Original articleSeizure outcome of infantile spasms with focal cortical dysplasia☆
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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2022, SeizureCitation Excerpt :Owing to incomplete myelination in early life, around the time of onset of West syndrome, abnormalities in gray and white matter may not be clearly demonstrated on MRI [38]. The proportion of MRI-negative patients with epileptic spasms within published surgical cohorts varies between 20% [39] and 28.7% [10]. Younger age at surgery (mean, 15 months; range, 1.8–60 months) led to a higher proportion (41.3%) of MRI-negative cases in this series.
Epilepsy surgery for pediatric patients with mild malformation of cortical development
2020, SeizureCitation Excerpt :For example, the establishment of an epileptogenic network at an early stage of brain maturation may result in more widespread susceptibility to seizures [21,22]. The presence of mMCD poses challenges to surgery because the signs of focal abnormality are not immediately evident in MRI, particularly in very young age groups [23–25]. We experienced difficulties in accurately diagnosing lesions due to a high rate of negative MRI lesions and subtle pattern of MRI findings frequently observed at the gray-white matter junction, which is affected by myelination status.
The ketogenic diet for infants: How long can you go?
2020, Epilepsy ResearchCitation Excerpt :Table 3 displays specific data on infants below 12 months of age. Most of these studies were cohort studies and case series (Nordli et al., 2001; Kossoff et al., 2002, 2004; Rubenstein et al., 2005; Dressler et al., 2015, 2019a; Kim et al., 2019; Hong et al., 2010; Eun et al., 2006; Kang et al., 2007; Cole et al., 2010; Numis et al., 2011; Pires et al., 2013; Lee et al., 2013; Kang et al., 2013; Kayyali et al., 2014; Hirano et al., 2015; Fenton et al., 2015; Le Pichon et al., 2019; Dressler et al., 2019b) and have shown substantial effectiveness of the KD, although most studies also included children beyond the first year of life. The KD achieved seizure reduction > 50 % in 48%–80% of patients and seizure freedom in 14%–54% (Table 1).
Epileptic spasms as the presenting seizure type in a patient with a new “O” of TORCH, congenital Zika virus infection
2019, Epilepsy and Behavior Case ReportsCitation Excerpt :ES, or infantile spasms, result from several symptomatic causes, including developmental malformations and intrauterine insults, with imaging of these cases sufficient to identify more than half of symptomatic causes. Malformations of cortical development (MCDs) contribute to 8% of ES cases, and they have lower rates of response to treatment [7,8]. Congenital infections such as the TORCH etiologies are implicated in the development of ES.
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Part of this work has been presented on the International Symposium on Surgery for Catastrophic Epilepsy in Infants (ISCE), the Fourteenth Annual Meeting of ISS, Tokyo, February 18–19, 2012.