Hypothalamic hamartomas are rare congenital gray matter heterotopias associated with a triad of gelastic seizures, precocious puberty, and developmental delay. Gelastic seizures typically have a childhood onset and are drug-resistant, with scalp electroencephalogram (EEG) having limited utility to identify abnormalities. On neuroimaging with brain MRI, the hamartomas are usually nonenhancing, are nearly isointense relative to the adjacent gray matter on all sequences, and are not associated with abnormal restricted diffusion. Drug-resistant hypothalamic hamartoma-related epilepsy is best treated with surgery, which should be done as early as possible. Open microsurgical resection has largely been replaced by radiosurgery as well as both stereotactic radiofrequency thermocoagulation and MRI-guided laser interstitial thermotherapy (LITT), which allow a rate of seizure control of approximately 80-90%, while keeping the rate of complications at 0-2%.
CITATION STYLE
Grewal, S. S., Garcia, D. M., Middlebrooks, E. H., & Wharen, R. E. (2020). Minimally invasive surgery. In Epilepsy Case Studies: Pearls for Patient Care: Second Edition (pp. 247–251). Springer International Publishing. https://doi.org/10.1007/978-3-030-59078-9_44
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