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0883073808315341v1
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First published on March 19, 2008, doi:10.1177/0883073808315341

Journal of Child Neurology 2008;23:954.

A more recent version of this article appeared on August 1, 2008

Article

Intracranial Hypertension in 2 Children With Marfan Syndrome

Yvonne Hilhorst-Hofstee, MD1*, Lucia J. M. Kroft, MD, PhD2, Gerard Pals, PhD3, Jeroen P. P. van Vugt, MD, PhD4, and Wouterina C. G. Overweg-Plandsoen, MD, PhD5

1 Center for Human and Clinical Genetics, Leiden University Medical Center, Leiden, the Netherlands
2 Department of Radiology, Leiden University Medical Center, Leiden, the Netherlands
3 Department of Molecular Genetics, VU University Medical Center, Amsterdam, The Netherlands
4 Department of Neurology, Medisch Spectrum Twente, Enschede, The Netherlands
5 Department of Neurology, Leiden University Medical Center, Leiden, the Netherlands

* To whom correspondence should be addressed. E-mail: hilhorst{at}lumc.nl.


  Abstract
Two unrelated children with Marfan syndrome presented with recurrent intracranial hypertension. Both children complained of headache, nausea, and vomiting and one of them had papilledema. Both had increased cerebrospinal fluid pressure, and their complaints disappeared after lumbar puncture. Although severe headache has been reported in Marfan syndrome due to intracranial hypotension, this is to our knowledge the first report of intracranial hypertension in Marfan patients.


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