- A 6-year-old boy presents for evaluation due to an episode of screaming and confusion at night.
The boy’s parents heard him scream in his room, and when they went to him, he exhibited rapid
twitching of his left arm and hand, stiffening of his left leg, rolled back eyes, and some blinking of
both eyes. He was incoherent and minimally responsive for 5 to 10 minutes. After the episode,
he was weak on the left side of his body. By morning, he had returned to a normal status.
Of the following, the MOST likely diagnosis is
A. benign rolandic epilepsy
B. juvenile myoclonic epilepsy
C. night terrors
D. nocturnal frontal lobe epilepsy
E. rapid eye movement sleep behavior disorder - The first unprovoked seizure described for the boy in the vignette occurred out of sleep and
was clearly partial, with vocalization, left-sided motor movements, and transient focal weakness
afterward. He is otherwise completely healthy and normal. It is possible he has had other
nocturnal seizures that did not awaken his parents. The most common diagnosis for such
findings in childhood is benign rolandic epilepsy, a childhood-onset epilepsy that typically occurs
between the ages of 3 and 13 years and resolves before adulthood. Seizures may be
infrequent, and the child usually has no other problems. Inheritance is autosomal dominant.
Interictal electroencephalography showing characteristic centrotemporal spikes is confirmatory.
Treatment generally is not needed because the nocturnal seizures are infrequent and do not
cause any problems the following day. Partial seizure medications such as carbamazepine are
effective, if used. - Juvenile myoclonic epilepsy has a later onset, typically in teenage years, and is
characterized by one or more of the following: 1) myoclonic jerks, usually in the morning; 2)
generalized tonic-clonic seizures, often in the morning, and 3) absence seizures. Unlike benign
rolandic epilepsy, juvenile myoclonic epilepsy is a form of generalized epilepsy.
Nocturnal frontal lobe epilepsy has a variable and unusual nocturnal presentation that
involves complex, stereotyped dystonic movements and sometimes vocalizations that can lead
to confusion with parasomnias. The associated seizures typically last fewer than 2 minutes and
may cluster, occurring many times per night, at any time of the night. The affected child may
have some partial recall of the events. - Night terrors are a non-rapid eye movement (REM) sleep parasomnia that can be confused
with nocturnal seizures. Night terrors can start as early as 18 months of age, earlier than benign
rolandic epilepsy, peak at age 5 to 7 years, and usually resolve by adolescence. The
phenomenology involves a sudden arousal, vocalization, and confusion, with autonomic
changes such as mydriasis and tachycardia. The movements are not repetitive clonic
movements or twitching, like a seizure. The child is unconscious during the episode and does
not recall it the next day. Events usually occur, at most, twice per night and during deep slow
wave sleep, in the first half of the night, 1 to 2 hours after falling asleep. - REM sleep behavior disorder is an uncommon parasomnia in children. Paralysis normally
occurs during REM sleep. In REM sleep behavior disorder, there is a partial or full loss of this
paralysis. As a result, the individual may act out dreams that may be vivid, intense, or violent.
This disorder tends to occur in adults who have neurodegenerative diseases and is associated
with the use of psychiatric medications or alcohol withdrawal. However, cases have been
described in children, including those who have autism
Dr. Jayaprakash
Superintendent, ICH.
Institute of Child Health.
Gov. Medical College Kottayam.
Kerala, India.