Elsevier

Epilepsy Research

Volume 78, Issues 2–3, February 2008, Pages 240-243
Epilepsy Research

Short communication
Ictal SPECT in Sturge-Weber syndrome

https://doi.org/10.1016/j.eplepsyres.2007.12.006Get rights and content

Summary

We report on a patient with right-sided Sturge-Weber syndrome (SWS), in whom earlier functional hemispherectomy failed. Subtraction of ictal and interictal single-photon-emission-computed-tomography (SPECT) superimposed on individual MRI showed a right fronto-orbital hyperperfusion, with a left-sided EEG seizure pattern. Ictal SPECT supported our assumption that right frontal originated seizure pattern propagated to left hemisphere via the remaining right frontal bridge. Right orbito-frontal resection and disconnection from corpus callosum resulted in seizure freedom.

Section snippets

Case report

A 6-year-old boy had frequent daily bilateral asymmetric tonic seizures since he was 8 months old. He had been diagnosed to have right-sided SWS. He had flaccid hemiparesis on left side more than on the right, and was not able to stand or walk. He was mentally retarded and unable to speak. As he was resistant to multiple anti-epileptic drugs (AED), epilepsy surgery was performed in 2003. Temporal and central lobectomy and frontal and occipital hemispherotomy were performed on the right side. He

Discussion

Our patient demonstrates that even if previous epilepsy surgery, for example, combined hemispherectomy and hemispherotomy has failed in SWS patients, they may benefit from repeated surgery if the seizure onset zone can be localized. Epilepsy surgery should be considered in these patients if high seizure frequency and developmental deterioration do not respond to AED treatment. Anatomical hemispherectomy has an excellent outcome but was formerly reported to be associated with chronic

Conflicts of interest statement

The authors report no conflicts of interest.

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