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Generalized epilepsy in hypothalamic hamartoma

Evolution and postoperative resolution

From the Children’s Epilepsy Program (Drs. Freeman, Harvey, Rosenfeld, and Berkovic, and J.A. Wrennall and C.A. Bailey), Departments of Neurology (Drs. Freeman and Harvey, and C.A. Bailey) and Psychology (J.A. Wrennall), and Murdoch Childrens Research Institute (Dr. Freeman), Royal Children’s Hospital, Parkville; Departments of Paediatrics (Drs. Freeman and Harvey) and Medicine (Dr. Berkovic), University of Melbourne; Departments of Neurosurgery and Surgery (Dr. Rosenfeld), The Alfred Hospital and Monash University, Prahran; and Epilepsy Research Institute (Drs. Freeman, Harvey, and Berkovic), Austin and Repatriation Medical Centre, Heidelberg West, Victoria, Australia.

Address correspondence and reprint requests to Dr. Jeremy L. Freeman, Department of Neurology, Royal Children’s Hospital, Flemington Road, Parkville VIC 3052, Australia; e-mail: jeremy.freeman{at}rch.org.au

Objective: To better understand the epileptogenesis of symptomatic generalized epilepsy in patients with hypothalamic hamartoma and intractable epilepsy, many of whom experience remission of generalized seizures and slow spike-wave discharges following surgery.

Methods: The authors documented the evolution of symptomatic generalized epilepsy in 12 of 20 children who underwent transcallosal microsurgical hypothalamic hamartoma resection. In seven patients they recorded intraoperative EEG from the hamartoma and simultaneously from the scalp and frontal cortex before, during, and after resection.

Results: Gelastic seizures began on average at 6 months of age (range birth to 3 years); tonic seizures began at 6 years (range 2 months to 9 years). Normal EEG were reported in early childhood; thereafter, abnormalities were progressive. Interictal spike-wave was recorded intraoperatively over the scalp and cortex in six patients, but not from the hypothalamic hamartoma. Hamartoma resection had no immediate effect on cortical spike-wave, but waking spike-wave was absent in seven patients on subsequent postoperative EEG. Tonic seizures ceased in 11 of 12 patients, but 6 of these had postoperative generalized seizures that resolved over 1 to 6 months.

Conclusion: Gelastic seizures in hypothalamic hamartoma arise from the hamartoma itself; the interictal spike-wave does not. The evolution of EEG abnormalities, the development of generalized seizures years after onset of gelastic seizures, and the postoperative running down of interictal spike-wave and generalized seizures in these patients may reflect secondary epileptogenesis.

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