Mitchell Schertz

A multi-site study of functional outcomes following a themed approach to hand-arm bimanual intensive therapy for children with hemiplegia.

This study investigated the effects of a theme‐based (‘magic’) variation of the hand–arm bimanual intensive therapy programme, in two different countries, in improving activity performance for children with hemiplegia, including those with severe movement restrictions.

Changing the model: a call for a re-examination of intervention approaches and translational research in children with developmental disabilities

Current services for children with disabilities are provided based largely on traditional approaches that lack evidence of efficacy. Clinicians and families are, therefore, left with few evidence-based alternatives. For example, neurodevelopmental treatment, a major treatment approach for children with cerebral palsy (CP), has been found to be of questionable value. Fortunately, however, the situation is changing. Neuroscientific research has begun to elucidate the fundamentals of brain plasticity, including how the developing brain interacts with the social environment and how the brain learns and recovers after insult. One such fundamental is that optimal brain plasticity occurs when interventions incorporate the following three essential elements: (1) intensive task repetition, (2) progressive challenges to the learner with increasing difficulty, and (3) the presence of motivators and rewards. Another fundamental, subsumed under the rubric term ‘motor learning’, is that training be task-oriented (and as such, motivating). Thus, training must involve active participation and skill acquisition of a functional goal. Finally, the practice must be structured. Concurrently, the development of valid and reliable instruments, together with focus on function by the adoption of the ICF, has improved our ability to measure change. All the above have contributed to T1 translational research, i.e. the application of basic science to clinical models. In such research, interventions developed for individuals with a disability demonstrated a significant improvement in function. Studies based on motor learning have been particularly exciting, demonstrating change in neural representations and function after intervention. Such studies could provide key insights into neuropathophysiology, thus enabling development of even more effective methods of treatment for children with disabilities. Much of the initial intervention work that incorporated research based on brain plasticity has come from adult stroke literature, where multiple studies have demonstrated the efficacy of constraint-induced movement therapy (CIMT), treadmill training, robotic training, neuromuscular stimulation and, more recently, training in virtual environments. Recent studies have extended these interventions to the cognitive domain. Studies have followed, with demonstrated efficacy for those with CP, in pediatric CIMT, and bimanual training, and for those with Down syndrome in treadmill training. Similarly, striking advances have been made in the treatment of children with autism using early, intensive behavioral intervention with good evidence supporting its efficacy. Common to these interventions is the fact that that they are intensive in application, substantially beyond usual and customary care, structured, and task-oriented. As such, it is quite notable how slowly these interventions are being introduced into the community. An example of this is CIMT, for which evidence is approaching Level I, yet currently is not widely available clinically, even in a large metropolitan area such as New York City that has a comprehensive research center. Is the delay in implementing novel intervention approaches a result of early skepticism, resistance to abandoning traditional practices, funding limitations, or lack of awareness of the emerging science? Recent evidence suggests, at least in part, the latter. Clinicians treating children with disabilities should take note. Provision of ongoing, once or twice-weekly therapy that lacks functional goals and rigorous measures of change should be strongly questioned. Conversely, in a recent study, traditional and conductive education therapies for children with CP were provided with high intensity yet were not found to be efficacious. Therefore, intensive application of a therapy that lacks a task-oriented approach should also be reconsidered. Thus it appears that treatment intensity is necessary but not sufficient. Important questions remain regarding other intervention components, including family characteristics, and future research will need to examine these. Challenges to the provision of intensive and novel interventions are inevitable. However, there is room for optimism as translational research continues to evolve and is gaining prominence. We will need to advocate for changes in therapies that are paid for by insurance or governmental agencies, thus allowing the science to dictate the funding rather than the reverse. We also

MRI-based radiologic scoring system for extent of brain injury in children with hemiplegia.

BACKGROUND AND PURPOSE: Brain MR imaging is recommended in children with cerebral palsy. Descriptions of MR imaging findings lack uniformity, due to the absence of a validated quantitative approach. We developed a quantitative scoring method for brain injury based on anatomic MR imaging and examined the reliability and validity in correlation to motor function in children with hemiplegia. MATERIALS AND METHODS: Twenty-seven children with hemiplegia underwent MR imaging (T1, T2-weighted sequences, DTI) and motor assessment (Manual Ability Classification System, Gross Motor Functional Classification System, Assisting Hand Assessment, Jebsen Taylor Test of Hand Function, and Childrens Hand Experience Questionnaire). A scoring system devised in our center was applied to all scans. Radiologic score covered 4 domains: number of affected lobes, volume and type of white matter injury, extent of gray matter damage, and major white matter tract injury. Inter- and intrarater reliability was evaluated and the relationship between radiologic score and motor assessments determined. RESULTS: Mean total radiologic score was 11.3 ± 4.5 (range 4–18). Good inter- (ρ = 0.909, P < .001) and intrarater (ρ = 0.926, P = < .001) reliability was demonstrated. Radiologic score correlated significantly with manual ability classification systems (ρ = 0.708, P < .001), and with motor assessments (assisting hand assessment [ρ = −0.753, P < .001]; Jebsen Taylor test of hand function [ρ = 0. 766, P < .001]; childrens hand experience questionnaire [ρ = −0. 716, P < .001]), as well as with DTI parameters. CONCLUSIONS: We present a novel MR imaging–based scoring system that demonstrated high inter- and intrarater reliability and significant associations with manual ability classification systems and motor evaluations. This score provides a standardized radiologic assessment of brain injury extent in hemiplegic patients with predominantly unilateral injury, allowing comparison between groups, and providing an additional tool for counseling families.

Long-term neurodevelopmental follow-up of children with congenital muscular torticollis

Congenital muscular torticollis is a common condition, but long-term neurodevelopmental follow-up is lacking. This study reports on neurodevelopmental outcome of 68 children, aged 7 to 9 years, with a history of congenital muscular torticollis, excluding children with torticollis due to other conditions. Thirty-eight children were examined for presence of neurodevelopmental disorders. Telephone interview data were available for an additional 30 children. Of those examined, 22/38 (57.9%) had or were at risk for a developmental disorder (attention-deficit hyperactivity disorder (ADHD), developmental coordination disorder, language impairment, autistic spectrum disorder) on at least 1 of the assessments administered, 23/38 (60.5%) had received developmental treatment during childhood. One child, based on a telephone interview, had a history of developmental treatment. Therefore, 30/68 (44.1%) children of the total sample demonstrated a developmental delay/disorder, currently (22/68) or previously (8/68). Our findings suggest congenital muscular torticollis to be a significant risk factor for later neurodevelopmental conditions with disorders presenting at different stages of development.

Frequency of pediatric migraine with aura in a clinic‐based sample

To assess the prevalence and risk factors for pediatric migraine with aura (MWA) among patients presenting to pediatric neurology clinics.

Cortical reorganization following injury early in life

The brain has a remarkable capacity for reorganization following injury, especially during the first years of life. Knowledge of structural reorganization and its consequences following perinatal injury is sparse. Here we studied changes in brain tissue volume, morphology, perfusion, and integrity in children with hemiplegia compared to typically developing children, using MRI. Children with hemiplegia demonstrated reduced total cerebral volume, with increased cerebrospinal fluid (CSF) and reduced total white matter volumes, with no differences in total gray matter volume, compared to typically developing children. An increase in cortical thickness at the hemisphere contralateral to the lesion (CLH) was detected in motor and language areas, which may reflect compensation for the gray matter loss in the lesion area or retention of ipsilateral pathways. In addition, reduced cortical thickness, perfusion, and surface area were detected in limbic areas. Increased CSF volume and precentral cortical thickness and reduced white matter volume were correlated with worse motor performance. Brain reorganization of the gray matter within the CLH, while not necessarily indicating better outcome, is suggested as a response to neuronal deficits following injury early in life.

Brain Plasticity following Intensive Bimanual Therapy in Children with Hemiparesis: Preliminary Evidence

Neuroplasticity studies examining children with hemiparesis (CH) have focused predominantly on unilateral interventions. CH also have bimanual coordination impairments with bimanual interventions showing benefits. We explored neuroplasticity following hand-arm bimanual intensive therapy (HABIT) of 60 hours in twelve CH (6 females, mean age 11 ± 3.6 y). Serial behavioral evaluations and MR imaging including diffusion tensor (DTI) and functional (fMRI) imaging were performed before, immediately after, and at 6-week follow-up. Manual skills were assessed repeatedly with the Assisting Hand Assessment, Childrens Hand Experience Questionnaire, and Jebsen-Taylor Test of Hand Function. Beta values, indicating the level of activation, and lateralization index (LI), indicating the pattern of brain activation, were computed from fMRI. White matter integrity of major fibers was assessed using DTI. 11/12 children showed improvement after intervention in at least one measure, with 8/12 improving on two or more tests. Changes were retained in 6/8 children at follow-up. Beta activation in the affected hemisphere increased at follow-up, and LI increased both after intervention and at follow-up. Correlations between LI and motor function emerged after intervention. Increased white matter integrity was detected in the corpus callosum and corticospinal tract after intervention in about half of the participants. Results provide first evidence for neuroplasticity changes following bimanual intervention in CH.

Imaging Predictors of Improvement From a Motor Learning–Based Intervention for Children With Unilateral Cerebral Palsy

Background. Motor-learning interventions may improve hand function in children with unilateral cerebral palsy (UCP) but with inconsistent outcomes across participants. Objective. To examine if pre-intervention brain imaging predicts benefit from bimanual intervention. Method. Twenty children with UCP with Manual Ability Classification System levels I to III, aged 7-16 years, participated in an intensive bimanual intervention. Assessments included the Assisting Hand Assessment (AHA), Jebsen Taylor Test of Hand Function (JTTHF) and Children’s Hand Experience Questionnaire (CHEQ) at baseline (T1), completion (T2) and 8-10 weeks post-intervention (T3). Imaging at baseline included conventional structural (radiological score), functional (fMRI) and diffusion tensor imaging (DTI). Results. Improvements were seen across assessments; AHA (P = 0.04), JTTHF (P < .001) and CHEQ (P < 0.001). Radiological score significantly correlated with improvement at T2; AHA (r = .475) and CHEQ (r = .632), but negatively with improvement on unimanual measures at T3 (JTTFH r = −.514). fMRI showed negative correlations between contralesional brain activation when moving the affected hand and AHA improvements (T2: r = −.562, T3: r = −0.479). Fractional Anisotropy in the affected posterior limb of the internal capsule correlated negatively with increased bimanual use on CHEQ at T2 (r = −547) and AHA at T3 (r = −.656). Conclusions. Children with greater structural, functional and connective brain damage showed enhanced responses to bimanual intervention. Baseline imaging may identify parameters predicting response to intervention in children with UCP.

The Role of Prematurity in Patients With Hemiplegic Cerebral Palsy

A multicenter retrospective study was conducted to investigate the perinatal factors, imaging findings and clinical characteristics of hemiplegic cerebral palsy with a particular focus on children born prematurely. Our cohort included 135 patients of whom 42% were born prematurely; 16% were extreme premature infants who were born at 30 weeks or earlier. Nineteen (14%) were twins. Right hemiplegia was slightly more common and accounted for 59% of the patients. Imaging findings of intraventricular hemorrhage and periventricular leukomalacia were more prevalent in premature children whereas stroke, porencephaly, cerebral hemorrhage and cerebral atrophy were more evenly distributed in both term-born and prematurely-born children (p < 0.01). The overall prevalence of epilepsy in the cohort was 26% with no differences in full-term compared to prematurely-born children. Regardless of the gestational birth age, intellectual deficits were more common in the presence of comorbidity of both hemiplegia and epilepsy (p < 0.05).

Neurodevelopmental outcomes in children with large temporal arachnoid cysts

OBJECTIVE Management of children with large temporal arachnoid cysts (TACs) remains controversial, with limited data available on their neurodevelopmental outcome. The aim of this study was to examine neurodevelopmental outcomes in children with large TACs. METHODS In this medical center-based cohort study, 25 patients (19 males) who were diagnosed in childhood with large TACs (9 patients [36%] with a Galassi type II and 16 patients [64%] with a Galassi type III TAC) were examined. The mean ± SD age at assessment was 11.1 ± 5.6 years (range 2.7-22 years). Twelve patients (48%) had right-sided, 12 (48%) had left-sided, and 1 (4%) had bilateral cysts. Nine patients (36%) underwent surgery for the cyst. The siblings of 21 patients (84%) served as control participants. Neurodevelopmental function was assessed using the Adaptive Behavior Assessment System (ABAS), Vanderbilt Behavioral Rating Scale (VBRS), and Developmental Coordination Disorder Questionnaire (DCDQ), and quality of life was measured using the treatment-oriented screening questionnaire (TOSQ). The results of all instruments except for TOSQ were compared with those of the sibling control participants. RESULTS The mean ± SD ABAS score of the patients was 93.3 ± 20.09 compared with 98.3 ± 18.04 of the sibling control participants (p = 0.251). Regarding the incidence of poor outcome (ABAS score < 80), there was a trend for more patients with TAC to have poor outcome than the sibling controls (p = 0.058). Patients who underwent surgery scored significantly worse with regard to the VBRS total score compared with those who did not (p = 0.020), but not on ABAS, DCD, or TOSQ. The mean score of the cognitive and psychological items on TOSQ was lower than that for the physical items (p < 0.001). CONCLUSIONS Children with a large TAC performed similarly to their sibling control participants in neurodevelopmental function. However, a subgroup of those with cysts did have an increased risk for poor outcomes in general function. Neurodevelopmental assessment should be part of the management of all patients with TAC.