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Many childhood disorders have paroxysmal features. It is therefore not
uncommon that these episodes are interpreted as seizures. In this section,
common disorders that may mimic epilepsy are emphasized.
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Breath-holding spells (BHS) occur most often between the ages
of 6 months and 6 years, with a prevalence rate of 4% to
27%1. There may be a familial predisposition
to BHS in 20% to 35% of cases.2 The
predisposing setting is frustration, anger, fear, or reaction to pain,
which leads to crying or a tantrum. The child then holds his or
her breath in expiration, often resulting in pallor, cyanosis, or
a mixture of both.3 When the spell resolves without loss
of consciousness, it is called simple. The child
may appear dazed and ultimately lose consciousness and may even
have a few convulsive movements or tonic stiffening. Convulsive
activity has been seen in up to 55% of children with BHS.
The entire episode lasts less than a few minutes, and the child
is not impaired for a prolonged period after the attack. Lambroso
and Lerman classified the spells in 1960 as pallid, cyanotic, and mixed, in
an attempt to separate them into different pathophysiological mechanisms. The
etiology is unclear but data suggest excessive centrally mediated
sympathetic reflex activity in cyanotic breath-holders, and excessive
centrally mediated parasympathetic reflex activity in pallid breath-holders. EEGs
during the event fail to show any electrographic seizures; diffuse
background slowing of the EEG in the theta-delta range accompanied
with slowing of the heart rate is often seen. Conditions besides
seizures that can mimic BHS include central apnea due to an Arnold-Chiari
malformation, gastroesophageal reflux (GER), prolonged QT syndrome
with syncope, brain stem lesions, rage, panic attacks, and Munchausen-en-proxy.
Treatment is directed at reassuring the parents of the benign nature
of BHS and its ultimate natural remission. Oral iron therapy at
5 to 6 mg/kg/day of elemental iron has been found
to be beneficial in some cases.4 The exact mechanism
of iron in improving the spells is unclear, but it may act via its
effect on serotonin, its degrading enzyme aldehyde oxidase, sympathomimetic neurotransmitter
norepinephrine, or its degrading enzyme monoamine oxidase. Use of
atropine or scopolamine in refractory pallid breath-holding spells,
tetrabenazine or clonidine in refractory cyanotic breath-holding
spells, and cardiac pacing in refractory spells accompanied with
prolonged asystole have been tried with some success.5 Medications
that have been tried on an experimental basis include theophylline,
piracetam, naltrexone, acetazolamide, and madroxyprogesterone, but
none have definite clinical utility. Educating the parent about
intervention during the event including placing in the lateral position
to prevent aspiration and applying a gentle thrust on the back to
reinitiate respiration is important. Issues regarding the disruptive
effects on the parents and extended family and difficulty in obtaining
child care and baby-sitting need to be addressed. Seventeen percent
go on to develop syncope by teenage years.
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Gastroesophageal reflux (GER) signifies the retrograde movement of
gastric contents ...