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Epilepsia, 49(Suppl.

1):40–44, 2008
doi: 10.1111/j.1528-1167.2008.01449.x

SUPPLEMENT - MANAGEMENT OF A FIRST SEIZURE

Special considerations for a first seizure in childhood


and adolescence
Peter Camfield and Carol Camfield

Department of Pediatrics, Dalhousie University and the IWK Health Centre, Halifax, Nova Scotia, Canada

SUMMARY rent literature allows at least partial answers to


Children with a first unprovoked seizure almost al- parents’ six most common questions: Will it hap-
ways present with a convulsive seizure. The differ- pen again? How long do I have to wait for a re-
ential diagnosis includes many paroxysmal events, currence? Could my child die during a recurrence?
especially convulsive syncope but even with a good Could there be brain damage with a recurrence? If
history; there is often uncertainty that cannot be medication treatment is delayed will there be any
eliminated by investigations. In general, an EEG long-term change in the chance of a permanent re-
and MRI are indicated with other investigations mission? Now that my child has had a seizure, how
determined on a case-by-case basis. Epilepsy syn- should his/her activities be restricted?
drome identification may be very valuable. Cur- KEY WORDS: First seizure, Child, Adolescent.

This paper focuses on children who present to the health within a 24-h period. Investigators from Albert Einstein in
care system with a first, unprovoked seizure and no previ- New York have argued that these two events should be con-
ous seizures. Almost all of these “first” seizures are dra- sidered as a single seizure (Shinnar et al., 2000). In their
matic convulsive attacks—children who experience only experience with 407 children with a first seizure, the re-
simple partial, complex partial, absence, myoclonus, and currence risk was the same after one unprovoked seizure
other short seizure types usually come to medical atten- as after 2 provoked seizures within 24 h, as judged by a
tion only after multiple attacks. By convulsion we mean 10-year follow-up. By contrast, we studied 490 children
a seizure with generalized or focal clonic activity. Based with epilepsy characterized by generalized tonic–clonic
on the Nova Scotia population-based childhood epilepsy or partial seizures with an average follow-up of 7 years
study, we estimate that fewer than 50% of children who (Camfield & Camfield, 2000). Seventy children had their
will go on to develop epilepsy will present with their first first two unprovoked seizures on the “same day” with com-
seizure (Camfield et al., 1985). Of 127 children present- plete recovery between the seizures, while 420 had their
ing with a “first” seizure to an expert pediatric clinic in first two seizures on “different” days. The recurrence risk
Calgary, Alberta, of the 94 who were diagnosed as hav- (80% and 81%), long-term remission (59% and 56%), and
ing an “epilepsy” seizure, nearly one-quarter were discov- risk of intractable epilepsy (7% and 8%) were so similar
ered to have a history of previous less dramatic seizures that we argued that when two first unprovoked seizures oc-
(Hamiwka et al., 2007 in press). These events were either cur on the same day with recovery between the seizures,
unrecognized by the family, unclear to the treating physi- then these two seizures should mandate the diagnosis of
cian or the family did not seek medical attention. epilepsy. The clinical course will be identical to children
with the first two seizures on separate days. The differences
D EFINITION between the New York and Nova Scotia data have not been
explained.
A controversial issue in pediatric epilepsy is how to cat-
egorize one versus two unprovoked first seizures occurring
D IFFERENTIAL D IAGNOSIS
Address correspondence to Dr. Peter Camfield, M.D., F.R.C.P.(C),
IWK Health Centre, 5850 University Ave, PO Box 9700, Halifax, Nova The diagnosis of a first seizure is usually straightforward
Scotia B3K 6R8, Canada. E-mail: camfield@dal.ca but the differential diagnosis in children includes a num-
Blackwell Publishing, Inc. ber of special pediatric disorders—most prominent are pal-

C International League Against Epilepsy lid syncope and breath holding (Stephenson, 1990). As in

40
41
Special Considerations: Childhood and Adolescence

adulthood, a frequent first seizure mimic is syncope with a We prefer to accept the ambiguity and suggest that not ev-
reflex anoxic seizure. This is particularly common in ado- eryone with a defined epilepsy syndrome has or will have
lescent girls. two or more seizures. It is possible to have an epilepsy syn-
The history does not always allow a confident diagnosis. drome but not have epilepsy (King et al., 1998; Pohlmann-
The Dutch group submitted 207 case summaries prepared Eden et al., 2006).
by a neurologist of first seizures to a panel of three child There may be a tremendous advantage for many chil-
epilepsy experts (van Donsellar et al., 1989). All patients dren with a first seizure to have an epilepsy syndrome
were then followed for a year. Of 156 considered to have diagnosis. Normal children with a first nocturnal secon-
a definite seizure, 46% had a recurrence, which confirmed darily generalized seizure often turn out to have benign
the seizure diagnosis. Of the 54 with a “disputable” first rolandic epilepsy, a diagnosis that can be made by a typi-
attack, 14 recurred and the recurrences were convincingly cal seizure history, normal neurological and developmental
seizures in 5. The interictal EEG did not assist in the “dis- examination and centrotemporal spikes on EEG with spe-
putable” group. This group of investigators has advocated cial morphology plus an anterior-posterior dipole (Wirrell
a “watch and wait” approach when the diagnosis is ques- et al., 2005). When this epilepsy syndrome is diagnosed
tionable. We endorse this approach. after a first seizure, it is possible to counsel parents that
recurrences are likely to be during sleep and that the long-
range prognosis is virtually always favorable. This disorder
I NVESTIGATIONS vanishes in adolescence and AED treatment is often not
A practice parameter of the American Academy of needed.
Neurology has examined the value of investigations for Likewise, a normal child presenting with a noctur-
children with a first afebrile, unprovoked seizure (Hirtz nal prolonged focal seizure without convulsive features
et al., 2000). This review was based on the available peer- but with eye deviation and vomiting can be confidently
reviewed literature. The only investigation that reached the diagnosed as having Panayotopoulos syndrome, if the
status of a recommendation was an EEG, a test that is of EEG shows occipital spike waves (Panayotopoulus, 1999).
value for syndrome identification and prediction of recur- Again, the prognosis is excellent, even if there are recurrent
rence. The risk of recurrence is increased by interictal spike seizures.
discharge, especially focal spikes. Routine blood work has We are somewhat less sure of the value of a diag-
no proven benefit. nosis of primary generalized epilepsy, when a normal
Since publication of the practice parameter, several stud- child presents with a first generalized tonic-clonic seizure
ies have suggested that the yield on MRI for causative ab- and generalized spike-wave on EEG. If the EEG shows
normalities in children with a first seizure is about 20–30% photosensitivity and the seizure seemed to be provoked
(Sharma et al., 2003; Shinnar et al., 2001). Abnormalities by flashing lights then a syndrome diagnosis of idiopathic
that require intervention are considerably fewer, approxi- generalized epilepsy (IGE) with photosensitivity has con-
mately 1%. Therefore, one can make a reasonable case for siderable value in avoiding possible seizure triggers.
MRI, since occasionally there may be a treatable cause de- The diagnosis of the syndromes of cryptogenic partial
tected or the etiology may have prognostic value or point epilepsy or symptomatic partial epilepsy are of less use af-
to other health concerns. ter a first unprovoked seizure, except to focus the clinician
For selected patients it seems obvious that some blood on a specific area of the MRI scan. Although there are fre-
tests are indicated—for example, a serum glucose in a child quent MRI abnormalities in such children, most do not lead
with diabetes and a seizure. to direct intervention. It is still unclear how imaging find-
ings impact on the recurrence risk, although it is tempting
to assume that an MRI lesion increases the risk of recur-
S YNDROME I DENTIFICATION rence.
When a child presents with a first seizure it is often
possible to diagnose an epilepsy syndrome, a concept that Q UESTIONS P OSED BY PARENTS
leads to considerable confusion. Epilepsy is usually de-
AFTER A F IRST S EIZURE
fined as two or more unprovoked seizures. After a first un-
provoked seizure in children, the recurrence risk is about Following a child’s first seizure parents (and often the
50%; however, after 2 unprovoked seizures, the recurrence child) usually have six major questions—Will it happen
rate is roughly 80% (Camfield et al., 1985; Berg et al., again? How long do I have to wait for a recurrence? Could
1991). So, how can a child with only one seizure be said my child die during a recurrence? Could there be brain
to have an “epilepsy” syndrome? A recent suggestion has damage with a recurrence? If I choose to delay medication
been to change the definition of “epilepsy” to a single treatment will there be any long-term change in the chance
seizure plus a tendency for more seizures (Fisher et al., of a permanent remission? Now that my child has had a
2005). This definition is very difficult to use in practice. seizure, how should his/her activities be restricted?

Epilepsia, 49(Suppl. 1):40–44, 2008


doi: 10.1111/j.1528-1167.2008.01449.x
42
P. Camfield and C. Camfield

1. Will it happen again? 4. Could there be brain damage with a recurrence?


The recurrence risk after a first unprovoked seizure in There are little data about brain damage in studies of
childhood is essentially the same as in adulthood and fac- first seizures and recurrences; however, a larger literature
tors that increase the likelihood of recurrence are very sim- addresses this issue for children with established epilepsy.
ilar (Camfield et al., 1985; Berg & Shinnar, 1991). Risk It is clear that children with epilepsy have high rates of
is increased by focal versus generalized seizures, by pres- cognitive deficits before the first seizure ever occurs. The
ence of spike discharge on EEG and by the presence of critical question is whether they show worsening deficits
concomitant neurological deficits. Children with none of with additional seizures. Austin compared 98 children
these factors have approximately a 20% chance of recur- with epilepsy to 96 children with asthma over a 4-year
rence and children with all of these factors have about an period (Austin et al., 2000). As expected, the children
80% risk of recurrence (Camfield et al., 1985). While these with epilepsy had less satisfactory performance on school
differences are statistically robust, it is not possible to an- achievement tests at each point in the study compared with
swer this question with a definitive “yes” or “no.” In any those with asthma but there was no suggestion of deterio-
case, parents should understand appropriate first aid and ration over time, even in those with uncontrolled epilepsy.
have a plan in place for medical attention should there be a Bourgeois and coworkers noted no consistent decline
recurrence. in IQ in 72 children with newly diagnosed epilepsy
(Bourgeois et al., 1983). Psychology testing within 2 weeks
of diagnosis and then yearly for 4 years showed no change
2. How long might it be before we can be sure that
or improvement in most children. Eleven percent showed
our child will not have a recurrence?
some declined in IQ, but the decline was not related to
Again, the data for children are similar to adults. In the
seizure number but rather to drug intoxication.
Nova Scotia series of 168 children with a first seizure, 70%
The National Collaborative Perinatal Project (NCPP) as-
of recurrences were within 6 months of the first seizure,
certained 55,000 children prenatally and followed them to
77% by 1 year, and 90% by 2 years (Camfield et al., 1985).
7 years of age. There were 98 sibling pairs where one sib-
In the series of 407 children from the Bronx with a first
ling had one or more afebrile seizures and the other did
unprovoked seizure, 53% of recurrences were by 6 months
not (Ellenberg & Nelson, 1978). It is unclear how many of
and 88% by 2 years (Shinnar et al., 2000). The answer to
these children only had a single seizure. Intelligence and
this question is that recurrences may be delayed for 2 years
academic testing at 7 years of age showed no difference be-
or more, but most recurrences are within 6 months of the
tween the siblings with seizures and those without. Further-
first seizure.
more, 62 children had not experienced any seizure(s) by
age 4 years when they underwent cognitive testing. Then
3. Could my child die during a recurrence? between age 4 and 7 years, these same 62 children had
Perceptions of parents witnessing a first unprovoked one or more afebrile seizures. Comparisons of the cogni-
seizure have not been directly reported, although it is likely tive testing at age 4 with testing at age 7 showed no change.
that they are similar to the reactions of parents witnessing The answer to this question is that a robust literature
a first febrile seizure. During a first febrile seizure almost shows that clinically significant brain injury does not re-
all parents indicate that they thought their child was dying sult from a few recurrent unprovoked seizures.
(Rutter & Metcalfe, 1978). After a first afebrile seizure the
fear of death during a seizure may be further increased by 5. If medication treatment is delayed will there be
information about SUDEP. The risk of death during a first any long-term change in the chance of a permanent
recurrent seizure has not been reported; however, children remission?
with established epilepsy have a tiny risk of SUDEP. Three Most authorities (Hirtz et al., 2003) recommend no
large studies have addressed this issue. In Nova Scotia, 692 AED treatment after a first seizure in children, in part
children with epilepsy were followed for about 20 years because about half of these children will never have an-
(Camfield et al., 2002a). There were 26 deaths, 1 from sta- other seizure and in part because there seem to be few
tus and only 1 from SUDEP when she was 22 years of consequences from a second seizure. Few studies directly
age. In the Dutch study of childhood epilepsy, 472 chil- address the question of benefit from AED treatment af-
dren with epilepsy were followed for 5 years with 9 deaths ter a first seizure. In the Nova Scotia study of chil-
(Callenbach et al., 2001). None of the deaths were related dren with established epilepsy, children were prescribed
to seizures and there were no cases of SUDEP. In the Con- AED treatment after a variable number of seizures (Cam-
necticut study, 613 children were followed for a median of field et al., 1996). This was not a randomized study—
7.8 years (Berg et al., 2004). Of the 13 deaths, two were re- the decision to start AEDs depended on the number of
lated to seizures—one from status and one SUDEP. There- seizures before diagnosis and a clinical decision on the
fore, this question can be confidently answered—the risk part of the family and physician. There were between
of a child dying during a recurrent seizure is miniscule. 20 and 71 children in each group who were treated

Epilepsia, 49(Suppl. 1):40–44, 2008


doi: 10.1111/j.1528-1167.2008.01449.x
43

Special Considerations: Childhood and Adolescence

after 1, 2, and up to 10 seizures. Eighty-nine started (Unglaub et al., 2005), have an adult aware of when they
treatment only after >20 seizures. For a pretreatment num- are in the shower, and be sure not to lock the bathroom
ber of seizures <10, the long-term remission rates were door. For athletic activities, we do not recommend any
identical. For children with ≥10 seizures before AED treat- changes. Helmet use for bicycling makes sense for all chil-
ment, there was a statistically decreased chance of long- dren and adults. Swimming should not only be in a super-
term remission; however, children with ≥10 pretreatment vised area but the child should swim with a responsible
seizures were much more likely to have complex partial buddy. We suggest that the child continue to sleep in his/her
seizures—a seizure type that we reasoned was intrinsically own bed. For those who are found to be photosensitive on
less likely to remit. We concluded that waiting for up to 10 an initial EEG, we recommend moving back as far as possi-
seizures before starting AED treatment was not hazardous ble when using a video display terminal (VDT) and having
from the perspective on long-term remission. There cer- ambient lighting. Tree climbing and other high climbing
tainly was no penalty for waiting for a second seizure be- activity might be restricted for 3 months. We usually do
fore starting AED treatment. not recommend disclosing the first seizure to school au-
The two main randomized studies of treatment after a thorities because of the risk of stigma and possible further
first seizure are outlined elsewhere in this monograph. Nei- restrictions from normal school activities.
ther the MESS study (Marsden et al., 2005) nor the FIRST
study (Musicco et al., 1997) included large numbers of C ONCLUSIONS
children but both studies indicated that early AED treat-
ment reduced early recurrences but over several years the First seizures are disruptive events for a child and fam-
remission rates were identical in the early and late treat- ily. A careful workup including EEG and MRI is usually
ment groups. warranted. Restrictions on activities should be few and the
We carried out a modest randomized trial of no AED treatment strategy of deferral of daily AED treatment until
treatment (n = 14) versus daily carbamazepine (n = 14) af- a recurrence is appropriate and safe.
ter a first afebrile seizure (Camfield et al., 1989). Over the
Disclosure of Conflicts of Interest: The authors have declared no
next year, statistically fewer children in the carbamazepine
conflicts of interest.
arm had afebrile recurrences but when the children were
followed up about 20 years later, there was no difference
in the long-term remission rate between the two groups R EFERENCES
(Camfield et al., 2002b).
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Epilepsia, 49(Suppl. 1):40–44, 2008


doi: 10.1111/j.1528-1167.2008.01449.x

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