Surgical options for patients with Lennox‐Gastaut syndrome

Abstract

SUMMARYDespite ongoing investigation into pharmacologic treatments for Lennox-Gastaut syndrome (LGS), outcomes for chronic administration of medications remain disappointing. In many instances LGS is treatment refractory, resulting in poor prognoses that include intellectual disability, persisting seizures, and psychiatric conditions. For patients with treatment resistance to other modalities for LGS, a further option is surgical intervention. Evaluation for surgery should involve interictal electroencephalogram (EEG), magnetic resonance imaging (MRI) analysis, and age-appropriate neuropsychological/developmental assessment. Resective surgery, where seizure foci are removed, successfully controls seizures in many cases, particularly where lesionectomy or lobar resections are appropriate. Recent studies of resective surgery on individuals with LGS show promising results, with a high percentage of patients having improved seizure control. Corpus callosotomy is a palliative surgical approach that aims at controlling potentially injurious seizures, for example, atonic or drop seizures, by preventing the bilateral spread of epileptic activity. Once associated with a high risk for morbidity and mortality, microsurgical techniques and surgery limited to the anterior region of the callosum have greatly diminished complications of corpus callosotomy surgery. Vagus nerve stimulation, another palliative procedure, offers rates of seizure improvement similar to those of corpus callosotomy, with the exception of atonic seizure for which corpus callosotomy may lead to a greater reduction. Recent advances in surgical techniques offer encouraging options for treatment of LGS.

Lennox–Gastaut Syndrome: A State of the Art Review

“…Two main different groups of diagnostic investigations should be performed before the surgery: tests for the localization of epileptogenic focus for resection (single-photon emission computed tomography, positron emission tomography, intracranial EEG, and magnetoencephalogram) and tests for the definition of eloquent cortex to minimize the risk of neurological deficits related to surgery (electrocorticography and fMRI or Wada test if fMRI fails to show a clear lateralization). [61][62][63][64][65] Most of the abovementioned diagnostic tools are available in a few specialized centers. 61 For this reason, early referral to specialized pediatric neurosurgical centers is recommended if a clear surgical indication is defined.…”

Treatment-resistant Lennox-Gastaut syndrome: therapeutic trends, challenges and future directions

“…LGS has historically been described as an incurable epileptic encephalopathy due to the often diffuse or generalized EEG findings and intractable seizures despite aggressive medical management 56. However, numerous advances in neuroimaging and neurophysiology have enabled better identification and treatment of epileptogenic zones 57. Resective surgery may benefit those with focal lesions, such as dysplasias, hamartomas or infarcts.…”

Erster epileptischer Anfall und Epilepsien im Erwachsenenalter

“…Die kognitiven Folgen einer Kallosotomie können auch bei Patienten mit niedrigem IQ lebensbeeinträchtigend sein. Das Verfahren sollte daher ganz am Ende der Therapiekette stehen [186]. Bei Lennox-Gastaut-Patienten gehören dazu auch der Einsatz von hochdosiertem CLB und die Vagusnervstimulation.…”