The previously posted information about the Peabody Developmental Motor Scales-2 is current and up to date at this time.
A clinical series using intensive neurorehabilitation to promote functional motor and cognitive skills in three girls with CASK mutation.
The purpose of this study was to see if targeted physical therapy neurorehabilitation interventions could improve gross and fine motor skills, visual-motor coordination, social interaction, and communication in children with microcephaly due to a CASK gene mutation.
The participants in this study were three female children aged 54 months (Child A), 89 months (Child B), and 24 months old (Child C) that were diagnosed with microcephaly with pontine and cerebellar hypoplasia (MICPCH) due to a heterozygous mutation in the gene CASK. All of the participants had developmental delays and trouble with motor skills, communication and interactions including speech and cognition. All of the participants were already in some form of therapy and were behind typically developing children of their ages.
All three participants were assessed at the beginning of the trial using the Peabody Developmental Scale-2 (PDS-2) and their scores were recorded. All of the participants received treatment that focused on improving motor skills, social interactions and communications for four hours each weekday, for 10 days of treatment, for a total of 40 hours of therapy. After the trial treatments were over all of the participants were again assessed using the PDS-2 to see if changes had been made.
The Peabody Developmental Motor Scales-2 was given to all three participants both pre-intervention and post-intervention in order to see if there were improvements in the participants motor skills, coordination, social interactions and communication skills after the treatment was administered. The PDS-2 is made to be a reference for normative development in children from birth to 5 years old. Even though one of the participants (Child B) was older than 5 years the participant’s cognitive, communication, and motor skills were less than that of a typically developing 5 year old and thus the test was a good indicator of developmental age in this child. Five of the six subtests of the PDS-2 were used in this research (stationary, locomotion, object manipulation, grasping, and visual-motor integration), and only the reflex subtest was left out since it is targeted at children under the age of 12 months and none of the participants were of that age. Child C, who was only 24 months old, was also tested with the Gross Motor Functional Measure 88 (GMFM) since the child had limited mobility and the GMFM 88 is made for children with limited motor capabilities. Qualitative observations were also recorded throughout the study.
The intervention itself consisted of having the children participate in play and daily living activities that encouraged the children to target tasks using a technique called operant conditioning. Operant conditioning was used to reinforce specific skills that the child did, and then the therapist helped the child to link those skills together. Those skills linked together then became a more complicated task and the task was progressed as the child progressed. Progress was made through repetition of movements that the child was already doing and by encouraging movements, even unintentional movements, that were beneficial until the child intentionally repeated these movements as tasks. Similar therapy was done in terms of encouraging the children to copy speech and increase their vocabulary. All of these tasks were tailored specifically to each child to help with their unique development.
The raw scores showed that Child A improved +4 points on gross motor skills, decreased -3 points on fine motor skills for a total motor quotient of +1 points. Child B improved +2 points on gross motor skills, decreased +9 points on fine motor skills for a total motor quotient of +4 points. Child C improved +11 points on gross motor skills, decreased +21 points on fine motor skills for a total motor quotient of +14 points and had an increase of +23 points on the GMFM 88. The only subtest that had a significant difference from pre-test to post-test on all three children was the stationary motor skills subtest with a t = 12.12, p = .007. When the raw scores were changed to age-equivalents for the PDS-2 the results showed that Child A improved an equivalent of 14 months, Child B improved an equivalent of 39 months, and Child C improved an equivalent of 20 months.
Strengths of the Article
This article did a very good job of explaining this study in a streamlined and easy to understand way. It was easy to see in the study that the therapy was very beneficial to all of the participants looking at both the qualitative and quantitative data. The article was clear about explaining why each test was administered and what variations had to be taken in order to accommodate the specific children such as including the GMFM 88 for the youngest child. This study also showed how they modified not only the test but the treatment to target each child at their developmental stage.
Limitations of the Article
One of the main limitations of this study is that there were only three participants. This study would have been a lot stronger if the population tested was larger. Another limitation of this study is that this genetic disease manifests differently with each person and it is impossible to say that this therapy would work on all children with MICPCH. The article also could have done a better job of explaining the types of therapy utilized by the patients in greater detail. Lastly, the fact that one of the participants was over 5 years old even though the PDS-2 was made for children from birth to five years was a limitation in this study.
According to this study this intensive form of neurorehabilitation therapy using operant condition can be extremely beneficial in progressing developmental skills in children of different ages with MICPCH due to a CASK gene mutation. Based on this data, I would recommend this form of therapy for a child with developmental delays in motor skills, social interactions and communication due to MICPCH.
Deluca SC, Wallace DA, Trucks MR, Mukherjee K. A clinical series using intensive neurorehabilitation to promote functional motor and cognitive skills in three girls with CASK mutation. BMC Research Notes. 2017;10(1). doi:10.1186/s13104-017-3065-z.