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1):40–44, 2008
doi: 10.1111/j.1528-1167.2008.01449.x
Department of Pediatrics, Dalhousie University and the IWK Health Centre, Halifax, Nova Scotia, Canada
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?
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
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