and/or developmental dyslexia.

OBJECTIVE: There is increasing evidence to suggest that developmental dyslexia (DD) and developmental coordination disorder (DCD) actually form part of a broader disorder. Their frequent association could be justified by a deficit of the procedural memory system, that subtends many of the cognitive, motor and linguistic abilities that are impaired in both DD and DCD. However, studies of procedural learning in these two disorders have yielded divergent results, and in any case no studies have so far addressed the issue of automatization (dual-task paradigm). METHODS: We administered a finger tapping task to participants aged 8-12 years (19 DCD, 18 DD, and 22 with both DD and DCD) to explore procedural learning and automatic movements in these three groups of children, comparing motor performances at the prelearning stage, after 2 weeks of training, and in a post-training dual-task condition. RESULTS: First, results indicated that all the children were able to learn a sequence of movements and even automatize their movements. Second, they revealed between-groups differences in procedural/automatization learning abilities, setting the DCD group apart from the other two. Third, contrary to our expectations concerning comorbidity, they suggested that the DD+DCD association does not have an additional impact on behavioral performances.