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Neuropediatrics 2000; 31(4): 206-210
DOI: 10.1055/s-2000-7455
Short Communication

Georg Thieme Verlag Stuttgart · New York

Syntelencephaly Presenting with Spastic Diplegia

L. T. Coleman1 , J. P. McCubbin2 , L. J. Smith3 , D. S. Reddihough4 , M. R. J. Gardner5
  • 1 Department of Radiology, Royal Children's Hospital, Melbourne, Australia
  • 2 Department of Paediatrics, Gippsland Hospital, Sale, Australia
  • 3 Department of Neurology, Royal Children's Hospital, Melbourne, Australia
  • 4 Department of Child Development & Rehabilitation, Royal Children's Hospital, Melbourne, Australia
  • 5 Victorian Clinical Genetics Services, Royal Children's Hospital, Melbourne, Australia
Further Information

Publication History

Publication Date:
31 December 2000 (online)

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Two children with syntelencephaly presented with the predominant clinical picture of spastic diplegia. Brain scanning showed deficient formation of the interhemispheric fissure with fusion of occipital and parietal lobes, and an infolding and protrusion of occipital cortex into the telencephalic ventricle. The fusion extended further posteriorly than the cases of “middle interhemispheric fusion” thus far reported. One 7-year old has poor language development, while the other is regarded, at her present age of 3 years, as being cognitively within the normal range.

Key words

Brain malformation - Spastic diplegia - Syntelencephaly - Semilobar holoprosencephaly

References

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Dr. M. R. J. Gardner

Victorian Clinical Genetics Services The Murdoch Institute Royal Children's Hospital

Flemington Road

Parkville Melbourne, Vic. 3052

Australia

Email: E-mail: gardner@cryptic.rch.unimelb.edu.au