Keywords
BECTS - SeLECTS - CSWS - ESES - benign epilepsy with centrotemporal spikes
We present the case of an 8-year-old male, presented with complaints of infrequent
episodes (three witnessed episodes over 1 year) of nocturnal awakening and behavioral
arrest with sustained deviation of head and eyes to the right side with subtle focal
clonic jerks of the right upper and lower limbs, with onset at 6 years of age. He
was born nonconsanguineously, with no abnormal antenatal, perinatal, or developmental
history with prior good academic performance. Since the onset of the seizures he had
difficulty in academic performance with reduced attention and memory in school. Intelligence
quotient and neuropsychological assessment was incomplete due to poor cooperation
of the child. Magnetic resonance imaging was normal and awake electroencephalogram
(EEG) had a normal background and frequent bisynchronous biphasic centro-parieto-temporal
large amplitude spikes with maximum negativity over the left temporal region ([Figs. 1] and [2]). His sleep record showed continuous runs of large amplitude generalized spikes
with maximum negativity over the centrotemporal region and spike-wave index (SWI) > 90
([Fig. 3]). He was treated with valproate, brivaracetam, and clobazam currently. Oxcarbazepine
previously administered was discontinued in view of rashes. In view of the scholastic
regression despite antiseizure medications, a course of intravenous immunoglobulin
(IVIG) was provided. Our patient reported a significant subjective increase in scholastic
performance with improvement in attention and memory after the administration of IVIG;
however, there was no discernible change in the EEG SWI done after 1, 3, or 6 months.
Fig. 1 Awake electroencephalogram (EEG) record showing normal background and bisynchronous
centro-parieto-temporal (CPT) large amplitude spikes with maximum negativity over
the temporal region (with an amplitude dominance over the left hemisphere).
Fig. 2 Activation of abundant bisynchronous biphasic centro-parieto-temporal (CPT) large
amplitude spikes with maximum negativity over the left temporal region highlighting
frontal positivity and tangential dipole in average montage.
Fig. 3 Activation of large amplitude continuous spike and waves with spike-wave index > 90
during slow wave sleep—electrical status epilepticus during sleep (ESES).
SeLECTS (self-limited epilepsy with centrotemporal spikes), formerly known as benign
epilepsy with centrotemporal spikes (BECTS)benign rolandic epilepsy, typically onsets
between 4 and 10 years of age (peak 7 years) and usually remits by mid to late adolescence
and has a male preponderance.[1] EEG is characterized by normal background activity and activation of high-amplitude
biphasic and triphasic centrotemporal spike and slow wave complexes with a transverse
dipole (frontal positivity, temporoparietal negativity) during drowsiness and sleep,
which may be unilateral, bilateral, or independent. The presence of continuous spike-wave
activation (SWI > 85% is required for diagnosis of electrical status epilepticus during
sleep [ESES]) during sleep warrants evaluation for progressive language or cognitive
impairment. The spectrum of BECTS, atypical benign focal epilepsy of childhood or
pseudo-Lennox syndrome, status epilepticus of benign epilepsy with centrotemporal
spikes, acquired epileptic aphasia or Landau–Kleffner syndrome, and epileptic encephalopathy
with continuous spike-and-wave during sleep was differentiated based on the degree
of language, neuropsychological impairment/regression, and the degree of ESES.[2] Recently retermed, epileptic encephalopathy with spike-wave activation in sleep
(EE-SWAS) is made when a developmentally normal child has neuropsychological regression
after seizure onset.[1] Developmental encephalopathy with spikes-wave activation in sleep (DE-SWAS) occurs
in one with preexisting developmental delay or just language delay. There is a clear
correlation with ESES and individuals with neuropsychological deficits when the SWI > 85%.[3] Seizures tend to be resistant to multiple antiseizure medications, and they usually
disappear before adolescence and deficits recede with the remission of ESES. Genetic
etiologies of heterozygous pathogenic variants in GRIN2A or fragile X syndrome have
been reported.[1]
[2]
