Congenital Hemiplegia Research Articles

Randomized Comparison Trial of Rehabilitation Very Early for Infants with Congenital Hemiplegia

To compare efficacy of constraint-induced movement therapy (Baby-CIMT) with bimanual therapy (Baby-BIM) in infants at high risk of unilateral cerebral palsy (UCP). Single-blind, randomized-comparison-trial that had the following inclusion criteria: (i) asymmetric brain lesion (ii) absent fidgety General Movements, (iii) Hammersmith Infant Neurological Examination below cerebral palsy cut-points, (iv) entry at 3 to 9 months corrected age (CA), and (v) >3 point difference between hands on Hand Assessment Infants (HAI). Infants were randomized to Baby-CIMT or Baby-BIM, which comprised 6 to 9 months of home-based intervention. Daily dose varied from 20 to 40-minutes according to age (total 70-89.2 hours). Primary outcome measure was the HAI post intervention, with secondary outcomes Mini-Assisting Hand Assessment and Bayley III cognition at 24-months CA. 96 infants (51 male, 52 right hemiplegia) born median at 37-weeks' gestation were randomized to Baby-CIMT (n=46) or Baby-BIM (n=50) and commenced intervention at a mean 6.5 (SD 1.6) months CA. There were no between group differences immediately post-intervention on HAI (mean difference [MD] 0.98 HAI units, 95% confidence interval [CI] 0.94-2.91; p=0.31). Both groups demonstrated significant clinically important improvements from baseline to post-intervention (Baby-BIM MD 3.48, 95%CI 2.09-4.87; Baby-CIMT MD 4.42, 95% CI 3.07-5.77). At 24 months, 64 infants were diagnosed with UCP (35 Baby-CIMT, 29 Baby-BIM). Infants who entered the study between 3 and 6-months CA had greater change in HAI Both Hands Sum Score compared with those who entered at ≥6-months CA (MD 7.17, 95% CI 2.93, 11.41, p=0.001). Baby-CIMT was not superior to Baby-BIM, and both interventions improved hand development. Infants commencing intervention at <6 months CA had greater improvements in hand function.

Motor imagery in congenital hemiplegia: Impairments are not universal

BackgroundMotor 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. MethodTwenty 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. ResultsThree 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. ConclusionMotor 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.

Congenital heart defects and skeletal malformation syndrome with congenital hemiplegia

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.

Efficacy of the use of unaffected hand containment in unimanual intensive therapy to increase visuomotor coordination in children with hemiplegia: a randomized controlled pilot study.

Background:The capacity of children with hemiplegia to be engaged in anticipatory action planning is affected. There is no balance among spatial, proprioceptive and visual information, thus altering the affected upper limb visuomotor coordination. The objective of the present study was to assess the improvement in visuomotor coordination after the application of a unimanual intensive therapy program, with the use of unaffected hand containment compared with not using unaffected hand containment.Methods:A simple blind randomized clinical trial was realized. A total of 16 subjects with congenital infantile hemiplegia participated in the study with an age mean of 5.54 years old (SD:1.55). Two intensive protocols for 5 weeks of modified constraint-induced movement therapy (mCIMT) or unimanual therapy without containment (UTWC) were executed 5 days per week (2 h/day). Affected upper limb visuomotor coordination (reaction time, task total time, active range, dynamic grasp) was measured before–after intensive therapy using a specific circuit with different slopes (10°/15°).Results:Statistically significant inter-group differences were found after the intervention, with clinically relevant results for the mCIMT group not seen in UTWC, in the following variables: reaction time 10°slope (p = 0.003, d = 2.44), reaction time 15°slope (p = 0.002, d = 2.15) as well as for the task total time 10°slope (p = 0.002, d = 2.25), active reach 10°slope (p = 0.002, d = 2.7), active reach 15°slope (p = 0.003, d = 2.29) and dynamic grasp 10°/15°slopes (p = <0.001, d = 2.69). There were not statistically significant inter-group differences in the total task time with 15°slope (p = 0.074, d = 1.27).Conclusions:The use of unaffected hand containment in mCIMT would allow improvements in the affected upper limb’s visuomotor coordination. Thus, it would favor clinical practice to make decisions on therapeutic approaches to increase the affected upper limb functionality and action planning in children diagnosed with infantile hemiplegia (4–8 years old).

Unimanual Intensive Therapy with or without Unaffected Hand Containment in Children with Hemiplegia. A Randomized Controlled Pilot Study.

Children with hemiplegia have lower spontaneous use and quality of movement in the affected upper limb. The modified constraint-induced movement therapy (mCIMT) is applied to improve the affected upper limb function. The objective of this study was to study the efficacy of unaffected hand containment to obtain changes in the function of the affected upper limb after applying two unimanual therapies. A randomized controlled pilot study was performed with 16 children diagnosed with congenital infantile hemiplegia, with eight children randomized in each group (average age: 5.54 years; SD: 1.55). mCIMT and unimanual therapy without containment (UTWC) were applied, with a total of 50 h distributed in five weeks (two h/per day). Two assessments were performed (pre- and post-treatment) to evaluate the affected upper limb spontaneous use, measured with the Shiners Hospital Upper Extremity Evaluation (SHUEE), and the quality of movement, measured with the Quality of Upper Extremity Skills Test (QUEST scale). The progression of the variables was different in both groups. The results are expressed in the median of the improvement percent and interquartile range (IQR). The spontaneous use analysis showed an improvement percent of 31.65 (IQR: 2.33, 110.42) in the mCIMT group with respect to 0.00 (IQR: 0.00, 0.00) in the UTWC group. The quality of movement increased in the mCIMT and UTWC groups, 24.21 (IQR: 13.44, 50.39), 1.34 (IQR: 0.00, 4.75), respectively and the greatest increase was obtained in the grasp variable for both groups. The use of unaffected hand containment in mCIMT would produce improvements in the affected upper limb functionality in children with hemiplegia (4–8 years old) compared to the same protocol without containment (UTWC).

Application of Low-Intensity Modified Constraint-Induced Movement Therapy to Improve the Affected Upper Limb Functionality in Infantile Hemiplegia with Moderate Manual Ability: Case Series.

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–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 < 0.05) between the pre-treatment and post–treatment results (Week 0–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–release and all functional variables (level of functionality and participation of the patient’s upper limbs) in the SHUEE Evaluation (p > 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.

Reorganization of action observation and sensory-motor networks after action observation therapy in children with congenital hemiplegia: A pilot study.

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: 6.2-14.5years) 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).

Intensive upper extremity training improved whole body movement control for children with unilateral spastic cerebral palsy

BackgroundChildren 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 questionThe 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. MethodsSixteen 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. ResultsAfter 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 < 0.05). SignificanceTwo 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.

Potentials of Ultrahigh-Field MRI for the Study of Somatosensory Reorganization in Congenital Hemiplegia.

Reorganization of somatosensory function influences the clinical recovery of subjects with congenital unilateral brain lesions. Ultrahigh-field (UHF) functional MRI (fMRI) with the use of a 7 T magnet has the potential to contribute fundamentally to the current knowledge of such plasticity mechanisms. The purpose of this study was to obtain preliminary information on the possible advantages of the study of somatosensory reorganization at UHF fMRI. We enrolled 6 young adults (mean age 25 ± 6 years) with congenital unilateral brain lesions (4 in the left hemisphere and 2 in the right hemisphere; 4 with perilesional motor reorganization and 2 with contralesional motor reorganization) and 7 healthy age-matched controls. Nondominant hand sensory assessment included stereognosis and 2-point discrimination. Task-dependent fMRI was performed to elicit a somatosensory activation by using a safe and quantitative device developed ad hoc to deliver a reproducible gentle tactile stimulus to the distal phalanx of thumb and index fingers. Group analysis was performed in the control group. Individual analyses in the native space were performed with data of hemiplegic subjects. The gentle tactile stimulus showed great accuracy in determining somatosensory cortex activation. Single-subject gentle tactile stimulus showed an S1 activation in the postcentral gyrus and an S2 activation in the inferior parietal insular cortex. A correlation emerged between an index of S1 reorganization (distance between expected and reorganized S1) and sensory deficit (p < 0.05) in subjects with hemiplegia, with higher distance related to a more severe sensory deficit. Increase in spatial resolution at 7 T allows a better localization of reorganized tactile function validated by its correlation with clinical measures. Our results support the S1 early-determination hypothesis and support the central role of topography of reorganized S1 compared to a less relevant S1-M1 integration.

Maturation of sensory and motor tracts in children with congenital hemiplegia using diffusion tensor imaging

Maturation of sensory and motor tracts in children with congenital hemiplegia using diffusion tensor imaging

Stepping Stones to Switch Access

This article will focus on teaching switch access for individuals who have significant communication and physical and sensory disabilities and are unable to use their hands for direct access to a s...

Familiar patients with congenital hemiplegia due to a COL4A1 mutation

Familiar patients with congenital hemiplegia due to a <i>COL4A1</i> mutation

REACH: study protocol of a randomised trial of rehabilitation very early in congenital hemiplegia

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...

Libertango par Frédérique Deghelt

Jesus conveys a loveliness and innocence and Mary Cassatt’s baby boys often reflect an incipient ambition, Mordersohn-Becker’s little girls seem weighed down in a contemplation , whose exact nature, due again to the eyes, is never quite clear. It could be the grim future, which most likely awaits these children from poor backgrounds, or perhaps their somber faces reflect a much too young awareness of the normal difficulties and disillusionments that await them in life. Modersohn-Becker’s women and children often appear to be less individuals than types as she provides a variety of images conveying her personal sense of what it meant to be a woman at the dawn of the twentieth century. Florida State University, emeritus William Cloonan Deghelt, Frédérique. Libertango. Arles: Actes Sud, 2016. ISBN 978-2-330-06329-0. Pp. 304. 22,50 a. Deghelt infuses her novel about a great conductor with a realism evoked in part by reference to figures of renown on the international music scene and to familiar social and political events spanning the life of its fictional protagonist. This richly developed detail pulls the story away from becoming a fable of untrained“genius”that inexplicably achieves artistic productivity and fame. The story: Luis Nilta-Bergo, born in 1935, grows up in Paris, his Spanish parents having fled Franco’s repressive regime. An only son, Luis finds no favor with his family for reasons of a congenital hemiplegia that hampers his mobility and his speech. His parents respond to his disability with undisguised repulsion and shame; his elder sister treats him with cruelty; only a younger sister discreetly expresses affection for him. Years of schooling confirm his status as resident pariah and scapegoat. Luis compensates for his isolation by retreating into the music he can capture on an old radio. These ill-starred beginnings and their profound effect on Luis’s psyche never quite fade from his life, but his focused attention to music enhances an exceptional ability to hear the distinctiveness of sound and silence in music and to feel in a non-physical realm what music can create. The narrative then follows Luis’s journey from uncanny hearer of music to gifted conductor, without traditional training as instrumentalist or even as vocalist. At his lowest point in youth, Luis serendipitously meets a down-and-out Astor Piazzolla who encourages his musicality (the novel’s title thus references an exquisite tango of Piazzolla’s). Later, Luis learns to listen to and coach pianists under the tutelage of Nadia Boulanger. He plucks what education he can from the rigidly codified training of the Paris Conservatoire, training he challenges by also indulging in jazz performances. He is mentored by no less than Sergiu Celibidache, the great Rumanian conductor, and eventually comes up with the idea for an Orchestre du monde that will bring great music to the suffering masses around the world, express solidarity, and alleviate however briefly their pain. This project is, of course, rooted directly in his own experience: 268 FRENCH REVIEW 90.4 Reviews 269 “[La musique] était ce qui échappait à nos âmes chevillées à notre corps, notre liberté d’être nu, d’être immatériel, enfin libre”(205). This fictionalized“biography”requires vigilance on the reader’s part. One must follow a chronology scrambled by association and memory and further broken up by a variety of forms (letters, journal entries, film interviews) and different narrators (Luis, a documentary filmmaker, an omniscient narrator).Additionally, there is that familiar difficulty of translating one medium into another (music into text). As the descriptors of music Deghelt uses are non-technical and intentionally allude to the ineffable, she sometimes risks losing us in recondite descriptions of Luis’s music. On balance, she demonstrates that the ineffable can be far from ineffective on human well-being. Lawrence University (WI) Eilene Hoft-March Ernaux, Annie. Mémoire de fille. Paris: Gallimard, 2016. ISBN 978-2-07-014597-3. Pp. 160. 15 a. Durant l’été 58, la jeune Annie Duchêne, âgée de 19 ans et monitrice de colonie de vacances, vit un éveil à la sexualité désastreux. Elle s’abandonne au désir d’un Autre sans comprendre...

Grasp control in children with congenital hemiplegia in a discrete motor task of intersegmental coordination

Grasp control in children with congenital hemiplegia in a discrete motor task of intersegmental coordination

Rapid On-Line Control to Reaching Is Preserved in Children With Congenital Spastic Hemiplegia: Evidence From Double-Step Reaching Performance.

This study aimed to investigate the integrity of on-line control of reaching in congenital spastic hemiplegia in light of disparate evidence. Twelve children with and without spastic hemiplegia (11-17 years old) completed a double-step reaching task requiring them to reach and touch a target that remained stationary for most trials (viz nonjump trial) but unexpectedly displaced laterally at movement onset for a minority of trials (20%: known as jump trials). Although children with spastic hemiplegia were generally slower than age-matched controls, they could account for target perturbation at age-appropriate levels shown by a lack of interaction effect on movement time and nonsignificant group difference for time to reach trajectory correction on jump trials. Our data suggest that at a group level, on-line control of reaching may be age-appropriate in spastic hemiplegia. However, our data also highlight the need to experimentally acknowledge the considerable heterogeneity of the spastic hemiplegia population when investigating motor cognition.

Precision grip in congenital and acquired hemiparesis: similarities in impairments and implications for neurorehabilitation.

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.

Mastery motivation as a predictor of occupational performance following upper limb intervention for school-aged children with congenital hemiplegia.

To determine the extent to which children's mastery motivation predicts occupational performance outcomes following upper limb intervention (ULI). 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. 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<0.001) post-intervention with no between group differences. Children who had greater persistence with object-oriented tasks (p=0.02) and better manual ability (p=0.03) achieved higher COPM performance scores at 13 weeks. Children's persistence on object-oriented tasks was the strongest predictor of COPM satisfaction (p=0.01). Children's persistence with object-oriented tasks as well as manual abilities needs to be considered when undertaking ULI. Predetermining children's motivational predispositions can assist clinicians to tailor therapy sessions individually based on children's strengths, contributing to effective engagement in ULI.

Numerical Abilities in Children With Congenital Hemiplegia: An Investigation of the Role of Finger Use in Number Processing

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.

Mastery motivation in children with congenital hemiplegia: individual and environmental associations

The aim of this study was to examine the relationship between mastery motivation and individual and environmental characteristics in school-aged children with congenital hemiplegia. Forty-eight child-caregiver dyads (children's mean age 7y 11mo, SD 2y 4mo; 33 males, 15 females; Manual Ability Classification System [MACS] level I, n=25, and level II, n=23; predominant motor type spastic hemiplegia, n=47) were recruited to this cross-sectional study. Children were assessed using the Melbourne Assessment of Unilateral Upper Limb Function (MUUL) and the Assisting Hand Assessment. Caregivers completed the Dimensions of Mastery Questionnaire, the Parenting Scale, and a demographic questionnaire. Consistent and positive parental disciplinary practices were associated with higher total motivation (p=0.006) and instrumental aspect scores (p=0.009). Children with siblings and from single-parent families experienced greater negative reactions to failure (p=0.006). Children from two-parent families (p=0.018) and with better bimanual performance (p=0.015) demonstrated greater object-oriented persistence. Age, sex, limitations in manual ability (MACS), and movement and body function of the impaired limb (MUUL) did not contribute significantly to mastery motivation. Inconsistent, excessively lax, and verbose parenting practices may discourage children from persevering with challenging tasks. Functional parenting styles, positive discipline practices, and autonomy-supportive strategies for task engagement should be encouraged when intervening with children with cerebral palsy. Parents should be supported to engage in these practices in all aspects of daily activities.