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Transcallosal resection of hypothalamic hamartoma for gelastic epilepsy.

Andrew M, Parr JR, Stacey R, Rosenfeld JV, Hart Y, Pretorius P, Nijhawan S, Zaiwalla Z, McShane MA

Department of Paediatric Neurology, Oxford Radcliffe Hospitals NHS Trust, Oxford, UK.

INTRODUCTION: Hypothalamic hamartomas (HHs) are commonly associated with severe epilepsy resistant to anticonvulsant therapy. Historically, surgical resection of HHs resulted in considerable morbidity. DISCUSSION: Two series of patients who successfully underwent resection using a transcallosal approach have now been published; we report the first UK experience of this technique in a series of five patients with HHs and gelastic epilepsy resistant to anticonvulsant therapy. Patients were assessed pre- and postoperatively for seizure activity, endocrine function, ophthalmology, and neurocognitive function. Two patients had precocious puberty and all had evidence of developmental delay and behavioral problems. Postoperatively, all children experienced at least a 50% reduction in seizure frequency with abolition of major seizure types; one child remains seizure-free. One child developed a mild postoperative right hemiparesis and one developed transient diabetes insipidus. CONCLUSION: There were no adverse developmental effects of surgery. Transcallosal resection of HHs ameliorates resistant epilepsy syndromes associated with HH.

Published 24 December 2007 in Childs Nerv Syst, 24(2): 275-9.
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