Development of a Play-Based Motor Learning Approach (A.MO.GIOCO) in Children with Bilateral Cerebral Palsy: Theoretical Framework and Intervention Methodology.

The early intervention of motor training based on specific tasks and parent empowerment represents the new paradigm for the rehabilitation of children with Cerebral Palsy (CP). However, most published studies address the problem of the effectiveness of rehabilitation intervention without describing the treatment methodology or briefly mentioning it. The purpose of the study is to illustrate the development of a play-based motor learning approach titled A.MO.GIOCO (Apprendimento MOtorio nel GIOCO) and its systematization. Fifteen children aged between 2 and 6 years with bilateral CP will be enrolled and treated for 6-8 weeks (48 h). Motor Teaching methods applied by senior therapists have been extensively described, starting from rehabilitation goals and proposed therapeutic play activities, tailored to the functional profile of each child. This child-friendly rehabilitative approach (A.MO.GIOCO) refers to the systemic cognitive model of learning and movement control and is implemented in the context of spontaneous play activities and in the therapist-child-family interaction. In this study the theoretical framework of the approach and the process followed by the therapists to transfer it into rehabilitative practice are highlighted. As a result, an operational guide has been created. Further studies will explore the efficacy of the proposed standardized approach.

Being adults with cerebral palsy: results of a multicenter Italian study on quality of life and participation.

Cerebral palsy (CP) is still the most common cause of disability developing in infancy. How such a complex disorder affects adult life raises important questions on the critical issues to consider and the most appropriate care pathway right from early childhood. We conducted a multicenter study on a sample of 109 individuals with CP followed up from infancy and recalled for an assessment at ages ranging between 18 and 50 years (mean age 26 years). Semi-structured interviews and specific questionnaires (SF36, LIFE-H and Hollingshead Index) were conducted to assess general psychological state, quality of life, and socio-economic conditions. Our findings showed a globally positive perception of quality of life, albeit with lower scores for physical than for mental health. Our cases generally showed good scores on participation scales, though those with more severe forms scored lower on parameters such as mobility, autonomy, and self-care. These findings were investigated in more depth in interviews, in which our participants painted a picture showing that gradual improvements have been made in several aspects over the years, in the academic attainment and employment, for instance. On the downside, our sample reported persistent limitations on autonomy in daily life. As for the more profound psychological domain, there was evidence of suffering due to isolation and relational difficulties in most cases that had not emerged from the questionnaires. Our data have possible implications for the management of CP during childhood, suggesting the need to avoid an exclusive focus on motor function goals, and to promote strategies to facilitate communication, participation, autonomy, and social relations.

Unimanual and bimanual intensive training in children with hemiplegic cerebral palsy and persistence in time of hand function improvement: 6-month follow-up results of a multisite clinical trial.

This study aims to compare in hemiplegic children the effectiveness of intensive training (unimanual and bimanual) versus standard treatment in improving hand function, assessing the persistence after 6 months. A multicenter, prospective, cluster-randomized controlled clinical trial was designed comparing 2 groups of children with hemiplegic cerebral palsy, treated for 10 weeks (3 h/d 7 d/wk; first with unimanual constraint-induced movement therapy, second with intensive bimanual training) with a standard treatment group. Children were assessed before and after treatment and at 3 and 6 months postintervention using Quality of Upper Extremity Skills Test (QUEST) and Besta Scales. One hundred five children were recruited (39 constraint-induced movement therapy, 33 intensive bimanual training, 33 standard treatment). Constraint-induced movement therapy and intensive bimanual training groups had significantly improved hand function, showing constant increase in time. Grasp improved immediately and significantly with constraint-induced movement therapy, and with bimanual training grasp improved gradually, reaching the same result. In both, spontaneous hand use increased in long-term assessment.

Multisite trial comparing the efficacy of constraint-induced movement therapy with that of bimanual intensive training in children with hemiplegic cerebral palsy: postintervention results.

OBJECTIVE: The aim of this study was to compare the effects of modified constraint-induced movement therapy (mCIMT; restraint of unaffected limb combined with unimanual intensive rehabilitation) with those of a bimanual intensive rehabilitation treatment (IRP) in children with hemiplegic cerebral palsy after a 10-wk practice vs. standard treatment (ST). DESIGN: This study is a multicenter, cluster-randomized controlled clinical trial of tested groups of children with hemiplegic cerebral palsy treated using mCIMT, IRP, or ST. For 10 wks, in mCIMT and IRP, the intensive practice lasted 3 hrs/day, 7 days/wk; in ST, 1-hr sessions twice a week were provided. The primary outcomes are upper limb/hand function (Quality of Upper Extremity Skills Test) and activities of daily living (Besta Scale), which are assessed before and after treatment. One hundred five patients were recruited, 39 to the mCIMT group, 33 to the IRP group, and 33 to the ST group. RESULTS: IRP and mCIMT significantly improved paretic hand function both in the Quality of Upper Extremity Skills Test and in the Besta Scale, whereas ST did not. mCIMT improved grasp more than IRP did (P < 0.01), whereas bimanual spontaneous use in play increased more with IRP (P = 0.0005). Activities of daily living in 2- to 6-yr-olds improved more with IRP (P < 0.0001) than with mCIMT (P = 0.011). Unaffected limb improved more from bimanual practice (IRP; P = 0.02). CONCLUSIONS: More advantages resulted from intensive practice than in the standard one, in mCIMT for grasp and in IRP for bimanual spontaneous use and activities of daily living in younger children.

The syndrome of perisylvian polymicrogyria with congenital arthrogryposis.

BACKGROUND: Bilateral perisylvian polymicrogyria (BPP) is a well-recognized malformation of cortical development commonly associated with epilepsy, cognitive impairment, and oromotor apraxia. Reports have suggested the association of BPP with arthrogryposis multiplex congenita. We sought to investigate the clinical, electrophysiological, and neuroradiological features of this combined syndrome to determine if there are unique features that distinguish BPP with arthrogryposis from BPP alone. METHODS: Cases of BPP with congenital arthrogryposis were identified from a large research database of individuals with polymicrogyria. Clinical features (including oromotor function, seizures, and joint contractures), MR brain imaging, and results of neuromuscular testing were reviewed. RESULTS: Ten cases of BPP with congenital arthrogryposis were identified. Most cases had some degree of oromotor apraxia. Only a few had seizures, but a majority of cases were still young children. Electrophysiological studies provided evidence for lower motor neuron or peripheral nervous system involvement. On brain imaging, bilateral polymicrogyria (PMG) centered along the Sylvian fissures was seen, with variable extension frontally or parietally; no other cortical malformations were present. We did not identify obvious neuroimaging features that distinguish this syndrome from that of BPP without arthrogryposis. CONCLUSIONS: The clinical and neuroimaging features of the syndrome of BPP with congenital arthrogryposis appear similar to those seen in cases of isolated BPP without joint contractures, but electrophysiological studies often demonstrate coexistent lower motor neuron or peripheral nervous system pathology. These findings suggest that BPP with arthrogryposis may have a genetic etiology with effects at two levels of the neuraxis.

Multisite trial on efficacy of constraint-induced movement therapy in children with hemiplegia: study design and methodology.

OBJECTIVE: In the past decades, several treatment approaches have been used to improve upper limb function in hemiplegic cerebral palsy. Only recently has constraint-induced movement therapy emerged as a treatment approach for children with hemiplegic cerebral palsy with the aim of reversing the behavioral suppression of movement in the affected upper limb. To date, evidence on this treatment has been very poor and limited, because all currently available trials reveal methodological limitations and a need for additional research to support the application of this treatment technique. This article presents the methodological choices, design, and main characteristics of an ongoing controlled clinical trial on the effectiveness and safety of constraint-induced movement therapy combined with an intensive rehabilitation program and compared with two comparison groups: one treated with an intensive rehabilitation program and the other with standard treatment. METHODS: Twenty-one rehabilitation sites are currently recruiting patients with hemiplegic cerebral palsy, aged between 2 and 8 yrs, who have never undergone constraint therapy. Primary outcome measures include two major domains: upper limb motor ability (Quality of Upper Extremity Skills Test) and hand function assessment evaluating both grip function and spontaneous use of the affected side (Besta scale). Secondary outcome measures concern overall function, behavior, compliance, and satisfaction with treatment program of both child and family. Patients' follow-up is of 12 mos after treatment. RESULTS: Research in children has always been neglected in comparison with adults, because of ethical reasons regarding the use of children for experimental purposes. The consequence has been the utilization of treatment and assessment tools and techniques that have not always been tested in pediatric patients or evidence is very scarce. CONCLUSION: Discussing and working on pediatric research methods represents an urgent need in rehabilitation research.

Cognitive profiles and visuoperceptual abilities in preterm and term spastic diplegic children with periventricular leukomalacia.

Although relations between the extent of periventricular leukomalacia and neuropsychological performance in preterm children with spastic diplegia have been extensively investigated, studies on term children with spastic diplegia are rare. The authors examined 15 preterm children and 9 term children with spastic diplegia, all of whom had periventricular leukomalacia as a main magnetic resonance imaging (MRI) finding (excluding full-term spastic diplegic children with other MRI findings). Cognitive abilities (Griffith scale) and visuoperceptual abilities (Developmental Test of Visual Perception) were compared in the 2 groups and related to periventricular leukomalacia severity. Cognitive performance was substantially similar in the 2 groups. However, the overall Developmental Test of Visual Perception scores were below normal in the preterm and were normal in the term children; furthermore, visuoperceptual abilities were differentially affected in the preterm children, with visuomotor abilities more compromised than nonmotor visuoperceptual abilities. These children had similar cognitive performance and MRI findings, so the greater visuoperceptual compromise in the preterm group suggests a direct influence of prematurity, which may have adversely influenced the reorganization of visual centers and pathways following the initial developmental insult. The strabismus present in most preterm children would also have contributed to their greater visuoperceptual compromise. The authors conclude that the management of preterm and term children should differ, with concentration on visuoperceptual skills and rehabilitation in the former.

Hand function in children with hemiplegic cerebral palsy: prospective follow-up and functional outcome in adolescence.

The aim of this prospective study was to determine the outcome of affected hand impairment and disability in a group of 31 children (16 males, 15 females) with hemiplegic cerebral palsy who were referred consecutively to our rehabilitation service and followed from a mean age of 2 years and 7 months to a mean age of 12 years and 9 months. The hand function assessment protocol consisted of video-recorded procedures: one to assess grip and the other to assess the extent of spontaneous use of the affected hand. Assessments at outset and at latest follow-up showed that the series as a whole had greater hand impairment in spontaneous manipulation than in gripping tasks. Comparison of hand function before age 4 years with the latest assessment over age 11 years revealed a non-significant improvement in grip, whereas spontaneous hand use remained stable. All but one of the children who had good grip and hand use scores at first assessment maintained them over time unless they were not treated for hand dysfunction. Among the children with low scores at outset, the improvement over time was more marked in grip than spontaneous hand use and occurred mainly in the early years. These results suggest that to evaluate the real disability of the affected hand in children with hemiplegia, grip assessment is insufficient and that an instrument assessing spontaneous hand use in bilateral manipulation is required. Furthermore, intensive treatment focused on hand function should be planned in the early years for children with more severe hand impairment, whereas regular follow-up is sufficient for less affected children.