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Objective: To assess the functionality of the affected upper limb in children diagnosed with hemiplegia aged between 4 and 8 years after applying low-intensity modified Constraint-Induced Movement Therapy (mCIMT). Methods: Prospective case series study. A mCIMT protocol was applied for five weeks, with two hours of containment per day. The study variables were quality of movement of the upper limb, spontaneous use, participation of the affected upper limb in activities of daily living, dynamic joint position, grasp&ndash, release action, grasp strength, supination and extension elbow movements. Four measurements were performed, using the quality of upper extremity test (QUEST) scale, the Shriners Hospital for Children Upper Extremity Evaluation (SHUEE) Evaluation, a hand dynamometer and a goniometer. Results: The sample was composed of eight children with moderate manual ability. Statistically significant differences were detected in all the studied variables (p &lt, 0.05) between the pre-treatment and post&ndash, treatment results (Week 0&ndash, Week 5), except for upper limb dressing, putting on splints and buttoning up. In the first week, the changes were statistically significant, except for protective extension, grasp strength, grasp&ndash, release and all functional variables (level of functionality and participation of the patient&rsquo, s upper limbs) in the SHUEE Evaluation (p &gt, 0.05). The greatest increase occurred in spontaneous use from Assessment 1 to Assessment 4 (p = 0.01), reaching 88.87% active participation in bimanual tasks. The quality of movement of the upper limb exhibited a significant value due to the increase in dissociated movements and grasp (p = 0.01). Conclusion: A low dose (50 h) of mCIMT increased the functionality of children diagnosed with congenital hemiplegia between 4 and 8 years of age with moderate manual ability.

New rehabilitation programs based on action observation therapy (AOT) are effective in improving motor function in children with congenital hemiplegia. In this pilot study we tested the potential effects of AOT on the reorganization of the motor system by functional magnetic resonance imaging (fMRI). As part of a randomized trial, eight subjects (age range: 6.2-14.5 years) with congenital hemiplegia were randomly assigned to an experimental (EG) or control (CG) group. All children underwent a clinical and neurophysiological assessment with Assisting Hand Assessment (AHA), MRI, and fMRI at baseline (T0), 1(T1), and 8(T2) weeks after the end of 3-week treatment. For the EG, AOT consisted in the observation of uni/bimanual goal-directed actions followed by their execution. CG watched same-duration computer games and then performed the same actions in the same order used in the EG. fMRI study was carried out using two different paradigms, for exploring sensory-motor network (SMN) localization and action observation network (AON). The pattern of brain activation was generally similar between T0 and T1 for both groups, while it was more widespread at T2, compared to T0 and T1, in the EG. This enlargement was coupled with functional improvement at AHA. Single-subject analysis shows a reduction of lateralization indexes both for the AON and the SMN. This pilot study, despite the small sample, showed the fMRI feasibility for providing relevant biomarkers of brain plasticity for monitoring the AOT response in children with congenital hemiplegia. The study was registered at http://www.clinicaltrials.gov (identifier NCT01016496).

Background Motor imagery (MI) training may benefit children with congenital hemiplegia, but reports on MI ability are mixed. This study considered individual patterns of performance to better understand MI ability in children with hemiplegia. Method Twenty children with hemiplegia (7–13 years; 10 with right hemiplegia), completed a MI task, IQ estimate and functional tests. Children with hemiplegia scoring above chance on the MI task were compared to a group of age-matched peers. The performance patterns of those scoring below chance were considered individually. Results Three children with right hemiplegia were excluded due to low IQ. Seven of 10 children with left hemiplegia and three of seven with right hemiplegia performed MI at an equivalent level to peers without hemiplegia. The seven children with hemiplegia who scored significantly below chance scored lower on functional tests, but differences here failed to reach an adjusted significance level. Four of the seven appeared engaged in MI, but performed very poorly. The remaining three had unique performance patterns explored in more detail. Conclusion Motor imagery deficits are not universally observed in children with congenital hemiplegia and individual performance should be examined before completing group analyses. Recommendations for exclusions and reporting in future studies are made.

Infants with West Syndrome and underlying structural pathology typically experience persistent symptomatic focal seizures and intellectual disability. We performed a retrospective case review of 84 patients with West Syndrome evaluated at one institution between 1990 and 2013. From this group we identified three patients with West syndrome and congenital hemiplegia who later developed genetic epilepsy features and had normal intellectual development. This outcome is highly unusual and raises important questions about the relationship and possible influence of genetic epilepsy in patients with pre-existent West Syndrome and brain injury.

Background Children with unilateral spastic cerebral palsy (USCP) exhibit impaired bimanual coordination, gait control, and whole body movement control. Intensive upper extremity training has been found to be effective for improving upper extremity function. However, the effectiveness of the intensive upper extremity training on whole body movement control is not known. Research question The present study aimed to evaluate the effects of Constraint Induced Movement Therapy (CIMT) and Hand Arm Bimanual Intensive Therapy (HABIT) on bimanual coordination and gait control during a complex whole body task. Methods Sixteen children with congenital hemiplegia (age 6–12 years; GMFCS: I-II, MACS: I-II) were randomly assigned to either CIMT or HABIT for 6 h per day training for 15 days. Children were asked to perform two whole body tasks (walking with and without a tray carrying) while 3-D kinematic analysis was performed before and after training. Results After training, the HABIT group increased the symmetry in height of their hands during tray carrying (more leveled tray). Both CIMT and HABIT groups decreased the lateral motion of the tray. The CIMT group increased speed and stride length after training in both the walking and tray carrying tasks. Both groups also increased their minimum toe clearance (all p Significance Two types of intensive upper extremity training have provided significant improvements to whole body movement control for children with USCP. Adhering to the specificity of practice concept, HABIT improved bimanual coordination after training during the whole body tray carrying tasks. Given extensive interactions between the upper and lower extremities in real-world activities, future studies should focus on the effects of such combined training.

Background Detecting differences in upper limb use in children with unilateral cerebral palsy (UCP) is challenging and highly dependent on examiner experience. The recent introduction of technologies in the clinical environment, and in particular the use of wearable sensors, can provide quantitative measurement to overcome this issue. This study aims to evaluate ActiGraph GT3X+ as a tool for measuring asymmetry in the use of the two upper limbs (ULs) during the assessment with a standardized clinical tool, the Assisting Hand Assessment (AHA) in UCP patients aged 3–25 years compared to age-matched typically developing (TD) subjects. Methods Fifty children with UCP and 50 TD subjects were assessed with AHA while wearing ActiGraphs GT3X+ on both wrists. The mean activity of each hand (dominant and non-dominant, MADH and MANDH, respectively) and the asymmetry index (AI) were calculated. Two linear mixed model analyses were carried out to evaluate how dependent actigraphic variables (i.e. MANDH and AI) varied by group (TD vs UCP) and among levels of manual ability based on Manual Ability Classification System (MACS). In both models age, sex, side of hemiplegia, presence/absence of mirror movements were specified as random effects. Results The MANDH was significantly lower in UCP compared to TD, while the AI was significantly higher in UCP compared to TD. Moreover, in UCP group there were significant differences related to MACS levels, both for MANDH and AI. None of the random variables (i.e. age, sex, side, presence/absence of mirror movements) showed significant interaction with MANDH and AI. Conclusions These results confirm that actigraphy could provide, in a standardized setting, a quantitative description of differences between upper limbs activity. Trial registration ClincalTrials.gov, NCT03054441. Registered 15 February 2017.

Congenital heart defects and skeletal malformation syndrome is very rare syndrome. Most of the patients had germline mutations in ABL1 gene. A 30-year-old gentleman presented with history of congenital heart disease (ventricular septal defect) and skeletal malformations which are typical of CHDSKM. Patient also had congenital hemiplegia which is rare in CHDSKMS. Patient also had lactose intolerance since childhood. Patients were evaluated thoroughly to rule out other causes. Current report is one of the rare case reports of CHDSKMS, only few case reports have been published till now.

Background. Crossed cerebellar diaschisis is the disruption of functional connectivity between cerebrum and cerebellum after hemispheric unilateral brain lesions. In adults and to a lesser extent in children, crossed cerebellar diaschisis has been largely investigated by functional connectivity and demonstrated to influence paretic hand function. Objective. We aim to demonstrate a disruption in structural corticopontocerebellar (CPC) connectivity in children with congenital brain lesions and examine its correlation with paretic hand motor function. Methods. Thirty-six children (Manual Ability Classification System: I, n = 21; II, n = 15) with unilateral brain lesions and 18 controls were analyzed in a case-control study, and diffusion magnetic resonance imaging data were acquired at 3T. High angular resolution diffusion imaging probabilistic tractography was employed for the region of interest–based reconstruction of CPC tracts. To identify statistical differences in structural cerebrocerebellar connectivity between case and control groups, an asymmetry index based on the number of streamlines of CPC tracts was used. In the case group, the correlation between asymmetry index and hand function measures was also determined. Results. Projections through the middle cerebellar peduncle to the contralateral cerebral cortex showed greater asymmetry in children with congenital unilateral brain lesion compared to controls ( P = .03), thus indicating a disruption of structural cerebrocerebellar connectivity. The degree of asymmetry index showed a correlation ( P < .03; r = −0.31) with impaired hand abilities in bimanual tasks. Conclusions. Disruption of structural cerebrocerebellar connectivity is present in patients with congenital unilateral brain injury and might be related to impaired hand function in bimanual skills, with potential implication in tailoring early intervention strategies.

ObjectivesCongenital hemiplegia is the most common form of cerebral palsy (CP). Children with unilateral CP show signs of upper limb asymmetry by 8 months corrected age (ca) but are frequently not referred to therapy until after 12 months ca. This study compares the efficacy of infant-friendly modified constraint-induced movement therapy (Baby mCIMT) to infant friendly bimanual therapy (Baby BIM) on upper limb, cognitive and neuroplasticity outcomes in a multisite randomised comparison trial.Methods and analysis150 infants (75 in each group), aged between 3 and 6 months ca, with asymmetric brain injury and clinical signs of upper extremity asymmetry will be recruited. Children will be randomised centrally to receive equal doses of either Baby mCIMT or Baby BIM. Baby mCIMT comprises restraint of the unimpaired hand using a simple restraint (eg, glove, sock), combined with intensive parent implemented practice focusing on active use of the impaired hand in a play-based context. In contrast, Baby BIM promotes active play requiring both hands in a play-based context. Both interventions will be delivered by parents at home with monthly home visits and interim telecommunication support by study therapists. Assessments will be conducted at study entry; at 6, 12 months ca immediately postintervention (primary outcome) and 24 months ca (retention). The primary outcome will be the Mini-Assisting Hand Assessment. Secondary outcomes include the Bayley Scale for Infant and Toddler Development (cognitive and motor domains) and the Hand Assessment of Infants. A subset of children will undertake MRI scans at 24 months ca to evaluate brain lesion severity and brain (re)organisation after intervention.Ethics and disseminationFull ethical approvals for this study have been obtained from the relevant sites. The findings will be disseminated in peer-reviewed publications.Trial registration numberAustralian and New Zealand Clinical Trials Registry: ACTRN12615000180516, Pre results.

Background Cerebral palsy (CP) is a term to describe the spectrum of disorders of impaired motor and sensory function caused by a brain lesion occurring early during development. Diffusion MRI and tractography have been shown to be useful in the study of white matter (WM) microstructure in tracts likely to be impacted by the static brain lesion. Aim The purpose of this study was to identify WM pathways with altered connectivity in children with unilateral CP caused by periventricular white matter lesions using a whole-brain connectivity approach. Methods Data of 50 children with unilateral CP caused by periventricular white matter lesions (5–17 years; manual ability classification system [MACS] I = 25/II = 25) and 17 children with typical development (CTD; 7–16 years) were analysed. Structural and High Angular Resolution Diffusion weighted Images (HARDI; 64 directions, b = 3000 s/mm2) were acquired at 3 T. Connectomes were calculated using whole-brain probabilistic tractography in combination with structural parcellation of the cortex and subcortical structures. Connections with altered fractional anisotropy (FA) in children with unilateral CP compared to CTD were identified using network-based statistics (NBS). The relationship between FA and performance of the impaired hand in bimanual tasks (Assisting Hand Assessment—AHA) was assessed in connections that showed significant differences in FA compared to CTD. Results FA was reduced in children with unilateral CP compared to CTD. Seven pathways, including the corticospinal, thalamocortical, and fronto-parietal association pathways were identified simultaneously in children with left and right unilateral CP. There was a positive relationship between performance of the impaired hand in bimanual tasks and FA within the cortico-spinal and thalamo-cortical pathways (r2 = 0.16–0.44; p, Highlights • Alterations in FA in children with CP were assessed using the connectome approach. • FA is reduced in corticospinal, thalamocortical, and association tracts in CP. • Higher FA is associated with better performance in bimanual tasks.

Background/Objectives: Children’s motivational predisposition may directly impact on engagement in therapy and adherence to intervention. To examine relationships between mastery motivation and other personal and environmental factors in school aged children with congenital hemiplegia.

Conclusions/Significance: The DMQ has evidence of good test retest reliability for individual subscales, instrumental aspect and total motivation scores in school aged children with congenital hemiplegia. Clinicians can confidently use this measure when investigating mastery motivation in children with congenital hemiplegia...

Visual perception difficulties are common in children with cerebral palsy - hemiplegia, however it is not known which assessment tool is the best for this population. This systematic review evaluates the clinimetric properties of visual perception assessments for children with hemiplegia.Databases were searched for assessments that: (i) measured visual perception; (ii) were reported in studies with children with hemiplegia and (iii) had clinimetric data available to assessors. Three assessments met criteria: the Test of Visual Perceptual Skills (TVPS), Motor-Free Visual Perceptual Test (MVPT) and Developmental Test of Visual Perception (DTVP). Factor analysis has been completed for the TVPS and DTVP, with both assessments and especially the TVPS, demonstrating some subtests that do not load significantly for the first factor of motor-free visual perception. All three assessments demonstrate variable construct and criterion validity with other clinical assessments. The DTVP, MVPT and TVPS demonstrate high test-retest reliability for total scores, but individual TVPS subtests are less reliable. The MVPT and DTVP show the best clinimetric data, however, less research has been completed on these tests than the TVPS. Further research is required to confirm the validity and reliability of the MVPT and DTVP for children with hemiplegia.

Background/Objectives: To understand the neural mechanisms underlying response to Constraint Induced Movement Therapy (CIMT) compared with Bimanual Training (BIM). Conclusions/Significance: Our results provide direct evidence of neural plasticity for the group who received constraint induced movement therapy (increased cortical excitability of the impaired motor cortex supported by neurovascular changes on fMRI). Both groups made significant improvements with movement efficiency (JTHFT) and bimanual coordination (AHA) however the CIMT group demonstrated a significant improvement in unimanual capacity (MUUL). Our data suggest that upper limb rehabilitation should commence with CIMT followed by bimanual training to maximize neural plasticity. © 2010 The Authors. Journal compilation © 2010 Mac Keith Press Developmental Medicine & Child Neurology

AIM This study explores the relationship between unimanual capacity and bimanual performance for children with congenital hemiplegia aged 5 to 16 years. It also examines the relationship between impairments and unimanual capacity and bimanual performance.

SUMMARY Following the introduction defining the term ‘speech disorder’ and describing the physiology of the speech mechanism, the various types of speech disorder occurring in children are described. In the first place it is necessary to exclude mental defect by suitable intelligence tests, and also various forms of deafness. The remaining disorders of speech, which include defects of comprehension as well as expression, are grouped as follows: (1) Organic disorders of the nervous system of congenital origin—a heterogeneous group of cases which include congenital cerebral diplegia, congenital suprabulbar paresis, double hemiplegia, congenital hemiplegia, bilateral athetosis, ataxias of articulation mainly due to cerebellar dysgenesis, and defects of the cranial nerve nuclei. (2) Various forms of aphasia—developmental executive or expressive aphasia and receptive aphasia, including four grades of congenital auditory imperception. (3) Acquired forms due to head injury, encephalitis, cerebral abscess and cerebral tumours. (4) Mechanical defects including congenital cleft palate, congenital short soft palate, ‘tongue-tie’, and ‘pharyngomegaly’, in which the entrance to the nasopharynx is too wide for even a normal palate to bridge. (5) Functional disturbances with no recognisable organic or structural defect either of the central nervous system or of peripheral organs of speech, including stammering (or stuttering) and various forms of ‘dyslalia’. RESUME A la suite d'une introduction definissant le terme ‘troubles de la parole’ et decrivant la physiologie du mecanisme de la parole, cette communication presente les divers types de troubles de la parole survenant chez les enfants. En premier lieu il faut exclure la deficience mentale par des tests d'intelligence appropries, et aussi les differentes formes de surdite. Il reste les troubles de la parole suivants, qui comprennent les deficiences de comprehension comme celles d'expression: (1) Troubles organiques du systeme nerveux d'origine congenitale—un groupe heterogene de cas qui comprennent la diplegie cerebrale congenitale, la paresie suprabulbaire congenitale, l'hemiplegie double, l'hemiplegie congenitale, l'athetose bilaterale, les ataxies des articulations dues principalement a la dysgenese cerebelleuse, et des deficiences des noyaux nerveux crâniens. (2) Des formes variees d'aphasie—aphasie de developpement, d'execution ou d'expression et aphasie receptive, comprenant les quatre degres d'imperception auditoire congenitale. (3) Les formes acquises dues a des blessures de la tete, encephalite, abces et tumeurs cerebrales. (4) Les defauts mecaniques comprenant scissure congenitale du voile du palais court et mou congenital, ankyloglosse, et pharynomegalie dans laquelle l'entree du nasopharynx est trop large pour qu'un palais meme normal Penjambe. (5) Troubles fonctionnels sans defaut organique ou structural reconnaissable du systeme nerveux central ou des organes peripheriques de la parole y compris begaiement (ou bredouillement) et diverses formes de ‘dyslalie’. ZUSAMMENFASSUNG Nach der Einleitung in welcher die Bezeichnung ‘Sprachstorung’ definiert und die Physiologic des Sprachmechanismus beschrieben wird, werden dann die verschiedenen Typen der in Kindern auftretenden Sprachstorungen beschrieben. Zuallererst mussen Mentaldefekte mithilfe von sachgemassen Intelligenz-Tests ausgeschlossen werden, wie auch die verschiedenen Formen von Taubheit. Die ubrigbleibenden Sprachstorungen, darunter Defekte sowohl des Verstehens wie auch des Ausdrucksvermogens, werden folgendermassen eingeteilt: (1) Organische Storungen des Nervensystems, kongenitalen Ursprungs—eine heterogene Gruppe von Fallen wie kongenitale zerebrale Diplegie, kongenitale suprabulbare Parese, Doppel-Hemiplegie, kongenitale Hemiplegie, bilaterale Athetosis, Ataxien der Artikulation welche hauptsachlich von zerebellarer Dysgenesis herruhren, und Defekte der kranialen Nerven-Nuclei. (2) Verschiedene Formen der Aphasie—fortschreitende exekutive oder expressive Aphasie und rezeptive Aphasie, darunter auch vier Grade der kongenitalen auditorischen Impel zeption. (3) Erworbene Formen die von Kopfverletzungen, Enzephalitis, zerebralen Abszessen und Gehirntumoren verursacht sind. (4) Mechanische Defekte wie kongenital gespaltener Gaumen, kongenitaler kurzer weicher Gaumen, ‘gebundene Zunge’ und Pharyngomelagie bei welcher die Offnung zum Naso-Pharynx selbst fur einen normalen Gaumen zu weit ware. (5) Funktionsstorungen mit keinem erkennbaren organischen oder strukturmassigen Defekt im zentralen Nerven-system oder in den periferischen Sprechorganen, darunter das Stottern und verschiedene Formen von ‘Dyslalie’. RESUMEN Despues de una introduccion que define el termino ‘desorden de lenguaje’ y describe la fisiologia del mecanismo de lenguaje, se describen los varios tipos de desorden de lenguaje. En primer lugar hay que excluir un defecto menial por medio de tests apropiados de inteligencia; hay tambien que excluir ciertos tipos de sordera. Los defectos de lenguaje que quedan, y que comprenden defectos de comprension ademas de defectos de expresion, caen dentro de los grupos siguientes: (1) Desordenes organicos del sistema nervioso de origen congenito—un grupo heterogeneo de casos que incluyen diplegjia cerebral congenita, paresia suprabulbar congenita, hemiplejia doble, hemiplejia congenita, atetosis bilateral, ataxias de articulacion debidas principalmente a disgenia cerebelar, y defectos de los nucleos de los nervios craneales. (2) Varios tipos de afasia-afasia ejecutiva o expresiva de desarrollo y afasia sensorial, inclusocuatro grados de impercepcion acustica congenita. (3) Formas adquiridas debidas a lesiones de la cabeza, encefalitis, absceso cerebral y tumores cerebrates, (4) Defectos mecanicos, incluso fisura congenita del paladar, paladar blando congenitamente corto, anquiloglosia, y faringomegalia, donde la entrada a la nasofaringe es demasiado ancha para que un paladar aun normal se extienda sobre ella. (5) Disturbios functionales no acompanados de ningun defecto organico o estructural que puede reconocerse, del sistema nervioso central o de los organos perifericos de lenguaje, los cuales incluyen el balbuceo (o el tartamudeo) y varias formas de ‘dislalia’.

SUMMARY Twins were more than three times more common in a large sample of London children with congenital hemiplegia than in the general population. This over-representation of twins could largely be explained by their higher rate of preterm birth, though twin-specific risk factors, including the consequences of a co-twin's death in utero, may also have played a part. None of the 34 co-twins who survived infancy had hemiplegia or any other form of cerebral palsy. Among 155 siblings of singletons with congenital hemiplegia, no child had hemiplegia and only one had cerebral palsy. Perhaps it is chance rather then genetic liability or an adverse environment that primarily governs who does and does not become congenitally hemiplegic. RESUME L'hemiplegie congenitale chez les jumeaux Les jumeaux sont apparus trois fois plus nombreux dans un large echantillon d'hemiplegie congenitale chez l'enfant que dans la population generale. Cette sur representation des jumeaux pourrait s'expliquer largement par un taux plus eleve de naissance prematuree, bien que les facteurs de risques propres au jumeaux, incluant les consequences de la mort in utero du co-jumeau, aient pu jouer egalement un role. Aucun des 34 co-jumeaux survivants n'avait d'hemiplegie ou d'autres formes d'IMC. Chez 155 enfants de-la fratrie d'hemiplegiques enfant unique, aucun n'avait d'hemiplegie et un seul avait une IMC. C'est peut-etre le hasard plus que le poids genetique ou un environnement pathogene qui fait fondamentalement qu'un enfant naisse hemiplegique ou non. ZUSAMMENFASSUNG Eine Zwillingsstudie der kongenitalen Hemiplegie In einer grosen Untersuchungsgruppe Londoner Kinder mit kongenitaler Hemiplegic waren Zwillinge mehr als dreimal haufiger als in der Allgemeinbevolkerung. Diese Uberreprasentation von Zwillingen konnte weitgehend durch ihre hohere Rate an Fruhgeburten erklart werden, obwohl fur Zwillinge spezifischc Risikofaktoren, einschlieslich der Folgen durch das Absterben eines Zwillings in utero, ebenfalls eine Rolle gespielt haben konnen. Keines der 34 Zwillingskinder, die das Sauglingsalter uberlebten, hatte eine Hemiplegie oder irgendeine andere Form der Cerebralparese. Von 155 Geschwistern von Einzelkindern mit kongenitaler Hemiplegie hatte kein Kind eine Hemiplegie und nur eines hatte Cerebralparese. Vielleicht ist es eher Zufall als genetische Folge oder ein ungunstiges Umfeld wodurch bestimmt wird, wer eine kongenitale Hemiplegie bekommt und wer nicht. RESUMEN Estudio en gemelos de la hemiplegia congenita La incidencia de gemelos era tres veces superior a la de la poblacion general en una gran muestra de ninos londinenses con hemiplegia congenita. Esta hipcrrepresentacion de gemelos podria explicarse largamente por un mayor porcentaje de nacimientos prematures, aunque tambien pudieron jugar un papel los factores especificos de riesgo de los gemelos, incluyendo las consecuencias de la muerte in utero de uno de los hermanos. Ninguno de los 34 co-gemelos que sobrevivieron a la epoca de la lactancia tenia hemiplegia ni ninguna otra forma de paralisis cerebral. Entre los 155 hermanos de gemelos sobrevivientes con hemiplegia congenita, ninguno tenia hemiplegia, y solo uno tenia una paralisis cerebral. Tal vez esto sea mas suerte que fiabilidad genetica o una peristasi adversa, que primariamente gobieman a los que llegan a tener una hemiplegia congenita y a los que no.

OBJECTIVES. Precision grasping critically relies on the integrity of the corticospinal tract as evidenced in congenital hemiplegia by the correlation found between corticospinal dysgenesis and hand-movement deficits. Therefore, corticospinal dysgenesis could be used to anticipate upper-limb deficits in young infants with congenital hemiplegia. However, most studies have quantified corticospinal dysgenesis by measuring the cross-sectional area of cerebral peduncles on T1 MRI, a measure biased by other structures present in the peduncles. The purpose of this study was to evaluate the extent to which this may have hampered the conclusions of previous studies. We also aimed to investigate the relationship between upper-limb deficits and a more accurate measure of corticospinal dysgenesis to provide a tool for anticipating upper-limb deficits in infants with congenital hemiplegia. METHODS. To address this issue, we measured corticospinal tract areas in 12 patients with congenital hemiplegia and 12 matched control subjects by using the diffusion tensor imaging technique. Corticospinal dysgenesis was quantified by computing a symmetry index between the area of the contralateral and ipsilateral corticospinal tracts. This value was then compared with that resulting from the conventional MRI method. RESULTS. The symmetry indexes gathered with these 2 methods were highly correlated, although the diffusion tensor imaging symmetry indexes were significantly smaller. This indicates that, in patients with congenital hemiplegia, the conventional MRI measurement has led to a systematic underestimate of corticospinal dysgenesis. These 2 estimates of corticospinal dysgenesis were also correlated with upper-limb impairments and disabilities. Although the symmetry index computed from peduncle measurements was correlated solely with deficits in stereognosis, the diffusion tensor imaging index correlated with stereognosis, digital and manual dexterities, and ABILHAND-Kids, a measure of manual ability in daily life activities. CONCLUSIONS. The diffusion tensor imaging symmetry index provides a useful prognostic tool for anticipating upper-limb deficits and their consequences in daily life activities.

Using functional magnetic resonance imaging, the brain activation related to unilateral sequential finger-to-thumb opposition was studied in six children with a right congenital hemiplegia of cortical origin. They were compared to six age-matched controls. In the control group, movements with either hand asymmetrically activated the sensorimotor cortex and premotor areas in both cerebral hemispheres with a typical contralateral predominance. By contrast, paretic finger movements activated both hemispheres in the hemiplegic patients, with a strong ipsilateral predominance favoring the undamaged hemisphere. The activation induced by nonparetic finger movements was restricted to the contralateral undamaged hemisphere. Furthermore, the level of activation in the undamaged cortex was partly related to residual finger dexterity, according to covariance analysis. These activation patterns indicate an adaptive reorganization of the cortical motor networks in this group of patients, with a prominent involvement of the undamaged hemisphere in the control of finger movements with either hand.

Background: Patients with congenital and acquired hemiparesis incur long-term functional deficits, among which the loss of prehension that may impact their functional independence. Identifying, understanding, and comparing the underlying mechanisms of prehension impairments represent an opportunity to better adapt neurorehabilitation. Objective: The present review aims to provide a better understanding of precision grip deficits in congenital and acquired hemiparesis and to determine whether the severity and type of fine motor control impairments depend on whether or not the lesions are congenital or acquired in adulthood. Methods: Using combinations of the following key words: fingertip force, grip force, precision grip, cerebral palsy, stroke, PubMed, and Scopus databases were used to search studies from 1984 to 2013. Results: Individuals with both congenital and acquired hemiparesis were able to some extent to use anticipatory motor control in precision grip tasks, even if this control was impaired in the paretic hand. In both congenital and acquired hemiparesis, the ability to plan efficient anticipatory motor control when the less-affected hand is used provides a possibility to remediate impairments in anticipatory motor control of the paretic hand. Conclusion: Surprisingly, we observed very few differences between the results of studies in children with congenital hemiplegia and stroke patients. We suggest that the underlying specific strategies of neurorehabilitation developed for each one could benefit the other.

To examine internal consistency, test-retest reproducibility, and parent-child concordance of the Dimensions of Mastery Questionnaire 17.0 (DMQ) in school-aged children with congenital hemiplegia.Forty-two children (8.24 ± 2.38 years, Manual Ability Classification System (MACS) I = 23, MACS II = 19) and their parents completed the DMQ, and a subset on two occasions 2-30 days apart (n = 27). Cronbach's alpha (α), intraclass correlation coefficients (ICCs), standard error of measurement (SEM), and 95% limits of agreement were calculated.Internal consistency for child self-report was variable (α = 0.57-0.90). Cronbach's alphas for parent proxy report were good (α = 0.69-0.86). Test-retest reproducibility for instrumental aspect (ICC = 0.86) and total motivation (ICC = 0.84) were excellent with subscales ranging from 0.70 to 0.91. The SEM for total motivation was 0.23 points. Parent-child concordance was poor across all scores (ICC = -0.04 to 0.42) with a large SEM (0.50-0.91).The DMQ parent report has good test-retest reproducibility for subscales, instrumental, and total motivation scores in school-aged children with congenital hemiplegia. Parent-child concordance was low highlighting differences in individual and contextual perspectives.

In this study, we assessed basic and more complex non-symbolic and symbolic numerical task abilities in children with hemiplegia and obtained a detailed picture of their strengths and weaknesses in the numerical domain. Those children, who experience difficulties in finger gnosia and spontaneous use of fingers in counting, exhibit difficulties in finger pattern recognition and symbolic numerical tasks. However, their non-symbolic numerical abilities and arithmetic skills are preserved. These original results are discussed in light of the "manumerical cognition" hypothesis, which postulates that the use of fingers in numerical activities during childhood shapes our comprehension of numbers.

Aim To determine the extent to which children's mastery motivation predicts occupational performance outcomes following upper limb intervention (ULI). Method In this cohort study, participants received 45 hours of ULI, either in an intensive group-based or distributed individualized model. The Dimensions of Mastery Questionnaire (DMQ) measured mastery motivation at baseline. Occupational performance outcomes were assessed at baseline and 13 weeks’ post-intervention using the Canadian Occupational Performance Measure (COPM). Multivariable models determined the contribution of mastery motivation to COPM outcome irrespective of group membership. Results Forty-two children with congenital hemiplegia (29 males, 13 females; mean age 7y 8mo [SD 2y 2mo]; range 5y 1mo–12y 8mo; Manual Ability Classification System [MACS] I=20 and II=22; predominant motor type unilateral spastic n=41) participated in the study. Significant gains were seen in COPM performance and satisfaction scores (p

Background: Cerebral lesions causing hemiplegia can affect the retrochiasmatical visual pathways. Objective: The aim of this study was to investigate visual function in children suffering from congenital hemiplegia. Methods: Twenty children (mean age: 5 yrs, 5 mo; SD: 2 yrs, 11 mo) underwent orthoptic and ophthalmological examinations. Visual acuity was assessed using the Acuity Card Procedure and standard optotypes. In addition, visual fields were assessed using conventional perimetry (Goldmann’s perimeter or Peritest) or behavioral test (BEFIE test). Results: A remarkable finding was that 75% of the subjects showed a visual field defect, mainly homolateral to the hemiplegic side and contralateral to the (most extensive) cerebral lesions. Conclusion: Visual functions of children suffering from congenital hemiplegia should be routinely assessed, with particular attention to the visual field.

This study describes the results of the assessment of 51 children with congenital hemiplegia, 28 right and 23 left hemiplegics, on the Wechsler Intelligence Scales (WISC-R). These two groups were compared to a Control group of thirty non-hemiplegic children who were chosen to match the subjects on age at testing, full scale IQ, socioeconomic status and sex distribution. Hemiplegic subjects showed significantly greater differences between Verbal and Performance IQ scores compared to Controls, while the scores on the Object Assembly subscale and the Performance IQ compared to Controls. The left-right maturation gradient of the two hemispheres as a possible explanation for the unexpected results in the right hemiplegics is discussed, although lack of differences between the hemiplegic groups may be the more striking feature. Children with congenital hemiplegia may have fewer cognitive deficits due to early reorganization of lateralized cerebral functions, or the WISC-R may not be sensitive enough to...

"Mirror movements" are an axonal guidance disorder that consists of involuntary contralateral movements that mimic unilateral intentional ones, typically involving the fingers of the hand. They can be isolated or associated with conditions such as Klippel-Feil syndrome, Kallmann syndrome, or congenital hemiplegia. Isolated congenital mirror movements are sometimes caused by autosomal dominant mutation in the genes DCC or RAD51 . At least 4 previously reported cases had strabismus, 3 with Moebius syndrome and 1 with Duane retraction syndrome. We report the case of a boy with an unusual incomitant strabismus consistent with orbital dysinnervation and suggest that for some patients with congenital mirror movements the neurological miswiring extends to the orbit, causing congenital cranial dysinnervation disorder.

We report three children with pure congenital hemiplegia found to have congenital bilateral perisylvian polymicrogyria (CBPP). None of our patients had the seizures, oromotor dysfunction, or cognitive impairment usually associated with CBPP. CBPP may be more common and heterogeneous than previously thought, is easily recognized by MRI, and should be included in the differential diagnosis of the young child presenting with congenital hemiplegia.

Fifty-one children with congenital hemiplegia (6–16 years) were included in this study, 28 with right hemiplegia (14 male and 14 female) and 23 with left hemiplegia (15 male and 8 female) as well as a control group (15 male and 15 female) chosen to match the subjects on age at testing, full scale IQ and socioeconomic status. A neurological examination, parent questionnaires and an interview were used to assess neurological and socioeconomic status, development and current educational placement, behavioural profile and handedness of family members. Birth records and brain scans (CT) were examined. Strict inclusion criteria ensured the unilaterality of the lesion. There were more right than left hemiplegic children identified and relatively more males than females in the left hemiplegic group. The two hemiplegic groups were similar on all descriptive and developmental data. They differed significantly from the controls only in that the left hemiplegic group was more premature and smaller at birth; ...

Fourteen children with congenital hemiplegia were studied with a detailed assessment of various aspects of vision (linear acuity, stereopsis, visual fields) and MRI. The aim of this study was to evaluate the effect of a congenital lesion on visual function. The results showed a very high incidence (78 %) of children who had abnormal results on at least one of the visual tests. Visual abnormalities were not correlated with the clinical severity of hemiplegia or with a specific pattern of lesion on MRI. Similarly no constant association could be found between visual structures (optic radiations and primary visual cortex) and visual function. Finally, our results would suggest that all the children with congenital hemiplegia need to be investigated irrespective of the clinical severity or of the type or the extent of the lesion. This would help to identify children with minor visual abnormalities which can affect everyday life performance.

Dichotic listening and WISC IQ data are reported from a monozygotic male twin pair, with left- and right-sided congenital hemiplegia, respectively. The twin brothers were 13 years old at the time of testing. They were tested with dichotic listening to consonant-vowel syllables under three different attentional instructions. Thus, both laterality and cognitive functions were investigated. The neurological examination revealed normal sensory functions in the hand contralateral to the lesion, but impaired motor function. The WISC results showed lowered verbal IQ compared with performance IQ in the left-hemisphere impaired twin, and vice-versa in the right-hemisphere impaired twin. The dichotic listening results followed the WISC data with a left-ear advantage in the left-hemisphere impaired twin and a corresponding right-ear advantage in the right-hemisphere impaired twin. Neither twin could modify their ear advantage scores by instructions to attend specifically to and report only from the left or ...