Elsevier

Brain and Development

Volume 35, Issue 8, September 2013, Pages 816-820
Brain and Development

Original article
Seizure outcome of infantile spasms with focal cortical dysplasia

https://doi.org/10.1016/j.braindev.2013.06.013Get rights and content

Abstract

Purpose: This study sought to evaluate the seizure outcome of infantile spasms (IS) with focal cortical dysplasia (FCD). Methods: We retrospectively reviewed infantile spasms patients with FCD from 2004 to 2010. We investigated seizure outcome from antiepileptic drug (AED), ketogenic diet (KD), resective surgery, and analyzed the results according to individual imaging studies. Results: Among 404 patients of IS, FCD was confirmed in 51 patients. In retrospective review of brain MRI, only 21 patients (41.2%) were suspected of FCD before 1 year of age, but 45 patients (88.2%) became confirmed to FCD by MRI after the age of 1 year. Once the spasms were not controlled by 1 or 2 AEDs, the chance of becoming seizure free with additional third or more drugs was very low (2.3%). The seizure free rate was 33.3% (7/21) in patients treated with ketogenic diet, and 73.3% (22/30) in surgical patients, who were both intractable to AEDs. There were no significant differences in seizure free rate in both ketogenic diet and surgical patients, between MRI negative and positive patients prior to 1 year of age. Conclusions: KD and surgery should be considered in medically refractory IS with FCD.

Introduction

Infantile spasms (IS) is a form of epileptic encephalopathy characterized with spasms, hypsarrhythmia and neurodevelopmental regression [1], [2].

Focal cortical dysplasia (FCD) is one major cause of infantile spasms [3], [4], [5]. FCD can produce generalized epilepsy, such as IS through cortico–subcortical interaction [6]. Because myelination is not well developed yet in infancy, FCD is not well detected by MRI in this age [7], [8].

Ketogenic diet (KD) is proven to be effective in IS [9], as well as resective surgery in IS with FCD [10], [11].

We reviewed MRI findings of IS patients with FCD in serial follow-up and seizure outcomes from various types of treatment, including KD and surgery, and compared the outcomes between MRI positive and negative groups at the time of diagnosis in infancy.

Section snippets

Patients

The study was approved by the institutional review boards at Severance Children’s Hospital. Patient consent was not required in this retrospective clinical study. We retrospectively reviewed medical records of patients with infantile spasms who were managed in Severance Children’s Hospital from January 2004 to December 2010. Among the total of 404 patients with infantile spasms, we selected patients who were eventually confirmed with the diagnosis of FCD through brain MRI and/or pathological

Methods

We looked into individual patient history, laboratory findings, imaging studies, seizure outcomes of antiepileptic drugs (AED), KD, and surgery, and developmental outcomes. Seizure outcome was measured from caregiver’s reports and seizure diary at each scheduled visit.

First-line treatment was AED, and commonly used AEDs were vigabatrin (96.2%), topiramate (62.3%), clobazam (56.6%), valproic acids (50.9%), and oral steroids (28.3%) with 2–3 mg/kg of predisolone. When failed to achieve complete

Patient’s profile (Table 1)

Among the 404 IS patients, we were able to detect FCD in 51 patients confirmed by MRI and/or pathological findings of resective brain cortex. The average onset age of spasms was 5.6 ± 4.2 months (range from 0 to 20 months). The mean follow-up period was 54.3 ± 23.1 months (range from 13 to 103 months), and the age at last follow-up was 66.9 ± 27.3 months (range from 16 to 129 months). The distribution of FCD was multilobar in 26 cases (51.0%), and singe lobar in 20 cases (39.2%) with 13 cases of frontal, 4

Discussion

Overall 72.5% of IS patients with FCD were able to achieve seizure freedom with AED, KD and epilepsy surgery in this study. Once seizure remission was not achieved by second AED, KD or epilepsy surgery should be considered in IS with FCD. Seizure remission was obtained in 7 cases (13.7%) with 1st or 2nd AED trial, but only an additional 1 patient (2.3%) was benefited with such results with additional AED to the first 2 medications. Additional remission was obtained with either KD, which

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