Dystonia assessment in children with cerebral palsy and periventricular leukomalacia
Ioanna Papadimitriou1, Zoi Dalivigka2, Chrysa Outsika1
11st Department of Pediatrics, Aghia Sofia Children's Hospital, National and Kapodistrian University of Athens, Thivon and Levadias, Athens, 11527, Greece.
Insights
Dystonia is frequent and impacts motor function in cerebral palsy (CP) patients with periventricular leukomalacia. Its severity, not spasticity, correlates with functional disability, highlighting dystonia's key role.
Area of Science:
- Neurology
- Pediatrics
- Developmental Neuroscience
Background:
- Cerebral palsy (CP) is a common neurodevelopmental disorder.
- Periventricular leukomalacia (PVL) is a significant cause of CP, often associated with motor impairments.
- Dystonia is a motor disorder characterized by involuntary sustained muscle contractions, frequently observed in CP.
Purpose of the Study:
- To investigate the prevalence, motor characteristics, clinical presentation, and functional outcomes of dystonia in individuals with CP secondary to PVL.
- To elucidate the relationship between dystonia, spasticity, and functional abilities in this specific CP population.
Main Methods:
- Retrospective analysis of 31 patients diagnosed with CP and PVL.
- Utilized the Gross Motor Function Classification System (GMFCS) and Manual Ability Classification System (MACS) for functional assessment.
- Assessed dystonia presence and severity using video review and the Burke-Fahn-Marsden Dystonia Rating Scale; spasticity was measured with the Modified Ashworth Scale.
Main Results:
- All patients exhibited dystonia, frequently affecting limbs, neck, trunk, mouth, and eyes.
- Dystonia was multifocal in 71% of cases, with severity varying from mild to severe.
- Dystonia severity and distribution did not correlate with demographic or neonatal factors, but GMFCS and MACS scores correlated with dystonia, not spasticity.
Conclusions:
- Dystonia is a prevalent and significant motor impairment in CP patients with PVL.
- The severity of dystonia, rather than spasticity, is strongly associated with the degree of motor functional disability.
- These findings underscore the critical role of dystonia in determining motor function and disability in this patient cohort.
Objective:
To describe the frequency, motor phenotype, clinical patterns and functional consequences of dystonia in patients with cerebral palsy (CP) in the setting of periventricular leukomalacia.
Methods:
Retrospective analysis of a cohort of 31 patients with CP and periventricular leukomalacia. Gross Motor Function Classification System (GMFCS) and Manual Ability Classification System (MACS) were used to classify functional ability. Spasticity was rated using the Modified Ashworth Scale. Presence of dystonia was assessed by reviewing video recordings, and its severity by using the Burke-Fahn-Marsden Dystonia Rating Scale.
Results:
All patients showed evidence of dystonia involving upper and/or lower limbs, neck, trunk, mouth and eyes in order of frequency. In 29% of patients dystonia involved only the limbs and in 71% it was multifocal. Dystonia severity ranged from slight to severe. Severity and distribution of dystonia did not correlate with gender, age, weeks of gestation or duration of neonatal unit stay. GMFCS and MACS correlated with dystonia but not with spasticity.
Conclusions:
Severity of dystonia, but not spasticity is associated with the severity of motor functional disability in CP patients with periventricular leukomalacia and demonstrates the key role of dystonia in the motor function of these patients.
More Related Videos
Related Concept Videos
01:12Alterations in Muscle Tone ll
01:11Alterations in Muscle Tone lll