Neuropediatrics 2021; 52(S 01): S1-S53
DOI: 10.1055/s-0041-1739707
Freier Vortrag

Brain Malformations and Their Correlation to Cognitive Performance in Patients with Spina Bifida

Joanna Schneider
1   Neuropediatric, Center for Chronically Sick Children, Charité – Universitätsmedizin Berlin, Berlin, Germany
2   Department of Neuropediatrics, Charité – Universitätsmedizin Berlin, Berlin, Germany
3   Berlin Institute of Health, Berlin, Germany
,
Naomi Mohr
1   Neuropediatric, Center for Chronically Sick Children, Charité – Universitätsmedizin Berlin, Berlin, Germany
2   Department of Neuropediatrics, Charité – Universitätsmedizin Berlin, Berlin, Germany
,
Rainer John
1   Neuropediatric, Center for Chronically Sick Children, Charité – Universitätsmedizin Berlin, Berlin, Germany
,
Niko Aliatakis
1   Neuropediatric, Center for Chronically Sick Children, Charité – Universitätsmedizin Berlin, Berlin, Germany
2   Department of Neuropediatrics, Charité – Universitätsmedizin Berlin, Berlin, Germany
,
Birgit Spors
4   Department of Radiology, Charité – Universitätsmedizin Berlin, Berlin, Germany
,
Angela Kaindl
1   Neuropediatric, Center for Chronically Sick Children, Charité – Universitätsmedizin Berlin, Berlin, Germany
2   Department of Neuropediatrics, Charité – Universitätsmedizin Berlin, Berlin, Germany
› Author Affiliations
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    Background/Purpose: This retrospective study was aimed to systematically analyze the radiological abnormalities of the brain and spine in children and adolescents with spina bifida (SB) and their association with cognitive function. Based on this systematic analysis, corresponding differences between spina bifida aperta (SBA) and spina bifida occulta (SBO) were identified.

    Methods: The retrospective analysis included 265 patients (SBA, n = 206; SBO, n = 59). Magnetic resonance imaging of the brain and spine was used to determine the patients’ radiological phenotype. In 126 children and adolescents (SBA, n = 116; SBO, n = 10), cognitive testing was performed using KABC, WISC-IV/WAIS-IV.

    Results: While all patients with SBA had multiple brain malformations, only few patients with SBO showed brain abnormalities. The most common brain abnormalities included hydrocephalus (78%), abnormalities of the corpus callosum (69%), hypoplastic pons (50%), and hypoplastic mesencephalon (20%). The total IQ score in SBA patients was significantly lower than in SBO patients (WISC-IV/WAIS-IV, p = 0.007; KABC, p = 0.01). We detected also significant differences across several cognitive domains based on the presence of various brain malformations. Stenogyria (p = 0.006), pons hypoplasia (p = 0.003), and mesencephalon hypoplasia (p = 0.01) correlated with lower total IQ score and verbal comprehension. The lesion level of the defect correlated only with the processing speed.

    Conclusion: We show the first large systematic report of brain malformations in SB and the association of particular brain malformations with a lower IQ. Due to the significant differences between the SBA and SBO in terms of brain malformations and cognitive abilities, these two groups should be considered separately, including prenatal counseling.

    Study published in: Dev Med Child Neurol. 2021;63(3):295–302. doi: 10.1111/dmcn.14717


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    No conflict of interest has been declared by the author(s).

    Publication History

    Article published online:
    28 October 2021

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