A follow-up study in children with status epilepticus during sleep: From clinical spectrum to outcome

Abstract

To evaluate the correlation between clinical spectrum and therapeutic outcomes and neuropsychological deficits in children with status epilepticus during sleep (SES). Methods: The clinical spectrum of patients with SES was defined as follows: status epilepticus of benign childhood epilepsy with centro-temporal spikes (SEBECTs), atypical benign focal epilepsy during childhood (ABFEC), non-idiopathic focal epilepsy (NIFE), and Landau-Kleffner syndrome (LKS). SES cases were divided into 4 groups according to neuropsychological findings before treatment: developmental delay/ intellectual disability (DD/ID), cognitive impairment (CI), attention deficit and/or hyperactivity behaviors (AHD), and normal group (NG). The therapeutic outcomes were classified into 3 groups: satisfactory response, recurrence, and seizure control. Results: A total of 39 cases (24 males and 15 females) were recruited, including 3 cases with SEBECTs, 26 with ABFEC, 8 with NIFE [2 with focal cortical dysplasia (FCD)], and 2 with LKS. There were 7 patients in the DD/ID group, 8 in the CI group, 19 in the AHD group, and 5 in the NG group. Neuropsychological outcomes were significantly different among clinical spectrum (P < 0.001), and neuropsychological deficits frequently occurred in the ABFEC group or in the NIFE group. Besides, 18 patients in the satisfactory group had satisfactory response to medicine or surgery (2 out of 18 cases with FCD), whereas recurrence was observed at least one session within one year in 16 cases in the recurrence group, and no improvement in spike-wave index and cognition/behavior was noted in 5 patients in the seizure control group, although seizure could be controlled. There were significant differences in therapeutic outcomes among clinical spectrum (P = 0.041), with the worst outcomes in the NIFE group (only 1 out of 8 with satisfactory good response). Conclusions: It is important to categorize patients with SES into epilepsy syndromes, including SEBECTs, ABFPEC, NIFE, and LKS; the clinical spectrum may be a significant determinant to influence the outcomes of SES, including neuropsychological deficits and therapeutic outcomes

Continuous Spike–Waves during Slow Sleep Today: An Update

“…Seizures are present in the majority of LKS cases (around two-thirds of patients) and are semiologically heterogeneous: focal motor, tonicclonic seizures, and atypical absences (e.g., with chewing gestures or with lip smacking). Behavioral symptoms are frequently associated: hyperactivity; an attention deficit; impulsiveness (leading up to an ADHD picture); irritability; aggressive behavior; in some cases, autistic-like symptoms; anxiety; and depression [27,29,93,160,[162][163][164][165][166][167][168]. In LKS, autism could be due to the epilepsy itself, but the presence of genetic factors common to LKS and autism should not be overlooked [28].…”

Risk factors of electrical status epilepticus during sleep in children with benign childhood epilepsy with centro-temporal spikes

“…In the past, it was thought that the prognosis of BECT was favorable, which, however, was not the case according to a growing body of research. 1 There is a significantly increased discharge during non-rapid eye movement (NREM) sleep in some children, with a discharge index of > 85%, which is called electrical status epilepticus during sleep (ESES). 2 - 3 ESES is generally characterized by diverse forms of seizures and poor response to drugs.…”