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Neurological dysfunction above cele level in children with spina bifida cystica: a prospective study to three years
1Department of Paediatrics, University Children's Hospital, Uppsala, Sweden.
Insights
Most children with spina bifida cystica develop neurological dysfunction. Severe symptoms in infants are linked to myeloschisis, tethered cord, and shunt issues.
Area of Science:
- Pediatric Neurology
- Developmental Neuroscience
- Spinal Cord Malformations
Background:
- Spina bifida cystica is a complex congenital condition.
- Neurological deficits above the cele level present significant challenges.
- Early characterization is crucial for management.
Purpose of the Study:
- To characterize neurological dysfunction above the cele level in neonates with spina bifida cystica.
- To identify factors associated with severe neurological impairment.
- To track neurological development up to three years of age.
Main Methods:
- Prospective investigation of 22 neonates with spina bifida cystica.
- Serial assessments including MRI, vocal cord function, and physical therapy evaluations.
- Follow-up to a median age of three years.
Main Results:
- 18 out of 22 children developed neurological dysfunction above the cele level.
- 19 children had associated Chiari malformation.
- Severe impairment (respiration, feeding, motor) within the first three months was linked to myeloschisis, tethered cord, and shunt dysfunction.
Conclusions:
- Neurological dysfunction above the cele level is common in spina bifida cystica.
- Early-onset severe impairment indicates a poorer prognosis.
- Associated conditions like Chiari malformation and myeloschisis are significant factors.
Abstract:
The aim of this study was to characterize the neurological dysfunction above the cele level in children with spina bifida cystica. 22 neonates were investigated prospectively to a median age of three years. Before primary closure of the spinal malformation and at three and 18 months of age, MRI and inspection of vocal cord function were performed. The children were also assessed by a physical therapist at 12 and 24 months, 19 children had a Chiari malformation, 18 children developed neurological dysfunction above the cele level. Children with signs of isolated motor impairment stabilized or improved during the second year. Six children developed severe functional impairment of respiration, feeding and motor performance within the first three months of life. Severe neurological signs/symptoms were associated with myeloschisis, clinical signs of a tethered cord and recurrent periods of shunt dysfunction.