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Early motor repertoire and developmental function outcomes in infants with West syndrome: a case series
Gülsen Sırtbaş-Işık1, Bilge Nur Yardımcı-Lokmanoğlu1, Hasan Tolga Çelik2
1Faculty of Physical Therapy and Rehabilitation, Hacettepe University, Samanpazarı, Ankara, Türkiye.
Insights
Infants with West syndrome (WS) showed abnormal early motor skills and severe developmental delays. Early motor repertoire may predict later developmental outcomes in infants with WS, warranting further investigation.
Area of Science:
- Pediatric Neurology
- Developmental Neuroscience
- Epileptology
Background:
- West syndrome (WS), or infantile spasms, is a severe, rare epilepsy presenting in early infancy.
- Early identification and assessment of developmental trajectories in WS are crucial.
Purpose of the Study:
- To describe the early motor repertoire of infants with West syndrome.
- To examine the association between early motor repertoire and later developmental outcomes in infants with WS.
Main Methods:
- Case series involving three infants diagnosed with West syndrome.
- General Movement Assessment (GMA) used to evaluate early motor repertoire at 4 and 12 weeks post-term.
- Bayley Scales of Infant and Toddler Development - Third Edition (Bayley-III) administered at multiple time points to assess cognitive, language, and motor development.
Main Results:
- Infants exhibited abnormal movements, including poor repertoire and cramped-synchronized movements, with low General Movement Optimality Scores (GMOS) and Motor Optimality Scores (MOS).
- All infants demonstrated sporadic or absent fidgety movements.
- Bayley-III assessments revealed severe developmental delay across all sub-domains (scores <70) at all follow-up evaluations.
Conclusions:
- Infants with West syndrome present with suboptimal early motor repertoire and significant developmental delays.
- Early motor repertoire assessment may serve as a predictive indicator for later developmental function in infants with WS.
- Further research is recommended to validate early motor repertoire as a prognostic tool for developmental outcomes in West syndrome.
Background:
West syndrome (WS), also known as infantile spasm, is a rare form of severe epilepsy that begins during early infancy. This case series aimed to describe the early motor repertoire and examine the developmental function outcomes of infants with WS.
Case Descriptions:
Three infants (one female) with WS were assessed for early motor repertoire using the General Movement Assessment (GMA) which determined General Movement Optimality Scores (GMOS) at 4 post-term weeks of age, and Motor Optimality Scores (MOS) at 12 post-term weeks of age. Cognitive, language, and motor development were evaluated with the Bayley Scales of Infant and Toddler Development - Third Edition (Bayley-III) at 3, 6, 12, and 24 months of age.
Outcomes:
At 4-weeks post-term, one infant showed poor repertoire movements, while the other two showed cramped-synchronized movements with their GMOS ranging from 6 to 16 (out of 42). All infants showed sporadic/absent fidgety movements at 12 weeks post-term with their MOS ranging from 5 to 9 (out of 28). All sub-domain scores of Bayley-III were <2 SD at all follow-up assessments, that is <70, indicating severe developmental delay.
Conclusion:
These infants with WS had less than optimal scores of early motor repertoire, and developmental delay at a later age. Early motor repertoire might be an early sign for developmental function outcome at a later age in this population suggesting the need for additional research.
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