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Published on: January 12, 2019
Motor repertoire is age-inadequate in infants with cystic fibrosis
Haluk Tekerlek1, Akmer Mutlu2, Deniz Inal-Ince2
1Faculty of Physical Therapy and Rehabilitation, Hacettepe University, Ankara, Turkey. haluktekerlek@gmail.com.
Insights
Infants with cystic fibrosis (CF) show abnormal fidgety movements (FMs) and a lower motor optimality score (MOS). MOS may predict CF disease severity and guide early intervention planning for affected infants.
Area of Science:
- Pediatric neurology
- Developmental pediatrics
- Neonatology
Background:
- General movements (GMs) in infants, specifically fidgety movements (FMs), emerge between 9 and 20 weeks postterm.
- Cystic Fibrosis (CF) is a genetic disorder that can impact neurodevelopment.
- Evaluating FMs and motor repertoire in infants with CF is crucial for understanding early developmental trajectories.
Purpose of the Study:
- To assess fidgety movements (FMs) and motor repertoire in infants with cystic fibrosis (CF).
- To investigate the relationship between motor optimality score (MOS) and clinical findings in infants with CF.
- To explore the potential of MOS in predicting CF disease severity and informing early intervention strategies.
Main Methods:
- Analysis of 5-minute video recordings of 18 infants with CF and 20 healthy controls (age 3-5 months postterm).
- Utilized the Prechtl General Movement Assessment to evaluate FMs and motor repertoire.
- Calculated the Motor Optimality Score (MOS) for all participants.
Main Results:
- Infants with CF exhibited a significantly lower MOS compared to healthy controls (p < 0.05).
- Fifty percent of infants with CF displayed abnormal or absent/sporadic FMs.
- MOS showed a negative association with hospitalization duration (r = -0.378) and a positive association with vitamin A levels (r = 0.665) in CF infants.
Conclusions:
- Lower MOS and abnormal/sporadic FMs were observed in infants with CF, indicating potential developmental challenges.
- MOS demonstrated a correlation with clinical factors like hospitalization duration and vitamin A levels.
- MOS may serve as a valuable tool, alongside genetic testing, for predicting CF disease severity and guiding the implementation of targeted early intervention programs.
Background:
General movements (GMs) in infants occur as fidgety movements (FMs) between postterm 9 and 20 weeks. We aimed to evaluate FMs and motor repertoire in infants with cystic fibrosis (CF) and their relation with clinical findings.
Methods:
Demographic and clinical characteristics were recorded. FMs and motor repertoire were analyzed from a 5-min video recording of each infant. Videos were rated based on the Prechtl General Movement Assessment and motor optimality score (MOS) was calculated.
Results:
The analysis included 18 infants with CF and 20 healthy infants at postterm age of 3-5 months. MOS was significantly lower in the infants with CF compared to controls (p < 0.05). Fifty percent of the infants with CF had abnormal or absent/sporadic FMs. MOS was negatively associated with hospitalization duration (r = -0.378, p = 0.036); and positively associated with vitamin A level in CF infants (r = 0.665, p = 0.026).
Conclusions:
Infants with genetically anticipated severe CF phenotype tended to have lower MOS. MOS may be used in addition to genetic testing to predict disease severity in infants with CF. Infants with CF, absent/sporadic FMs, and lower MOS could be considered for planning specific age-adequate early intervention programs.
Impact:
Motor repertoire was age-inadequate in infants with cystic fibrosis (CF). 50% of infants with CF had abnormal or absent/sporadic fidgety movements (FMs). Motor optimality score (MOS) was positively associated with vitamin A level and negatively correlated with hospitalization duration in infants with CF. MOS tended to decrease as genetically anticipated disease severity increased; thus, MOS might enable us to predict disease severity in CF. The relationship between motor repertoire and phenotype and genotype is unclear and warrants further study. CF infants with absent/sporadic FMs, and lower MOS could be considered for planning early intervention.
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