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Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation

  • Paediatric Neuroradiology
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Abstract

Half of patients with the Dandy-Walker malformation (DWM) have normal intellectual development. We aimed to identify feature on MRI associated with good intellectual prognosis. We reviewed 20  patients with DWM diagnosed on MRI, mean age 14.6±9.9 years. We assessed their intellectual development and related it to the MRI features. We found two groups with a statistically different intellectual outcome. All 14 patients with normal intellectual development had a normal lobulation of the vermis, without supratentorial anomalies. Of the six patients with mental retardation, three had an abnormal vermis, together with dysgenesis of the corpus callosum. In the other three, there were normal vermian anatomy with associated anomalies. Normal lobulation of the vermis, in the absence of any supratentorial anomaly, appears to be a good prognostic factor in DWM. Preservation of cerebrocerebellar pathways and neonatal plasticity could explain the normal intellectual development. These findings might be useful in prenatal diagnosis.

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Authors and Affiliations

  1. Paediatric Radiology Department, Hopital Necker Enfants-Malades, 149 rue de Sèvres, 75015, Paris, France

    N. Boddaert, N. Ferguson, P. Sonigo, L. Hertz-Pannier, J. Baraton, S. Emond, I. Simon, V. Chigot, P. Schmit & F. Brunelle

  2. ERM 0205 INSERM-CEA, Service Hospitalier Frederic Joliot, CEA, Orsay, France

    N. Boddaert & F. Brunelle

  3. Neurosurgical Department, Hopital Necker Enfants-Malades, 149 rue de Sèvres, 75015, Paris, France

    O. Klein, D. Parisot & A. Pierre-Kahn

Authors
  1. N. Boddaert
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  2. O. Klein
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  3. N. Ferguson
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  4. P. Sonigo
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  6. L. Hertz-Pannier
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  8. S. Emond
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  9. I. Simon
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  12. A. Pierre-Kahn
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  13. F. Brunelle
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Correspondence to N. Boddaert.

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Boddaert, N., Klein, O., Ferguson, N. et al. Intellectual prognosis of the Dandy-Walker malformation in children: the importance of vermian lobulation. Neuroradiology 45, 320–324 (2003). https://doi.org/10.1007/s00234-003-0980-6

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  • DOI: https://doi.org/10.1007/s00234-003-0980-6

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