doi:10.1016/j.braindev.2006.06.009
Copyright © 2006 Elsevier B.V. All rights reserved.
Pediatric intractable epilepsy syndromes: Reason for early surgical intervention
Shiyong Liua, 1, Ning Ana, 1, Hui Yanga,
,
, Meihua Yanga, Zhi Houa, Lihong Liub and Yong Liuc
aDepartment of Neurosurgery, Xinqiao Hospital, The Third Military Medical University, Chongqing, 400037, PR China
bDepartment of Pediatrics, Xinqiao Hospital, The Third Military Medical University, Chongqing, 400037, PR China
cDepartment of Neurology, Xinqiao Hospital, The Third Military Medical University, Chongqing, 400037, PR China
Received 5 December 2005;
revised 28 June 2006;
accepted 30 June 2006.
Available online 22 August 2006.
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Abstract
Drug-resistance in several childhood epilepsy syndromes is common, and these patients may tolerate epilepsy surgery. In this study, the surgical outcomes of 24 pediatric patients with various intractable epilepsy syndromes and three patients with tuberous sclerosis were examined at Xinqiao hospital between 1997 and 2004. The study included nine cases of Lennox–Gastaut syndrome, two cases of Rasmussen‘s syndrome, one case of Sturge–Weber syndrome, three cases of West syndrome, three cases of tuberous sclerosis and nine cases of mesial temporal lobe epilepsy syndrome. In each case, different surgical procedures were performed according to preoperative evaluation and ECoG. At an average of 4.5 years after surgery, 14 out of 27 patients (51.9%) had an Engel Class I outcome after surgery, and an additional eight patients (29.6%) had rare seizure (Engel ClassII). Three patients showed a significant decrease in seizure frequency (Engel Class III). The mean IQ increased from 61.4 ± 12.2 to 75.0 ± 11.0, and greater IQ increase was seen in patients with shorter seizure history and drug-resistance. Temporary complications were observed in four patients and there were no deaths. In conclusion, early surgical intervention in pediatric intractable epilepsy syndromes may results in a favorable outcome in a high percentage of cases and may provides an important opportunity to prevent irreversible decline in intelligence and disability.
Keywords: Intractable epilepsy; Epilepsy syndromes; Epilepsy surgery; Multilobe resection; Pediatric patients
Fig. 1. (A) EEG for a patient with West syndrome demonstrating hypsarrhythmia. (B) EEG for a patient with Lennox–Gastaut syndrome showing fast paroxysmal rhythms during sleep. (C) and (D) EEG for a patient with Lennox–Gastaut syndrome pre- and postoperatively. (C) Demonstrating the slow spike-and-wave in the right temporal region before surgery, and (D) showing a normal EEG one and a half year after surgery.
Fig. 2. (A–D) MRI of a patient with Rasmussen syndrome showing hemisphere atrophy. (E) EEG for a preoperative patient with Rasmussen syndrome.
Fig. 3. (A–D) MRI of a patient with Sturge–Weber syndrome demonstrating marked enhancement and leptomeningeal angiomatosis over areas of the right hemisphere. (E) Facial port wine stain of the patient. (F) Leptomeningeal angiomatosis over areas of the right hemisphere in operation.
Fig. 4. Pre- and postoperative IQ scores in each patient.
Table 1.
The profile of each patients

Abbreviations: CPS, complex partial seizure; EEG, electroencephalogram; HS, hippocampal sclerosis; LGS, Lennox–Gastaut syndrome; lt., Left; MRI, magnetic resonance imaging; MTLE, Mesial temporal lobe epilepsy; RS, Rasussen’s syndrome; rt., Right; SPS, simple partial seizure; SWS, Sturge–Weber syndrome; sz., seizure; TSS, tuberous sclerosis syndrome; WS, West syndrome.
Table 2.
The itemized statement of each epilepsy syndrome

Table 3.
Pre- and postoperative intellectual assessments of 23 cases categorized by the drug-resistance period
a Compare with pre-operation, paired-samples
t-test,
P < 0.05.
b There was a significant difference between

2 years group and >4 years group, independent-samples
t-test,
P < 0.05.
Table 4.
Pre- and postoperative intellectual assessments of 23 cases categorized by the lag between onset and surgery of epilepsy
a Compare with pre-operation, paired-samples
t test,
P < 0.05.
b There was a significant difference when compared with

3 years group, independent-samples
t test,
P < 0.05.