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Background Dystonia is characterized by sustained or intermittent muscle contractions, leading to abnormal posturing and twisting movements. In pediatric patients, dystonia often negatively influences quality of life. Pharmacological treatment for dystonia is often inadequate and causes adverse effects. Deep brain stimulation (DBS) appears to be a valid therapeutic option for pharmacoresistant dystonia in children. Methods To illustrate the current clinical practice, we hereby describe two pediatric cases of monogenetic movement disorders presenting with dystonia and treated with DBS. We provide a literature review of similar previously described cases and on different clinical aspects of DBS in pediatric dystonia. Results The first patient, a 6-year-old girl with severe dystonia, chorea, and myoclonus due to an ADCY5 gene mutation, received DBS in an elective setting. The second patient, an 8-year-old boy with GNAO1 -related dystonia and chorea, underwent emergency DBS due to a pharmacoresistant status dystonicus. A significant amelioration of motor symptoms (65% on the Burke-Fahn-Marsden Dystonia Rating Scale) was observed postoperatively in the first patient and her personal therapeutic goals were achieved. DBS was previously reported in five patients with ADCY5 -related movement disorders, of which three showed objective improvement. Emergency DBS in our second patient resulted in the successful termination of his GNAO1 -related status dystonicus, this being the eighth case reported in the literature. Conclusion DBS can be effective in monogenetic pediatric dystonia and should be considered early in the disease course. To better evaluate the effects of DBS on patients' functioning, patient-centered therapeutic goals should be discussed in a multidisciplinary approach. [ABSTRACT FROM AUTHOR]

AIM: to identify the use and utility of language comprehension tests for unintelligible or non-speaking children with severe cerebral palsy (CP). METHOD: severe CP was defined as severe dysarthria (unintelligible speech) or anarthria (absence of speech) combined with severe limited mobility, corresponding to Gross Motor Function Classification System levels IV to V. An electronic search in the databases of PubMed, PsychInfo, Embase, and CINAHL was made of studies published between January 1965 and December 2008. Indexing terms and free-text terms for 'cerebral palsy', 'language', and 'instrumentation' were used. Studies were included when (1) the focus was to investigate comprehension of spoken language of children (0-18 y) with severe CP, and (2) language tests were described. RESULTS: twelve standardized tests and five experimental instruments were identified. All standardized tests were developed for children without limited mobility. Only the Peabody Picture Vocabulary Test - Revised was frequently used and feasible for older children with severe CP (> 9 y). The other tests were used occasionally. To establish utility, adaptations of standardized test procedures were necessary. INTERPRETATION: language comprehension tests for children with severe CP are scarce. A language comprehension test specifically designed for these children is warranted. [ABSTRACT FROM AUTHOR]

Purpose: Reliable assessment of language comprehension is difficult for children with significant speech and motor limitations. The Computer-Based instrument for Low motor Language Testing (C-BiLLT) was designed for children with cerebral palsy (CP) and speech and motor limitations. A Canadian English version (C-BiLLT-CAN) has been validated. However, early investigation identified feasibility challenges necessitating further exploration. This study aimed to understand parents' perceived barriers and facilitators to implementing the C-BiLLT-CAN in the Canadian clinical context., Materials and Methods: Seven focus groups were conducted synchronously online with 16 parents from five Canadian provinces/territories. Transcripts were analyzed using semi-deductive thematic analysis, framing results within the Consolidated Framework for Implementation Research (CFIR)., Results: Parents unanimously expressed interest in making the C-BiLLT-CAN clinically available. Facilitators and barriers were discussed under five themes. Key facilitators included the unique design, standardized nature, and potential flexibility of the C-BiLLT-CAN. Barriers involved the inability to accommodate all children, the potential for unintended assessment impacts, and clinics' readiness and willingness to prioritize implementation., Conclusions: This study contributes new knowledge surrounding the assessment needs of parents of children with CP and speech and motor limitations. Alongside findings from a parallel clinician study, results will inform adaptations to the C-BiLLT-CAN to facilitate implementation.

Purpose: Cerebral palsy (CP) is the most prevalent motor disability affecting children. Many children with CP have significant speech difficulties and require augmentative and alternative communication (AAC) to participate in communication. Despite demonstrable benefits, the use of AAC systems among children with CP remains constrained, although research in Canada is lacking., Method: Data were collected as part of an exploratory survey of Canadian caregivers and clinicians ( N = 60) who shared their perspectives on children's need for, access to, and use of AAC systems. Quantitative data were summarized using descriptive statistics. Qualitative data were analyzed using inductive content analysis., Results: Caregivers and clinicians reported high rates of need for AAC systems (> 75%) among children with CP. Both groups reported that access was roughly equivalent to need, although caregiver reports were lower. Despite high rates of need and access, only 38% of children used AAC systems. Children who used AAC primarily used high-tech systems, mostly to make choices, rather than engaging in meaningful reciprocal interactions and conversations., Conclusions: Canadian children with CP who required AAC systems generally received them. However, AAC systems were not used to their full potential, suggesting limited participation in social and learning situations. Like reports on other pediatric populations, barriers to obtaining AAC systems related to service, family, and child-specific factors. Although our sample captured the complexity present in the CP population, sample sizes were small and unlikely to be representative of the population of Canada, indicating the need for further research on a national scale., Supplemental Material: https://doi.org/10.23641/asha.28229720.

Purpose: This study aimed to 1) investigate the convergent and discriminant validity, internal consistency, and test-retest reliability of the Canadian English version of the Computer-Based instrument for Low motor Language Testing (C-BiLLT-CAN), and 2) explore feasibility of the C-BiLLT assessment for children with cerebral palsy (CP) and complex communication needs in the Canadian health care context., Methods: Eighty typically developing children between 1.5 and 8.5 years of age completed the C-BiLLT-CAN, the Peabody Picture Vocabulary Test-IV (PPVT-4), the receptive language sub-test of the New Reynell Developmental Language Scales (NRDLS), and/or the Raven's 2. Correlations between raw scores were calculated for estimates of convergent and discriminant validity. Internal consistency was calculated for all items and separately for items pertaining to vocabulary and grammar. To calculate the standard error of measurement (SEM) and intraclass correlation coefficient (ICC), 33 participants were re-tested with the C-BiLLT within three weeks. Feasibility was explored with nine participants with CP., Results: C-BiLLT-CAN's convergent validity was good to excellent (Spearman's rho > 0.78) and discriminant validity was higher than hypothesized (Spearman's rho > 0.8). Internal consistency (Cronbach's alpha = 0.96), test-retest reliability (ICC > 0.9), and measurement error (SEM < 5%) were excellent. The feasibility study could not be fully completed due to the COVID-19 pandemic. Preliminary data demonstrated some technical and practical barriers for using the C-BiLLT in children with CP in Canada., Conclusion: The C-BiLLT-CAN showed good to excellent psychometric properties in a sample of typically developing children, indicating that it is an adequate test for measuring language comprehension in English-speaking Canadian children. Further research is needed to investigate the feasibility of the C-BiLLT-CAN in children with CP.

Background: Dystonic cerebral palsy is primarily caused by damage to the basal ganglia and central cortex. The daily care of these patients can be difficult due to dystonic movements. Intrathecal baclofen treatment is a potential treatment option for dystonia and has become common practice. Despite this widespread adoption, high quality evidence on the effects of intrathecal baclofen treatment on daily activities is lacking and prospective data are needed to judge the usefulness and indications for dystonic cerebral palsy. The primary aim of this study is to provide level one clinical evidence for the effects of intrathecal baclofen treatment on the level of activities and participation in dystonic cerebral palsy patients. Furthermore, we hope to identify clinical characteristics that will predict a beneficial effect of intrathecal baclofen in an individual patient., Methods/design: A double blind placebo-controlled multi-center randomized clinical trial will be performed in 30 children with dystonic cerebral palsy. Patients aged between 4 and 25 years old with a confirmed diagnosis of dystonic cerebral palsy, Gross Motor Functioning Classification System level IV or V, with lesions in the cerebral white matter, basal ganglia or central cortex and who are eligible for intrathecal baclofen treatment will be included. Group A will receive three months of continuous intrathecal baclofen treatment and group B will receive three months of placebo treatment, both via an implanted pump. After this three month period, all patients will receive intrathecal baclofen treatment, with a follow-up after nine months. The primary outcome measurement will be the effect on activities of and participation in daily life measured by Goal Attainment Scaling. Secondary outcome measurements on the level of body functions include dystonia, spasticity, pain, comfort and sleep-related breathing disorders. Side effects will be monitored and we will study whether patient characteristics influence outcome., Discussion: The results of this study will provide data for evidence-based use of intrathecal baclofen in dystonic cerebral palsy.

Patients with severe cerebral palsy (CP) often have poor speech ability but potentially better language comprehension. The arcuate fasciculus and the extreme capsule are two important language tracts between the Wernicke and Broca areas. Using diffusion tensor imaging, we visualized language tracts and pyramidal tracts in both hemispheres in 10 controls (5 to 18 years) and 5 patients (5 to 23 years) with severe CP. Language comprehension was assessed with a recently designed instrument (the Computer-Based instrument for Low motor Language Testing [C-BiLLT]). The language tracts were visualized in all control children and in four CP patients. In one CP patient without any objective language comprehension skills, no language tract could be visualized. Both language and pyramidal tracts were smaller in patients than in controls. These preliminary data indicate a relation between language tracts and language skills. Further research is necessary to study the value of structural integrity of language tracts in predicting language comprehension in CP patients., (Georg Thieme Verlag KG Stuttgart · New York.)

This paper aims to validate a head mounted inertial interface to characterize disorder movements in people with cerebral palsy (CP). The kinematic patterns extracted from this study will be used to design an alternative communication channel (using head motion) adapted to user's capabilities and limitations. Four people with CP participated (GMFCS level V) and three healthy subjects as reference group. The main outcome measures were divided into 1) Time-domain, 2) Frequency-domain and 3) Spatial domain. Results showed that the inertial interface succeeds assessing the pathological motion. Firstly, the system differentiates between voluntary and involuntary motion in terms of motor control, frequency and range of motion. Secondly some motion disorders such as hypertonia, hypotonia can be identified. These results suggest that people with motor disorders could benefit from the developed inertial system in three fields: 1) diagnosis of motor disorder by means of an objective quantification, 2) physical and cognitive rehabilitation by means of proprioceptive enhancement through visual-motor feedback and 3) functional compensation by means of an inertial person-machine interface for controlling computer and assistive devices (e.g. wheelchairs or walkers)., (© 2011 IEEE)