Gross Motor Outcomes After Dynamic Weight-Bearing in 2 Children With Trunk Hypotonia: A Case Series

NTRODUCTION: Children with trunk hypotonia may have limited ability to maintain an upright weight-bearing position, resulting in decreased postural control and a delay in achieving gross motor milestones. PURPOSE: The purpose of this case series is to report the effect of a home-based dynamic standing program on postural control and gross motor activity in 2 children with trunk hypotonia. DESCRIPTIONS: Child 1 (aged 24 months, Gross Motor Function Classification Scale Level IV) and Child 2 (aged 21 months, Gross Motor Function Classification Scale Level V) participated in a standing program using the Upsee harness at home 3 days per week for 12 weeks. OUTCOMES: Both children improved their gross motor function, and Child 1 demonstrated improved trunk control in sitting. WHAT THIS CASE ADDS: The use of the Upsee harness was an effective intervention for these children with trunk hypotonia to achieve weight-bearing and improve gross motor abilities

Relationship between Movement Quality, Functional Ambulation Status, and Spatiotemporal Gait Parameters in Children with Myelomeningocele.

Aims: We investigated relationships among the Pediatric Neuromuscular Recovery Scale (Peds NRS), modified Hoffer Scale, and spatiotemporal gait parameters in children with myelomeningocele (MMC). Methods: 21 children with MMC, age 5.3 years (SD = 2.6), were assessed by three clinicians using the Peds NRS and modified Hoffer Scale. In eight children, gait parameters were also measured. Results: The Peds NRS summary score demonstrated good correlation with modified Hoffer Scale score (r = -0.64, p = 0.002) that accounted for 41% of variation in summary score. Six Peds NRS seated/standing items exhibited good relationships with modified Hoffer Scale (r = -0.51 to -0.70, p≤ 0.023), and the sit-to-stand item demonstrated an excellent relationship (r = -0.85, ps = 0.81 to 0.88, p≤ 0.014), and swing and stance time (both Rs = -0.83 to -0.90, p≤ 0.01). Two Peds NRS standing items and modified Hoffer Scale score demonstrated good correlations with velocity (Rs = 0.71, p= 0.047; Rs = -0.73, p= 0.04, respectively). Conclusions: Our findings suggest that children with MMC who exhibit greater movement quality and trunk control are likely to be functional ambulators with more optimal spatiotemporal gait parameters

Upsee Daisy! Gross Motor Outcomes after Dynamic Weight Bearing in Two Children with Truncal Hypotonia: A Case Series

PURPOSE: The purpose of this case series was to report the impact of a home-based dynamic standing program on postural control and gross motor activity in two children with truncal hypotonia. BACKGROUNDS/SIGNIFICANCE: Poor postural control associated with central hypotonia limits a child’s ability to interact with the environment, delaying attainment of developmental milestones such as sitting, crawling, standing and walking. Participation in social interactions with peers and caregivers may be restricted resulting in a decreased quality of life. Supported standing programs are commonly used to ameliorate impairments and optimize function in children with poor postural control. Increased social interactions, as well as reduced burden of care have all been associated with supported standing programs. The Upsee is an orthotic standing and walking device which is worn by the child and parent. As the adult stands, moves, and walks the child is encouraged to stand, move, and step. Functional tasks and participation activities are encouraged while the child is in the Upsee. There are no reports of the effectiveness of the Upsee as a dynamic standing program. Because of its simple design and ease of use, the Upsee can be readily added to a home program to increase standing and weight shifting in children. SUBJECTS: Child 1 was a 24-month-old boy born at 37 weeks gestation and diagnosed at birth with a rare form of chromosome 3 deletion and agenesis of the corpus callosum. His Gross Motor Function Classification System (GMFCS) classification was Level IV. Child 2 was a 21-month-old boy born at 39 weeks gestation. Prior to birth, his mother experienced decreased fetal movement. A cesarean section was performed and the baby was found to have a true umbilical cord knot, causing hypoxic ischemic encephalopathy. He was diagnosed with Cerebral Palsy (CP) at 12 months of age. His GMFCS classification was Level V. Both children displayed truncal hypotonicity with significant gross motor delays for their ages. They both had cognitive ability to follow simple commands, but no verbal communication skills. The children had excellent family support and parents of both children expressed an overall goal for improvement with functional independence. METHODS AND MATERIALS: Each child participated in 12 weeks of a home-based program that included upright dynamic weight bearing using the Upsee device. The harness system was adjusted by a physical therapist during the first assessment period to provide appropriate anatomical alignment and positioning for the children to bear weight through their lower extremities. The families were educated in the correct use of the device, and followed a protocol that included up to 30 minutes of weight bearing per day, 3 to 5 days a week. The parents were educated on dynamic weight bearing to facilitate muscle activation in the trunk and legs while using the Upsee. Families were asked to keep a journal of the amount of time spent in the device and activities performed by their child. ANALYSES: The Segmental Assessment of Trunk Control (SATCo) and the Gross Motor Function Measure (GMFM- 66) identified changes in gross motor function and trunk control. Pre- and post-intervention scores were compared. RESULTS: Final scores revealed that both children improved gross motor function, and Child 1 demonstrated improved trunk control in sitting. Child 1 improved his SATCo score from 11/20 to 20/20 and GMFM-66 score from 35 to 42. Child 2 initially scored 0/20 on the SATCo outcome measure, which did not change. His GMFM-66 score improved from 16 to 21. By the end of the study period, both children were able to take steps independently with a gait trainer. This new ability expanded opportunities for physical therapy goals and interventions for both children. CONCLUSIONS: Children with truncal hypotonia can participate in, and benefit from, a dynamic standing program using the Upsee. We speculate that this program may be effective in improving the gross motor abilities of children with severely impaired postural control. Of the two children, Child 1 demonstrated more improvement in gross motor function and trunk control. One possible reason for this is that Child 2 presented with increased extensor tone of his four extremities, which increased when he became excited or when attempting to focus on an activity at hand and constrained his movement activity. Also, Child 2 spent less time overall in the Upsee, never progressing beyond 15 minutes of weight bearing per session. Future research is needed to further explore the necessary dosage of an upright dynamic weight bearing program for children with impaired postural control. Studies with a more homogenous and larger sample are needed to conduct a randomized controlled trial design comparing the use of the Upsee to a traditional standing frame. Future studies should also aim to capture changes across the full ICF-CY model, including activities and participation. The findings from this case series support the use of the Upsee as a new home-based upright dynamic weight bearing program for children with impaired postural control

Interrater Reliability of the Pediatric Neuromuscular Recovery Scale in Children with Spina Bifida

Purpose: There is a paucity of outcome measures to assess the quality of functional mobility in children with spina bifida1. The Pediatric Neuromuscular Recovery Scale (Peds NRS) is a valid and reliable outcome measure that was developed to assess the motor capacity of children with spinal cord injury2-6. The purpose of this study was to examine the interrater reliability of the Pediatric Neuromuscular Recovery Scale (Peds NRS) to classify motor capacity in children with myelomeningocele (MMC) form of spina bifida. Subjects: Twenty-one children with MMC (1.4- 10 years of age; mean age of 5.3 years) were recruited from clinics and support groups within a 250-mile radius of Austin, TX. Four clinicians, two physical therapists (PTs) and two occupational therapists (OTs) served as the raters for the study. Raters had an average of 14 years of clinical practice and 11 years of pediatric practice. Methods: Each child with MMC was scored on the Peds NRS three times: two live testing sessions and one video recorded session. Every child was scored by two PTs and one OT. Interrater reliability was analyzed using intraclass correlation coefficients (ICC) for individual items and the summary score. Results: The Peds NRS summary score demonstrated good reliability (ICC = 0.89; 95% CI, .80-.95). For the sixteen individual items, reliability was excellent for items forward reach and grasp (right and left), static stand, and walking (ICC= .919-.969), good for supine to sit, sit inside base of support, sit outside base of support, in-hand manipulation (right), overhead reach (right and left), sit to stand, dynamic stand, and step retraining (ICC= .765-.890) and moderate for in-hand manipulation (left), stand adaptability, step adaptability (.511-.745). None of the items had poor reliability. The summary score had consistent reliability across age categories and groups defined by modified Hoffer level. There was no difference in the summary scores among all raters at F(2,60)= .220, p=.804. Conclusions: Pediatric clinicians were able to reliably administer and score the Peds NRS on children with MMC, representing a wide range of ages and functional levels. Clinical Relevance: This is the first investigation of the use of the Peds NRS in children with MMC. This study adds to the literature regarding the psychometric properties of the Peds NRS and supports the use of this outcome measure as an instrument to assess motor capacity in children with MMC

Feasibility and Preliminary Outcomes of a Standardized Exercise Program in Adults with Down Syndrome: A Pilot Study

Purpose/ Hypothesis: Youth with Down Syndrome (DS) have limited participation in physical activity. When compared to their typically developing peers, adults with DS display decreased functional mobility and strength. Exercise has shown to improve the physical fitness, overall health, and quality of life in people with DS. The Lee Silverman Voice Treatment (LSVT® BIG) is an effective treatment approach designed for patients with Parkinson’s disease. The core components of LSVT® BIG include large amplitude movements, sensory calibration, high intensity, and maximum effort while performing the daily exercises. Due to hypotonicity associated with DS, these individuals typically display kyphotic posture, shortened step length and overall lower amplitude movements. The LSVT® BIG protocol has the potential to improve muscle tone and overall fitness in adults with DS similar to the improvements seen in the Parkinson’s disease population, but the effects have yet to be studied. The purpose of this pilot study was to test the feasibility of implementing a 4 week standardized group exercise program in adults with DS, and assess whether subjects improved after this protocol. Number of Subjects: A convenience sample of 8 adults with DS were recruited from a residential facility. Materials/Methods: This exploratory, feasibility pilot study employed a pretest-posttest design. Outcome measures were assessed at baseline and at one week post intervention. The independent variable for this study was the LSVT® BIG standardized exercise protocol. Outcome measures included: the Modified Clinical Test of Sensory Interaction on Balance (mCTSIB) and timed single leg stance (SLS) for balance, the 10 meter walk test (10MWT) for gait speed, the 30 second sit-to-stand test for lower extremity functional strength, and the timed-up-and-go (TUG) for overall functional mobility. Each participant completed the established LSVT® BIG protocol in a group exercise format, totaling 16 sessions. Results: LSVT® BIG certified instructors were able to lead the group exercises for all 8 adults with DS with no adverse effects. Attendance varied between 10 to 16 sessions, with an average participation rate of 13.75 days (SD = 2.17). Standard LSVT® BIG modifications were easy to implement when needed. There was a statistically significant increase in self-selected gait speed (median self-selected gait speed post-test score, Mdn = 1.01, vs. median pre-test score, Mdn = 0.78, z = 35, p = 0.02). There were no other significant changes in functional strength, balance or fast gait speed, however the median scores improved for all dependent variables except for one of the mCTSIB scores and 30 second sit-to-stand test. Conclusions: A 4 week high-amplitude standardized exercise program can improve self-selected gait speed in adults with DS, but may not have a statistically significant effect on their balance or functional strength. Clinical Relevance: The LSVT® BIG standardized exercise protocol can be used for adults with DS in a group exercise setting and may also improve the gait speed for this population

New Marsileaceous Fossils from the Late Cretaceous of South America and a Reevaluation of Marsileaceaephyllum.

Herein we report new macrofossils and associated microfossils representing the aquatic fern family Marsileaceae from the Campanian to Maastrichtian Cañadón del Irupé locality, La Colonia Formation, Chubut Province, Argentina. The macrofossils include compound leaves, detached leaflets, and sporocarps. The venation of the leaflets is consistent with the diagnosis of Marsileaceaephyllum, which we argue is poorly defined. Consequently, the circumscription and diagnosis of Marsileaceaephyllum are emended in order to limit the taxon to include only leaves or rhizomes bearing leaves with four leaflets that most resemble those of Marsilea among extant genera of Marsileaceae. The Cañadón del Irupé leaves and leaflets are assigned to the new genus Mirasolita, erected to encompass compound leaves bearing two petiolulate leaflets, each leaflet having a reniform lamina and reticulate venation. The architecture of Mirasolita leaves and leaflets is distinct from that of leaves of any of the living marsileaceous genera. The stalked sporocarps are assigned to the new genus Lugiomarsiglia. They are attributed to Marsileaceae because each has a sclerenchymatous wall surrounding circular bodies interpreted as sporangia. Associated megaspores are assigned to Molaspora lobata, a geographically widespread, primarily Late Cretaceous dispersed spore taxon. The co-occurrence of Molaspora lobata with an extinct marsileaceous leaf form fits a pattern wherein Cretaceous Regnellidium-like megaspores are associated with or found in situ within marsileaceous macrofossils that display a variety of morphologies.Fil: Hermsen, Elizabeth. Ohio State University; Estados Unidos de América;Fil: Gandolfo, Maria A.. Cornell University; Estados Unidos de América;Fil: Cuneo, Nestor Ruben. Consejo Nacional de Investigaciones Científicas y Técnicas; Argentina. Museo Paleontologico Egidio Feruglio; Argentin