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Surgical treatment of delayed epilepsy in hemiconvulsion-hemiplegia-epilepsy syndrome

From the Departments of Neurology (D.W.K., K.C., S.K.L.) and Neurosurgery (C.-K.C.), College of Medicine, Seoul National University Hospital; and Department of Neurology (K.-K.K.), College of Medicine, Dongguk University Hospital, Korea.

Address correspondence and reprint requests to Dr. Sang Kun Lee, Department of Neurology, Seoul National University Hospital, 28, Yongkeun dong, Chongno Ku, Seoul, 110-744, Korea sangunlee{at}dreamwiz.com

Objective: Hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome is an uncommon consequence of prolonged febrile convulsive seizures in infancy and early childhood. Delayed epilepsy in HHE syndrome is frequently intractable to medical treatment. The present study evaluated the role and prognosis of surgical treatment in patients with delayed epilepsy of HHE syndrome.

Methods: We included 26 consecutive patients who were diagnosed with HHE syndrome and underwent surgical treatment for delayed epilepsy at Seoul National University Hospital. The multidisciplinary presurgical evaluations included brain MRI, video-EEG monitoring, FDG-PET, and ictal SPECT. Anterior temporal lobectomy (ATL), cortical resection, functional hemispherectomy, and callosotomy were performed according to the results of presurgical evaluations.

Results: Patients were grouped into either the temporal lobe epilepsy (TLE) group (n = 12) or the neocortical/multifocal epilepsy group (n = 14) according to the results of presurgical evaluations. Patients were included in the TLE group if there was strong evidence that the mesial temporal lobe was the only ictal-onset area. The other patients were included in the neocortical/multifocal group. There were no significant differences in demographic characteristics between the two groups. All patients in the TLE group became seizure-free after ATL, but only four patients became seizure-free, and additional two patients showed improvement after various surgical procedures in the neocortical/multifocal group (p = 0.002).

Conclusion: Surgical intervention may be helpful in patients with delayed epilepsy in hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome, especially if the mesial temporal lobe appears to be the only epileptogenic area, regardless of the presence of additional abnormalities seen with neuroimaging. Therefore, surgical treatment should be considered for selected patients with delayed epilepsy in HHE syndrome.

Abbreviations: ATL = anterior temporal lobectomy; FCDs = focal cortical dysplasias; HHE = hemiconvulsion-hemiplegia-epilepsy; MTLE = mesial temporal lobe epilepsy; TLE = temporal lobe epilepsy.

Supplemental data at www.neurology.org

Editorial, page 2097

e-Pub ahead of print on January 30, 2008, at www.neurology.org.

Supported by the Ministry of Health and Welfare (A060452), Republic of Korea.

Disclosure: The authors report no conflicts of interest.

Received June 4, 2007. Accepted in final form August 30, 2007.

This article has been cited by other articles:

				K. D. Holland and J. Buchhalter Hemiconvulsion-hemiplegia-epilepsy syndrome: Another case for epilepsy surgery Neurology, May 27, 2008; 70(22_Part_2): 2097 - 2098. [Full Text] [PDF]