Case 1:
The
A healthy 9-year-old girl is brought to the
emergency room after her parents witnessed
her having an unusual event shortly after
going to bed. They reported hearing
gurgling noises coming from her room.
When they found her, she was sitting up in
bed and unable to speak. She had jerking of
her right lower face with excessive drooling.
She appeared awake during the spell. It
stopped spontaneously after 2 minutes. She
has had a cold but otherwise has been well
recently.
Physical Examination
General Examination: Normal. Neurologic
Examination: Mental Status: Alert and
cooperative. Language: Fluent without
dysarthria. Cranial Nerves: II through XII
intact. Motor: She has normal bulk and tone
with 5/5 strength throughout. Coordination:
There is no dysmetria on finger-to-nose
testing. Sensory: No deficits are noted. Gait:
She has a normal heel, toe, flat, and tandem
gait. Reflexes: 2+ throughout with bilateral
plantar flexor responses.
Guide to Case Discussion
 Briefly summarize this case.
 Localize the examination findings.
 Give the most likely diagnosis and
provide a differential diagnosis.
 Discuss an appropriate diagnostic
work-up.
 Discuss the management of this
patient.
Diagnosis
Benign childhood epilepsy with
centrotemporal spikes.
Discussion
Benign childhood epilepsy with
centrotemporal spikes (BECTS) or benign
rolandic epilepsy is the most common form
of idiopathic epilepsy in childhood.
peak ages of onset are between 5 and 10
years. However, onset may occur in children
as young as 3 years or as old as 13 years.
Affected children are typically
developmentally and intellectually normal.
There is a strong genetic predisposition to
epilepsy in BECTS. A gene on chromosome
15q14 has been implicated in some families.
The seizures in BECTS are brief and
infrequent. Approximately 10% of children
will experience only one seizure. In the
majority of children (70%), seizures will
occur only two to six times. Twenty percent
of patients will experience more frequent
seizures. In approximately 70% of children,
seizures occur only at night. The seizures in
BECTS have a characteristic semiology,
involving hemifacial clonic movements,
speech arrest, dysarthria, and excessive
drooling. Preceding paresthesias around the
mouth, gums, cheek, or lips may occur.
During the seizure, there may be
involvement of the ipsilateral arm or leg, as
well as secondary generalization. The
seizures usually last 1 to 2 minutes.
The EEG background in BECTS is normal
in the awake and sleep states. Epileptiform
paroxysms are described as focal, diphasic
spike-and-slow-wave activity of medium to
high voltage located over the rolandic or
centrotemporal regions. Epileptiform
discharges may occur either unilaterally or
independently and bilaterally. A horizontal
dipole with maximum spike negativity over
the central and temporal regions with
positivity in the frontal regions is also a
classic EEG finding. Characteristically in
this disorder, spike-and-wave activity
increases in frequency and amplitude during
drowsiness and nonrapid eye movement
(REM) sleep. Centrotemporal spikes may
occur only during sleep in approximately
30% of children.
Spontaneous resolution of seizures by
adulthood (18 years of age) occurs in almost
all patients with BECTS and, thus, it is
given the designation of a “benign”
epilepsy. Some academic and behavioral
problems have been reported in children
with BECTS, but the overall prognosis for
intellectual and neurologic outcome is
excellent.
Summary
The patient is a healthy 9-year-old girl who
presents after a simple partial seizure
involving right facial jerking, drooling, and
speech arrest. Her neurologic exam is
normal.
Localization
Right facial twitching as well as speech
arrest localize to the left centrotemporal,
rolandic region.
Differential Diagnosis
The patient in this case presents with the
classic features of a focal seizure in the
setting of BECTS, but the diagnosis of
BECTS can be made only when both the
clinical history and the EEG findings are
consistent with this syndrome. Other
localization-related epilepsies caused by
underlying structural lesions (trauma,
cerebrovascular disease, tumor, neuronal
migration abnormalities, mesial temporal
sclerosis) should also be considered.
Evaluation
For a patient who presents to the emergency
room with a new-onset focal seizure, a head
CT scan, as well as basic laboratory work
(basic chemistries, CBC, toxicology screen),
may be warranted. A referral (inpatient or
outpatient) for an awake and sleep EEG is
indicated to evaluate for focal epileptiform
activity. The diagnosis of BECTS may be
assigned only when the clinical history as
well as the EEG are consistent.
The role of neuroimaging in a patient with
BECTS is controversial. If the clinical
history is consistent and classic bilateral,
independent centrotemporal spike-and-wave
activity is found on the EEG, a brain MRI is
not always performed. However, if there are
atypical clinical features or the EEG shows
unilateral or atypical epileptiform activity, a
brain MRI is typically recommended to rule
out structural abnormalities.
Management
Treatment with anticonvulsants is often
unnecessary in BECTS as seizures are
typically infrequent and occur primarily in
sleep. However, when seizures are more
frequent or occur during wakefulness, they
may be disruptive to a child's quality of life.
A variety of anticonvulsants have been used
in the treatment of BECTS and most are
efficacious. However, carbamazepine,
oxcarbazepine, and gabapentin have
tolerable side effects and are most
commonly used in clinical practice.
Additional Questions
 How would your management be
different if this patient had been ill
for several days with a high fever?
 Can children with BECTS have other
EEG abnormalities?