Isolated Dandy-Walker malformation associated with brain stem dysgenesis in male sibs.

@article{AbdelSalam2006IsolatedDM,
  title={Isolated Dandy-Walker malformation associated with brain stem dysgenesis in male sibs.},
  author={Ghada M H Abdel-Salam and Marwa Shehab and M S Zaki},
  journal={Brain & development},
  year={2006},
  volume={28 8},
  pages={529-33}
}
We describe two brothers with isolated Dandy-Walker malformation (DWM). Interestingly, brain stem dysgenesis and abnormal gyral pattern were also observed in the sibs. They presented with psychomotor retardation and macrocrania. Both suffered from hypotonia with brisk deep tendon reflexes and ataxic gait. They had bilateral optic atrophy and the visual evoked potentials documented prolonged latencies. Further, motor and sensory conduction velocities were normal. Chromosomal examinations for the… CONTINUE READING