Connectome-based fingerprint of motor impairment is stable along the course of Parkinson's disease.

Functional alterations in brain connectivity have previously been described in Parkinson's disease, but it is not clear whether individual differences in connectivity profiles might be also linked to severity of motor-symptom manifestation. Here we investigated the relevance of individual functional connectivity patterns measured with resting-state fMRI with respect to motor-symptom severity in Parkinson's disease, through a whole-brain, data-driven approach (connectome-based predictive modeling). Neuroimaging and clinical data of Parkinson's disease patients from the Parkinson's Progression Markers Initiative were derived at baseline (session 1, n = 81) and at follow-up (session 2, n = 53). Connectome-based predictive modeling protocol was implemented to predict levels of motor impairment from individual connectivity profiles. The resulting predictive model comprised a network mainly involving functional connections between regions located in the cerebellum, and in the motor and frontoparietal networks. The predictive power of the model was stable along disease progression, as the connectivity within the same network could predict levels of motor impairment, even at a later stage of the disease. Finally, connectivity profiles within this network could be identified at the individual level, suggesting the presence of individual fingerprints within resting-state fMRI connectivity associated with motor manifestations in Parkinson's disease.

Comparison of the prevalence of malocclusion and oral habits between children with cerebral palsy and healthy children.

BACKGROUND: Cerebral palsy (CP) represents for children an important problem of health and affects roughly 2 per 1000 live births and is the most common pediatric developmental motor disability. Therefore, the purpose of this study was to determine the prevalence, type and severity of malocclusion and oral habits in children with Cerebral Palsy (CP) and to compare them with a control group of healthy children in Sana'a city. MATERIALS AND METHODS: A prospective, case-control study was made of two groups, a cerebral palsy and a control group. The study population consisted of 60 children who had CP, and a control group of 60 matched children with no physical or mental disabilities. Data were collected using a questionnaire and assessment for malocclusion was done clinically. The patients were compared with equal number of age-matched controls. The inclusion criteria were individuals aged over 6 years; absence of previous orthodontic treatment; no missing permanent first molars. RESULTS: Results showed an increased prevalence of malocclusion in children with cerebral palsy. Molar class II relationship was statistically higher in cerebral palsy children than healthy control (P = 0.001). Cerebral palsied children are likely to have a significantly increased protrusion of the anterior teeth (P < 0.001) when compared with normal children. Mouth breathing and Tongue thrust. Habits were significantly higher in the CP group (p = 0.0001) when compared with normal children. CONCLUSION: The prevalence of malocclusion was higher in children with Cerebral palsy than in normal children, and the present study concludes that in children with Cerebral Palsy, more oral Habits problems due to oral motor dysfunctions are common and problems of mouth breathing and Tongue thrust produce different malocclusion and poor oral hygiene complications in these children.

Pentraxin 3, a serum biomarker in human T-cell lymphotropic virus type-1-associated myelopathy patients and asymptomatic carriers.

Human T-cell lymphotropic virus type 1 (HTLV-1) can induce a neuroinflammatory condition that leads to myelopathy. Pentraxin 3 (PTX3) is an acute-phase protein that its plasma concentration increases during inflammation. We aimed to determine whether PTX3 serum level is elevated in HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients and HTLV-1 asymptomatic carriers (ACs) and evaluate its association with proviral load and clinical features. The serum level of PTX3 was measured using an enzyme-linked immunosorbent assay in 30 HAM patients, 30 HTLV-1 ACs, and 30 healthy controls. Also, the HTLV-1 proviral load was determined via real-time PCR technique. The findings showed that PTX3 serum level was significantly higher in HAM patients than in both asymptomatic carriers and healthy controls (p values < 0.0001). No correlation between PTX3 and the proviral load was observed in HAM patients and asymptomatic carriers (r = - 0.238, p = 0.205 and r = - 0.078, p = 0.681, respectively). The findings showed that there was no significant correlation between PTX3 and motor disability grading (MDG) (r = - 0.155, p = 0.41) nor urinary disturbance score (UDS) (r = - 0.238, p = 0.20). Higher levels of PTX3 are associated with HTLV-1-associated myelopathy compared to asymptomatic carriers. This finding may support the idea that PTX3 has the potential as a diagnostic biomarker.

Clinical correlates of R1 relaxometry and magnetic susceptibility changes in multiple sclerosis: a multi-parameter quantitative MRI study of brain iron and myelin.

OBJECTIVES: The clinical impact of brain microstructural abnormalities in multiple sclerosis (MS) remains elusive. We aimed to characterize the topography of longitudinal relaxation rate (R1) and quantitative susceptibility (χ) changes, as indices of iron and myelin, together with brain atrophy, and to clarify their contribution to cognitive and motor disability in MS. METHODS: In this cross-sectional study, voxel-based morphometry, and voxel-based quantification analyses of R1 and χ maps were conducted in gray matter (GM) and white matter (WM) of 117 MS patients and 53 healthy controls. Voxel-wise between-group differences were assessed with nonparametric permutation tests, while correlations between MRI metrics and clinical variables (global disability, cognitive and motor performance) were assessed both globally and voxel-wise within clusters emerging from the between-group comparisons. RESULTS: MS patients showed widespread R1 decrease associated with more limited modifications of χ, with atrophy mainly involving deep GM, posterior and infratentorial regions (p < 0.02). While R1 and χ showed a parallel reduction in several WM tracts (p < 0.001), reduced GM R1 values (p < 0.001) were associated with decreased thalamic χ (p < 0.001) and small clusters of increased χ in the caudate nucleus and prefrontal cortex (p < 0.02). In addition to the atrophy, χ values in the cingulum and corona radiata correlated with global disability and motor performance, while focal demyelination correlated with cognitive performance (p < 0.04). CONCLUSIONS: We confirmed the presence of widespread R1 changes, involving both GM and WM, and atrophy in MS, with less extensive modifications of tissue χ. While atrophy and χ changes are related to global and motor disability, R1 changes are meaningful correlates of cognition. KEY POINTS: • Compared to healthy controls, multiple sclerosis patients showed R1 and χ changes suggestive of iron increase within the basal ganglia and reduced iron and myelin content within (subnuclei of) the thalamus. • Thalamic volume and χ changes significantly predicted clinical disability, as well as pulvinar R1 and χ changes, independently from atrophy. • Atrophy-independent R1 and χ changes, suggestive of thalamic iron and myelin depletion, may represent a sensitive marker of subclinical inflammation.

Relations between nonmotor manifestations and motor disorders in patients with benign essential blepharospasm.

PURPOSE: To evaluate the relations between nonmotor manifestations (dry eye, mood disorders, and sleep disturbance) and motor disorders in patients with benign essential blepharospasm (BEB), and to determine whether relieving motor disorders by botulinum neurotoxin can improve the nonmotor manifestations. METHODS: In this prospective case series study, 123 BEB patients were enrolled for evaluations. Among them, 28 patients underwent botulinum neurotoxin therapy and attended another two postoperative visits at 1 month and 3 months. Motor severity was measured with Jankovic Rating Scale (JRS) and Blepharospasm Disability Index (BSDI). We assessed dry eye using OSDI questionnaire, Schirmer test, tear break-up time (TBUT), tear meniscus height, lipid layer thickness (LLT) and corneal fluorescence staining. Zung's Self-rating Anxiety and Depression Scale (SAS, SDS) and Pittsburgh Sleep Quality Index (PSQI) were for mood status and sleep quality evaluations. RESULTS: Patients with dry eye or mood disorders had higher JRS scores (5.78 ± 1.13, 5.97 ± 1.30) than those without (5.12 ± 1.40, 5.50 ± 1.16; P = 0.039, 0.019, respectively). BSDI values of patients with sleep disturbance (14.61 ± 4.71) was higher than those without (11.89 ± 5.44, P = 0.006). Correlations were found between JRS, BSDI and SAS, SDS, PSQI, OSDI, TBUT. Botulinum neurotoxin effectively relieved JRS, BSDI and improved PSQI, OSDI, TBUT, LLT (8.11 ± 5.81, 21.77 ± 15.76, 5.04 ± 2.15 s, 79.61 ± 24.11 nm) at the 1-month visit compared to baseline (9.75 ± 5.60, 33.58 ± 13.27, 4.14 ± 2.21 s, 62.33 ± 22.01 nm; P = 0.006, < 0.001, = 0.027, < 0.001, respectively). CONCLUSIONS: The BEB patients with dry eye, mood disorders, or sleep disturbance had more severe motor disorders. Motor severity was associated with the severity of the nonmotor manifestations. Relieving motor disorders by botulinum neurotoxin was effective in improving dry eye and sleep disturbance.

Interaction between cognitive and motor disorders for risk screening of incident falls: results of an elderly population-based observational cohort study.

BACKGROUND: This study aims to examine (1) the association of "Emergency Room Evaluation and Recommendations" (ER2) cognitive and motor items with incident falls (i.e., ≥ 1), their recurrence (i.e., ≥ 2) and post-fall fractures and (2) the performance criteria (i.e., sensitivity, specificity) of the greater identified association for each incident fall outcome in older community dwellers. METHODS: 7147 participants (80.5 ± 3.8; 100% female) of the EPIDémiologie de l'OStéoporose (EPIDOS) observational population-based cohort study were recruited in France. Inability to name the day's date and the use of a walking aid and/or an history of falls were recorded at baseline. Incident outcomes, which were ≥ 1 fall, ≥ 2 falls and post-fall fractures, were collected every 4 months over a period of 4 years. RESULTS: The overall incidence of ≥ 1 fall was 26.4%, 6.4% for ≥ 2 falls, and 19.1% for post-fall fractures. Cox regressions revealed that the use of a walking aid and/or an history of falls [Hazard ratio (HR) ≥ 1.03 with P ≤ 0.011], inability to name the day's date (HR ≥ 1.05 with P ≤ 0.003), and their combination (HR ≥ 1.37 with P ≤ 0.002) were significantly associated with both incident falls, regardless of their recurrence, and post-fall fractures. INTERPRETATION: A significant positive association between ER2 cognitive and motor items, both, respectively, and in combination, with an overall incidence of falls, regardless of their recurrence, as well as with post-fall fractures was demonstrated. However, the low sensitivity and high specificity of the combination of ER2 items suggest that these items cannot be used for risk screening of fall outcomes in the older population.

Arundic Acid (ONO-2506) Attenuates Neuroinflammation and Prevents Motor Impairment in Rats with Intracerebral Hemorrhage.

Intracerebral hemorrhage (ICH) is a severe stroke subtype caused by the rupture of blood vessels within the brain. Increased levels of S100B protein may contribute to neuroinflammation after ICH through activation of astrocytes and resident microglia, with the consequent production of proinflammatory cytokines and reactive oxygen species (ROS). Inhibition of astrocytic synthesis of S100B by arundic acid (AA) has shown beneficial effects in experimental central nervous system disorders. In present study, we administered AA in a collagenase-induced ICH rodent model in order to evaluate its effects on neurological deficits, S100B levels, astrocytic activation, inflammatory, and oxidative parameters. Rats underwent stereotactic surgery for injection of collagenase in the left striatum and AA (2 µg/µl; weight × 0.005) or vehicle in the left lateral ventricle. Neurological deficits were evaluated by the Ladder rung walking and Grip strength tests. Striatal S100B, astrogliosis, and microglial activation were assessed by immunofluorescence analysis. Striatal levels of interleukin 1ß (IL-1ß) and tumor necrosis factor α (TNF-α) were measured by ELISA, and the ROS production was analyzed by dichlorofluorescein (DCF) oxidation. AA treatment prevented motor dysfunction, reduced S100B levels, astrogliosis, and microglial activation in the damaged striatum, thus decreasing the release of proinflammatory cytokines IL-1ß and TNF-α, as well as ROS production. Taken together, present results suggest that AA could be a pharmacological tool to prevent the harmful effects of increased S100B, attenuating neuroinflammation and secondary brain damage after ICH.

Cerebellar Contributions to Motor Impairments in People with Multiple Sclerosis.

Although Charcot characterized classic cerebellar symptoms in people with multiple sclerosis (PwMS) in 1877, the impact of cerebellar dysfunction on MS symptoms has predominately been evaluated in the last two decades. Recent studies have clearly demonstrated the association between cerebellar pathology, including atrophy and reduced fractional anisotropy in the peduncles, and motor impairments, such as reduced gait velocity and time to complete walking tasks. However, future studies using novel imaging techniques are needed to elucidate all potential pathophysiology that is associated with disability in PwMS. Additionally, future studies are required to determine the most effective treatments for motor impairments in PwMS, including the specific type and duration of exercise interventions, and potential means to amplify their effects, such as transcranial direct current stimulation (tDCS). This mini-review critically discusses the distinct role of cerebellar dysfunction in motor impairments in PwMS, potential treatments, and directions for future studies.

Synergistic associations of cognitive and motor impairments with functional outcome in covert cerebral small vessel disease.

BACKGROUND: Cognitive and motor impairments are the key clinical manifestations of cerebral small vessel disease (SVD), but their combined effects on functional outcome have not been elucidated. This study investigated the interactions and mediating effects of cognitive and motor functions on instrumental activities of daily living (IADL) and quality of life in older individuals with various degrees of white matter hyperintensities (WMH). METHODS: Participants of the Helsinki Small Vessel Disease Study (n = 152) were assessed according to an extensive clinical, physical, neuropsychological and MRI protocol. Volumes of WMH and gray matter (GM) were obtained with automated segmentation. RESULTS: Cognitive (global cognition, executive functions, processing speed, memory) and motor functions (gait speed, single-leg stance, timed up-and-go) had strong interrelations with each other, and they were significantly associated with IADL, quality of life as well as WMH and GM volumes. A consistent pattern on significant interactions between cognitive and motor functions was found on informant-evaluated IADL, but not on self-evaluated quality of life. The association of WMH volume with IADL was mediated by global cognition, whereas the association of GM volume with IADL was mediated by global cognition and timed up-and-go performance. CONCLUSION: The results highlight the complex interplay and synergism between motor and cognitive abilities on functional outcome in SVD. The combined effect of motor and cognitive disturbances on IADL is likely to be greater than their individual effects. Patients with both impairments are at disproportionate risk for poor outcome. WMH and brain atrophy contribute to disability through cognitive and motor impairment.

Serial Long-Term Growth and Neurodevelopment of Very-Low-Birth-Weight Infants: 2022 Update on the Korean Neonatal Network.

BACKGROUND: We aimed to evaluate the long-term growth and neurodevelopmental outcomes of very-low-birth-weight infants (VLBWIs, birth weight < 1,500 g) born between 2013, the establishment of the Korean Neonatal Network (KNN), and 2018, both at 18-24 months of corrected age and three years of age, using a nationwide large cohort, and to evaluate whether these outcomes have improved over time since 2013. METHODS: This study used data from the annual reports of the KNN for 18-24 months of corrected age (follow-up 1) and three years of age (follow-up 2). Follow-up 1 data were collected from 10,065 eligible VLBWIs born between January 1, 2013, and December 31, 2018. Follow-up 2 data were collected from 8,156 eligible VLBWIs born between January 1, 2013, and December 31, 2017. RESULTS: The overall follow-up rates of VLBWIs at follow-ups 1 and 2 were 74.6% (7,512/10,065) and 57.7% (4,702/8,156), respectively. The overall mortality rate between discharge from the neonatal intensive care unit and follow-up 1 was 1% (104/10,065). The overall mortality rate between follow-ups 1 and 2 was 0.049% (4/8,156). Growth restrictions decreased over time, especially weight growth restrictions, which significantly decreased according to era (17% in infants born in 2013-2014 and 13% in infants born in 2017-2018). Fewer infants were re-hospitalized and required rehabilitative support according to era at follow-up 1. More infants had language developmental delays and required language support according to era, both at follow-ups 1 and 2. The incidence of cerebral palsy has significantly decreased over time, from 6% in infants born in 2013-2014 to 4% in infants born in 2017-2018 at follow-up 1, and from 8% in infants born in 2013-2014 to 5% in infants born in 2017 at follow-up 2. CONCLUSION: Long-term outcomes of VLBWIs regarding weight growth and cerebral palsy, the most common motor disability in childhood, have improved serially according to era since 2013. However, the rate of infants with language delays requiring language support has increased according to era. Further studies are required on the increased trends of language delay and language support while improving motor outcomes.

Comprehensive genetic analysis confers high diagnostic yield in 16 Japanese patients with corpus callosum anomalies.

Corpus callosum anomalies (CCA) is a common congenital brain anomaly with various etiologies. Although one of the most important etiologies is genetic factors, the genetic background of CCA is heterogenous and diverse types of variants are likely to be causative. In this study, we analyzed 16 Japanese patients with corpus callosum anomalies to delineate clinical features and the genetic background of CCAs. We observed the common phenotypes accompanied by CCAs: intellectual disability (100%), motor developmental delay (93.8%), seizures (60%), and facial dysmorphisms (50%). Brain magnetic resonance imaging showed colpocephaly (enlarged posterior horn of the lateral ventricles, 84.6%) and enlarged supracerebellar cistern (41.7%). Whole exome sequencing revealed genetic alterations in 9 of the 16 patients (56.3%), including 8 de novo alterations (2 copy number variants and variants in ARID1B, CDK8, HIVEP2, and TCF4) and a recessive variant of TBCK. De novo ARID1B variants were identified in three unrelated individuals, suggesting that ARID1B variants are major genetic causes of CCAs. A de novo TCF4 variant and somatic mosaic deletion at 18q21.31-qter encompassing TCF4 suggest an association of TCF4 abnormalities with CCAs. This study, which analyzes CCA patients usung whole exome sequencing, demonstrates that comprehensive genetic analysis would be useful for investigating various causal variants of CCAs.

Acidosis, cognitive dysfunction and motor impairments in patients with kidney disease.

Metabolic acidosis, defined as a plasma or serum bicarbonate concentration <22 mmol/L, is a frequent consequence of chronic kidney disease (CKD) and occurs in ~10-30% of patients with advanced stages of CKD. Likewise, in patients with a kidney transplant, prevalence rates of metabolic acidosis range from 20% to 50%. CKD has recently been associated with cognitive dysfunction, including mild cognitive impairment with memory and attention deficits, reduced executive functions and morphological damage detectable with imaging. Also, impaired motor functions and loss of muscle strength are often found in patients with advanced CKD, which in part may be attributed to altered central nervous system (CNS) functions. While the exact mechanisms of how CKD may cause cognitive dysfunction and reduced motor functions are still debated, recent data point towards the possibility that acidosis is one modifiable contributor to cognitive dysfunction. This review summarizes recent evidence for an association between acidosis and cognitive dysfunction in patients with CKD and discusses potential mechanisms by which acidosis may impact CNS functions. The review also identifies important open questions to be answered to improve prevention and therapy of cognitive dysfunction in the setting of metabolic acidosis in patients with CKD.

Epilepsy and lockdown: A survey of patients normally attending a Spanish centre.

BACKGROUND: Lockdown due to the SARS-CoV-2 pandemic became a challenge to maintain care for patients with epilepsy; we aimed to find out how the pandemic affected them. METHODS: We sent an online 22-item questionnaire to patients from our outpatient clinic, a reference centre in Spain for drug-resistant epilepsy, inquiring about the effects of lockdown, from March to May 2020. RESULTS: We sent the survey to 627 patients; 312 (58% women) sent a complete response and were included. Of all respondents, 57% took >2 antiseizure medications. One-third of respondents (29%) declared an associated cognitive or motor disability. A minority had confirmed infection with SARS-CoV-2 (1.92%). Seizure frequency remained like usual in 56% of patients, while 31.2% reported an increase. Less than 10% needed emergent assistance. Almost half reported anxiety or depression, and 25% increased behavioural disorders. Mood (F: 5.40; p: 0.002) and sleep disorders (F = 2.67; p: 0.05) were associated with increase in seizure frequency. Patients were able to contact their physicians when needed and were open to a future telematic approach to follow-up visits. CONCLUSIONS: Seizure frequency and severity remained unchanged in most patients during the lockdown. Mood and sleep disorders were common and associated with seizure worsening. Patients were open to telematic care in the future.

Preclinical Pharmacology of [2-(3-Fluoro-5-Methanesulfonyl-phenoxy)Ethyl](Propyl)amine (IRL790), a Novel Dopamine Transmission Modulator for the Treatment of Motor and Psychiatric Complications in Parkinson Disease.

IRL790 ([2-(3-fluoro-5-methanesulfonylphenoxy)ethyl](propyl)amine, mesdopetam) is a novel compound in development for the clinical management of motor and psychiatric disabilities in Parkinson disease. The discovery of IRL790 was made applying a systems pharmacology approach based on in vivo response profiling. The chemical design idea was to develop a new type of DA D3/D2 receptor type antagonist built on agonist rather than antagonist structural motifs. We hypothesized that such a dopamine antagonist with physicochemical properties similar to agonists would exert antidyskinetic and antipsychotic effects in states of dysregulated dopaminergic signaling while having little negative impact on physiologic dopamine transmission and, hence, minimal liability for side effects related to dopamine-dependent functions. At the level of in vivo pharmacology, IRL790 displays balancing effects on aberrant motor phenotypes, reducing l-DOPA-induced dyskinesias in the rodent 6-hydroxydopamine lesion model and reducing psychostimulant-induced locomotor hyperactivity elicited by pretreatment with either d-amphetamine or dizocilpine, without negatively impacting normal motor performance. Thus, IRL790 has the ability to normalize the behavioral phenotype in hyperdopaminergic as well as hypoglutamatergic states. Neurochemical and immediate early gene (IEG) response profiles suggest modulation of DA neurotransmission, with some features, such as increased DA metabolites and extracellular DA, shared by atypical antipsychotics and others, such as increased frontal cortex IEGs, unique to IRL790. IRL790 also increases extracellular levels of acetylcholine in the prefrontal cortex and ventral hippocampus. At the receptor level, IRL790 appears to act as a preferential DA D3 receptor antagonist. Computational docking studies support preferential affinity at D3 receptors with an agonist-like binding mode. SIGNIFICANCE STATEMENT: This paper reports preclinical pharmacology along with molecular modeling results on IRL790, a novel compound in clinical development for the treatment of motor and psychiatric complications in advanced Parkinson disease. IRL790 is active in models of perturbed dopaminergic and glutamatergic signaling, including rodent 6-hydroxydopamine l-DOPA-induced dyskinesias and psychostimulant-induced hyperactivity, in a dose range that does not impair normal behavior. This effect profile is attributed to interactions at dopamine D2/D3 receptors, with a 6- to 8-fold preference for the D3 subtype.

Microbial invasion of the amniotic cavity is associated with impaired cognitive and motor function at school age in preterm children.

BACKGROUND: Chorioamnionitis is an important cause of preterm delivery. Data on neurodevelopmental outcome in exposed infants are inconsistent due to difficulties in diagnosing intrauterine infection/inflammation and lack of detailed long-term follow-up. We investigate cognitive and motor function in preterm infants at early school age and relate the findings to bacteria in amniotic fluid obtained by amniocentesis (microbial invasion of the amniotic cavity (MIAC)) or placenta findings of histological chorioamnionitis (HCA) or fetal inflammatory response syndrome (FIRS). METHOD: Sixty-six infants with gestational age <34 weeks at birth and without major disabilities were assessed using WISC-III and the Bruininks-Oseretsky Test of Motor Proficiency. Results were corrected for gestational age and sex. RESULTS: Children exposed to MIAC had significantly lower scores for full-scale IQ and verbal IQ compared to the non-MIAC group and the difference in full-scale IQ remained after correction for confounding factors. The MIAC group had also significantly lower motor scores after correction. In contrast, motor function was not affected in infants exposed to HCA or FIRS and differences between groups for cognitive scores were lost after corrections. CONCLUSION: Exposure to bacteria in amniotic fluid is associated with lower motor and cognitive scores in school age preterm infants without major disabilities.

Impaired Coordination and Recruitment of Muscle Agonists, But Not Abnormal Synergies or Co-contraction, Have a Significant Effect on Motor Impairments After Stroke.

Movement synergies, muscle co-contraction, and decreased motor drive to muscle agonists were suggested to be major factors in motor impairments after stroke. The purpose of this study was to investigate the major muscle mechanisms contributing to motor impairment after stroke. Twelve healthy and 13 post-stroke patients participated in this observational study. Both groups participated in a single experimental session, performing hand pointing movements in multiple directions, during which EMG was assessed. Additionally, the patients underwent the Fugl-Meyer assessment. A set of features from the electromyography (EMG) signal and co-contraction ratios were used to compare the capacity to modulate the muscle activity between the two groups of participants. A correlation analysis was applied between the Euclidian distances of each target and the Fugl-Meyer scoring assessment in the post-stroke patients. We found that impaired modulation of muscle activity in post-stroke patients was characterized by significantly increased Euclidian distances between the EMG features of different target directions and by a higher variability between muscle activation compared to healthy subjects. Impaired capacity to modulate muscle activity significantly correlated with the impairment status. In conclusion, impaired motor performance post-stroke systematic disturbance in the control signal to limb muscles, which manifests as decreased capacity to modulate muscle activity, rather than co-contraction of muscle antagonists or stereotyped movement patterns.

Health characteristics and health care trajectory of polyhandicaped person before and after 1990.

INTRODUCTION: Polyhandicap is defined as the combination of severe mental impairment and severe motor deficit resulting in reduced mobility and an extreme reduction in autonomy. Over the last 20years, care management for these patients has become more structured, however, their care pathway is not always optimal. OBJECTIVE: To describe/compare the health characteristics, treatment and history of the care pathways of subjects who received care before and after 1990. METHOD: Multicentre cross-sectional study, population studied: patients with polyhandicap: (i) causal brain damage<3years, (ii) severe mental impairment, (iii) motor disability, (iv) reduced mobility, (v) extreme restriction of autonomy. DATA COLLECTED: clinical and medical, care procedures, treatments, history of care pathways. RESULTS: Patients are divided into 2 groups: 545 patients who received care after 1990 and 330 before 1990. Older patients present more recurrent urinary infections, slow transit, behavioural disorders and pain, and are prescribed a greater number of drugs. For those who received care before 1990, the age of admission to an establishment is lower, with one-third receiving a consultation dedicated to the transition from paediatric to adult teams. DISCUSSION/CONCLUSION: The care sector for patients with polyhandicap makes it possible to meet their needs throughout their lives, however, there is still progress to be made in terms of formalisation and of coordinating the care pathway in order to facilitate the transition from paediatric to adult services/establishments.

Communicative abilities in young children with a significant cognitive and motor developmental delay.

BACKGROUND: Children with a significant cognitive and motor developmental delay are pre-symbolic communicators. The primary aim of this study was to reveal the variability within the communicative functioning of this group of children in terms of communication level, the reasons to communicate and behavioural expressions. METHODS: Twenty-six children between 14 and 58 months with a significant cognitive and motor developmental delay were recruited. The Communication Matrix of Rowland (2011, Communication Disorders Quarterly, 32, 190) was used to integrate different sources of information on the children's communicative functioning. RESULTS: These children primarily communicated at the level of pre-intentional and intentional behaviour, aimed at refusing, obtaining and, to a lesser extent, social purposes. CONCLUSIONS: To develop or adapt early intervention strategies, and to monitor progress in communicative development, an even more nuanced view on these children's communicative utterances in terms of frequency, duration, idiosyncrasy and context relatedness is needed.

Psychometric Properties of Assessments of Cognition in Infants With Cerebral Palsy or Motor Impairment: A Systematic Review.

Objective: Approximately 50% of people with cerebral palsy have a cognitive impairment. However, many tools used to assess cognition in infants require almost normal fine motor ability, and thus may not accurately reflect cognitive abilities of infants with cerebral palsy or other motor impairments. This systematic review aimed to evaluate the psychometric properties of cognitive assessment tools for infants aged 0-24 months with motor impairments and to make recommendations about the most appropriate cognitive assessment tools for the purpose of discrimination, prediction, and evaluation. Method: A systematic review was conducted. CINAHL, Embase, ERIC, Medline, PsycINFO, and SCOPUS databases were searched to identify studies reporting on 1 or more psychometric properties of a standardized cognitive assessment tool or questionnaire in a sample/subsample of infants with motor impairment. Of the 4,480 articles reviewed, 9 assessment tools were identified in 20 publications, which met our inclusion criteria. Articles were appraised using the COnsensus-based Standards for the selection of health Measurement INstruments to assess study rigor. The GRADE framework was applied to develop recommendations for clinical practice. Results: The Mayes Motor-Free Compilation, Fagan Test of Infant Intelligence, and Bayley-III Low Motor/Vision have predictive and/or discriminative utility in this population. The Mullen Scales of Early Learning was the only tool with psychometric research available examining responsivity to change. Conclusions: Assessment tools with low-motor/motor-free accommodations have greater accuracy in estimating cognitive abilities of infants with motor impairment than conventional norm-referenced tests. There, however, remains a significant paucity of research in this area.

New and emerging access technologies for adults with complex communication needs and severe motor impairments: State of the science.

Individuals with complex communication needs often use alternative access technologies to control their augmentative and alternative communication (AAC) devices, their computers, and mobile technologies. While a range of access devices is available, many challenges continue to exist, particularly for those with severe motor-control limitations. For some, access options may not be readily available or access itself may be inaccurate and frustrating. For others, access may be available but only under optimal conditions and support. There is an urgent need to develop new options for individuals with severe motor impairments and to leverage existing technology to improve efficiency, increase accuracy, and decrease fatigue of access. This paper describes person-centred research and development activities related to new and emerging access technologies, with a particular focus on adults with acquired neurological conditions.