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Related Experiment Videos

Dandy-Walker syndrome and associated anomalies.

J A Golden1, L B Rorke, D A Bruce

  • 1University of Pennsylvania, School of Medicine, Philadelphia.

Pediatric Neuroscience
|January 1, 1987
PubMed
Summary

Dandy-Walker syndrome often co-occurs with other brain and body malformations in children. Associated anomalies complicate understanding the embryogenesis of this rare neurological condition.

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Area of Science:

  • Neurology
  • Pediatrics
  • Developmental Biology

Background:

  • Dandy-Walker syndrome is a congenital brain malformation.
  • Its embryogenesis and associated anomalies require further investigation.

Purpose of the Study:

  • To investigate the spectrum of malformations associated with Dandy-Walker syndrome.
  • To explore potential etiological factors based on observed anomalies.

Main Methods:

  • Retrospective study of 28 pediatric patients with Dandy-Walker syndrome.
  • Analysis of postmortem examinations (16 cases) and clinical follow-up data (12 cases).

Main Results:

  • Hydrocephalus and central nervous system malformations were common (11/16) in postmortem cases.
  • Visceral anomalies were present in 6/16 postmortem cases.
  • Clinically followed children showed fewer severe malformations, but neurological deficits and abnormal EEGs were frequent.

Conclusions:

  • Dandy-Walker syndrome frequently presents with diverse central nervous system and visceral anomalies.
  • The high rate of associated malformations hinders the proposal of a single unifying hypothesis for its embryogenesis.

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