IS is a difficult-to-treat infantile epileptic encephalopathy by medications. Frequent seizures of IS, which can be up to hundreds of clinical and subclinical seizures per day, cause devastating effects on the brain development and cognitive levels of children, and more than 90% of patients have mental or motor developmental retardation. Some research pointed out that pediatric epilepsy surgery in infants and toddlers (< 3 years) offers significant opportunities for improving seizure frequency, neuro-cognitive development, and quality of life [5]. There are seven preadmission predictors for surgical candidacy: single semiology at seizure onset, structural etiology, one or more interictal foci in the same hemisphere, focal background electroencephalograph slowing, focal or hemispheric abnormality on magnetic resonance imaging, male sex, and normal development. In the presence of more than four factors, 91% were found to be surgical candidates [6]. Therefore, early surgery is recommended for those who are suitable for surgery in order to avoid intellectual and cognitive deterioration [7, 8]. The surgical treatment of IS mainly includes two methods: resection and palliative surgery. The resection surgery is preferred for patients with definite etiology, focal seizures and an origin located in the non-functional areas [4]. The palliative surgery, which mainly cuts the corpus callosum, can be used for those who have no clear etiology or manifesting widely disseminated epileptiform discharges; neuromodulation techniques such as vagus nerve stimulation (VNS) and deep brain stimulation can also be considered [8, 9]. Delphine et al. [10] reported three such patients who presented with IS between 5 and 7 months of age; two of the patients who underwent early hemispherotomy acquired normal verbal intelligence, whereas the third patient, who was the oldest and had the longest duration of epilepsy, was operated on at 38 months of age and remained with severe mental retardation. This indicated that early diagnosis and a shorter lag time to treatment are essential for the overall outcome of WS patients [1]. At present, the earliest age for surgical treatment for IS reported in the international literature is 2.5 months [11]. The earliest age for surgical treatment for IS in China is 10 months. A history of infantile spasms has been shown to be associated with a median of 3.0 years’ shorter duration to surgery [12].
Kuwahara et al. [13] reported a 4-month-old female infant with atypical asymmetrical GH diagnosed with WS who underwent relatively low-dose ACTH therapy followed by a combination of valproic acid and clonazepam, and the seizures were well controlled. To date, the surgical treatment of GH-caused IS is rarely reported, and there is no consensus on how and when to treat GH combined with IS [14]. Here, we present a GH patient who manifested medically refractory epilepsy, including spasms and focal seizures, accompanied by severe developmental retardation. The patient underwent lesion resection at the age of 5 months, and showed seizure and behavioral improvement after the operation. As far as we know, this is the earliest report of neurosurgical intervention in GH-induced IS, and the patient is one of the few reported cases of lesionectomy with resultant improved behavior.
FCD is the most frequent histological finding in pediatric patients undergoing epilepsy surgery and is classified into different clinicopathological subtypes: type I, type II and type III [15]. FCD is a common structural cause of IS. Seizures from FCD are commonly refractory to medical treatment, and FCD accounts for up to 26% of pediatric epilepsy cases with surgical intervention [16]. Borggraefe et al. [17] have reported a patient who manifested FCD IIb-caused seizures within the first day of life, and received epilepsy surgery at the age of two and a half months with seizure-free outcome, suggesting that epilepsy surgery can be successfully performed in medically intractable patients with a clearly identifiable seizure onset zone within the first three months of life. This is the youngest age ever reported for surgical treatment of FCD-caused neonatal drug-resistant epilepsy. In addition, the report also indicated that surgical management with a shorter latency period tended to have better developmental outcomes, as demonstrated in animal models [18], in which a short duration of seizures has less influence on the near normal brain. To date, studies exploring the cognitive effects of epilepsy surgery for FCD are limited. In 2010, Rouleet-Perez et al. [19] studied 11 children who underwent epilepsy surgery before age 6. Two of the children with FCD IIb showed a rapid improvement in developmental quotient testing following surgery. Over a long-term period, only one child with FCD type IIb showed continued developmental improvement; the remaining patients were stable in their testing and did not show significant catch-up long after surgery. In this study, patient 2 was diagnosed with FCD IIa combined with IS and had a poor response to AEDs. However, after receiving neurosurgical treatment at the age of 4 months, the patient showed good surgical tolerability, rapid control of convulsions, and gradual reduction of AEDs during follow up. In addition, her mental and motor development gradually returned to normal after rehabilitation.
