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Sturge-Weber-Dimitri Disease: Role of Hemispherectomy in Prognosis

Published online by Cambridge University Press:  18 September 2015

A. Oluwole Ogunmekan ,
Paul A. Hwang  and
Harold J. Hoffman
A. Oluwole Ogunmekan*
Affiliation:
Division of Neurology, The Hospital for Sick Children, Toronto
Paul A. Hwang
Affiliation:
Division of Neurology, The Hospital for Sick Children, Toronto
Harold J. Hoffman
Affiliation:
Division of Neurosurgery, The Hospital for Sick Children, Toronto
*
Department of Paediatrics, Lagos University Teaching Hospital, P.M.B. 12,003 Lagos, Nigeria
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Abstract:

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We report 12 patients with Sturge-Weber-Dimitri disease treated surgically between January 1975 and December 1987. Hemispherectomy was performed on ten, two others underwent occipital lobectomy for intractable seizures. All operations were performed between the ages of 3 months and 20 months, except in two at age 8 and 9 years. The onset of seizures in all was between 2 and 8 months of age, except for two at 15 months. There were no postoperative deaths. Postoperative shunt procedures were required in 3 out of 12 (25%). Postoperative seizure control for one year or more was achieved in 11 out of 12 patients. The remaining patient is on medication with no seizures, but follow-up is less than a year. Intellectual deterioration was not seen after surgery except in 2 patients who had late operations. We conclude that patients with Sturge-Weber-Dimitri syndrome who have intractable seizures in the first 6 months of life and unilateral hemispheric involvement should be considered for early resection of the involved hemisphere.

Type
Original Articles
Information
Canadian Journal of Neurological Sciences , Volume 16 , Issue 1 , February 1989 , pp. 78 - 80
Copyright
Copyright © Canadian Neurological Sciences Federation 1989

References

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