Disconnection surgery to cure or palliate medically intractable epileptic spasms: a retrospective study

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  • 1 Departments of Neurosurgery and
  • | 2 Pediatrics, Osaka City University Graduate School of Medicine; and
  • | 3 Departments of Pediatric Neurosurgery and
  • | 4 Pediatric Neurology, Osaka City General Hospital, Osaka, Japan
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OBJECTIVE

Surgery is a treatment option for medically intractable epileptic spasms (ESs). However, outcomes of ES after surgery are not well understood, especially when surgeries aimed at seizure palliation are included. The purpose of the present study was to 1) investigate the proportion of favorable postoperative ES outcomes, 2) explore the preoperative factors related to favorable postoperative ES outcomes, and 3) examine the timing of ES recurrence after disconnection surgeries, including both curative and palliative indications.

METHODS

This retrospective study included patients who underwent disconnection surgery for medically intractable ES at the authors’ institution between May 2015 and April 2021. Patients with suggested focal-onset ES based on preoperative evaluations initially underwent lobar disconnection. Patients with suggested generalized or unknown-onset ES underwent corpus callosotomy (CC). If evaluations after initial CC showed focalized or lateralized change, they were considered secondarily revealed focal-onset ES, and lobar disconnection was performed. ES outcomes were evaluated using the International League Against Epilepsy classification. ES outcomes were divided into classes 1–4 as favorable outcomes and classes 5 and 6 as unfavorable outcomes. The relationship between the favorable postoperative ES outcomes and the following preoperative factors was analyzed: sex, age at onset (< or > 1 year), duration between seizure onset and initial surgery (< or > 2 years), type of seizure at onset (ES or others), presence of other types of seizures, substrate, hypsarrhythmia, and MRI abnormalities. The period between the last surgery and ES recurrence was also analyzed.

RESULTS

A total of 41 patients were included, of whom 75.6% achieved favorable ES outcomes. A longer seizure duration between seizure onset and initial surgery, presence of hypsarrhythmia, and positive MRI findings led to poorer postoperative ES outcomes (p = 0.0028, p = 0.0041, and p = 0.0241, respectively). A total of 60.9% of patients had ES recurrence during the follow-up period, and their ES recurred within 13 months after the last surgery.

CONCLUSIONS

Disconnection surgery is an effective treatment option for medically intractable ES, even when the preoperative evaluation suggests a generalized or unknown onset.

ABBREVIATIONS

CC = corpus callosotomy; EEG = electroencephalography; ES = epileptic spasm; FCD = focal cortical dysplasia; FD = frontal disconnection; ILAE = International League Against Epilepsy; PQD = posterior quadrant disconnection; VNS = vagus nerve stimulation; WS = West syndrome.

Supplementary Materials

    • Supplemental Table 1 (PDF 406 KB)

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