Neuropsychol Rev (2007) 17:427–444
DOI 10.1007/s11065-007-9048-4
Neuropsychological Deficits in Childhood
Epilepsy Syndromes
William S. MacAllister & Sarah G. Schaffer
Received: 1 August 2007 / Accepted: 4 October 2007 / Published online: 26 October 2007
# Springer Science + Business Media, LLC 2007
Abstract Seizure disorders are relatively common in
childhood, and the International League Against Epilepsy
(ILAE) provides a hierarchical classification system to
define seizure types. At the final level of classification,
specific epilepsy syndromes are defined that represent a
complex of signs and symptoms unique to an epilepsy
condition. The present review discusses the issues related to
several of these epilepsy syndromes in childhood, including
those classified as generalized idiopathic epilepsies (e.g.,
childhood absence epilepsy, juvenile absence epilepsy,
juvenile myoclonic epilepsy), focal epilepsies (benign
rolandic epilepsy, occipital epilepsy, temporal lobe epilepsy,
frontal lobe epilepsy) and the “epileptic encephalopathies,”
including Dravet’s Syndrome, West Syndrome, Lennox–
Gastaut Syndrome, Myoclonic Astatic Epilepsy, and
Landau–Kleffner Syndrome. For each syndrome, the
epidemiology, clinical manifestations, treatments, and neu-
ropsychological findings are discussed.
Keywords Epilepsy . Children . Syndromes .
Neuropsychological
Introduction
Estimates suggest that in the United States, approximately
5% of children will experience a seizure prior to the age of
20 (Hauser et al. 1996). About 25% of those who
experience a single convulsive episode will go on to meet
W. S. MacAllister (*) : S. G. Schaffer
New York University Comprehensive Epilepsy Center,
403 East 34th Street, 4th floor,
New York, NY 10016, USA
e-mail: William.macallister@med.nyu.edu
formal criteria for epilepsy, which requires recurrent
unprovoked seizures. Accordingly, a large amount of effort
and research has gone into understanding their etiology,
associated features, appropriate intervention, and manage-
ment. Children with epilepsy are known to have a range of
cognitive deficits. An understanding of these deficits is
essential to helping each child maximize their academic
potential, as research has shown that school achievement in
children with epilepsy is often lower than would be
predicted based on global measures of cognitive function
(Farwell et al. 1985).
Once a diagnosis of epilepsy is established in a child,
syndrome classification is considered such that the best
clinical management plan can be established. The Interna-
tional League Against Epilepsy (ILAE) classification
involves a hierarchical system with three tiers. At the first
level, the seizure type is described as generalized, localiza-
tion related, or undetermined. The second level classifies
seizures according to whether they are idiopathic, symp-
tomatic, or cryptogenic. It should be noted that it has been
proposed that the term cryptogenic be replaced with the
term “probably symptomatic,” to indicate that there is a
presumed underlying structural brain abnormality that has
not yet been identified. At the final tier of classification, a
specific syndrome is assigned. Specific epilepsy syndromes
represent a complex of signs and symptoms that define a
unique epilepsy condition. Historically, accurate syndrome
classification has been challenging, and prior research
documents some degree of inter-rater disagreement (Berg
et al. 1999, 2000). In reviewing the available literature on
the neuropsychological deficits in children with epilepsy
syndromes, it is clear that terms are not used consistently
across studies. As a result, it is not always known if
different studies on a given syndrome are evaluating
exactly the same types of children. Future studies should
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seek to use strict classification guidelines and standard
terminology.
The aims of the present review are to draw together the
relevant research on pediatric seizure syndromes. In doing
so we will review the etiology of syndromes, the
prevalence, treatment considerations, and the neuropsycho-
logical findings. An exhaustive review of all childhood
syndromes would not be possible in the space available.
Our review, therefore, will focus on syndromes that are
commonly evaluated by pediatric neuropsychologists and
have a strong empirical literature on the cognitive findings
of such syndromes. Syndromes for which there is a paucity
of cognitive data available will not be discussed. Specifi-
cally, the present review will discuss the generalized
idiopathic epilepsies (e.g., childhood absence epilepsy,
juvenile absence epilepsy, juvenile myoclonic epilepsy),
focal epilepsies (benign rolandic epilepsy [BRE], occipital
epilepsy, temporal lobe epilepsy [TLE], frontal lobe epilepsy
[FLE]), and the “epileptic encephalopathies” (Dravet’s
syndrome, West syndrome, Lennox–Gastaut syndrome,
myoclonic astatic epilepsy, and Landau–Kleffner syndrome).
Generalized Idiopathic Epilepsies
The ILAE specifies three syndromes under the classifica-
tion of generalized idiopathic epilepsies. These include
childhood absence seizures, juvenile absence seizures, and
juvenile myoclonic epilepsy. (There are other idiopathic
generalized epilepsies that are less commonly seen, such as
perioral myoclonia with absences and eyelid myoclonia
with absences, but these syndromes have not yet been
included in the ILAE classification and will not be
addressed here due to limited information.) Absence
seizures are further subclassified as typical absence sei-
zures, atypical absence seizures, and absence status. Typical
absence seizures may be either simple, with impairment of
consciousness as the sole manifestation, or complex, where
one may see mild clonic manifestations, changes in tone,
and automatisms. An earlier study verified that automatisms
during absences might involve repetitive movements.
Moreover, memory for interactions during absences may
vary from patient to patient and from seizure to seizure;
some may be entirely anmestic for events that occur during
absences, whereas others may have some memory for these
events (Freemon et al. 1973). In complex typical absence
seizures, differentiation from complex partial seizures can
be difficult and requires EEG corroboration. Atypical
absences are often characterized by a less abrupt onset
and cessation, and a longer duration. Moreover, there are
often more obvious changes in tone associated with these
events (Commission on Classification and Terminology of
the International League Against Epilepsy 1981). Atypical
Neuropsychol Rev (2007) 17:427–444
absence seizures commonly begin prior to the age of 5 years
and are nearly always associated with other seizure types,
such as generalized tonic-clonic seizures, myoclonic, atonic,
and tonic seizures. Mental retardation is common.
Childhood Absence Epilepsy and Juvenile Absence
Epilepsy
The term pyknolepsy is often used to describe typical
absence seizures (both simple and complex) in children and
is synonymous with the syndrome of childhood absence
epilepsy. The term “petit mal” was previously used to
describe absence seizures in school age children, and
though this term is not used in the formal classification, it
is still used colloquially. Onset is generally between age
three and puberty, and girls are more frequently affected
than boys. The absences in these children occur quite
frequently (several times per day) and tend to occur in
clusters (Wirrell 2003). In contrast to childhood absence
epilepsy, juvenile absence epilepsy tends to develop later,
typically with puberty. The two conditions can be chal-
lenging to differentiate, and many epidemiological and
neuropsychological studies of absence epilepsies consider
them together. Thus, the information available that is
specific to this type is somewhat limited. In addition to
absence seizures, this syndrome is often associated with
generalized tonic-clonic seizures upon awakening in about
80% of cases (Loiseau et al. 1995). Moreover, myoclonic
seizures are present in about 15% of cases, which can also
make this syndrome difficult to distinguish from juvenile
myoclonic epilepsy (Reutens and Berkovic 1995).
Absence seizures account for between two and 11
percent of all seizure types across all age ranges, with the
highest rates seen in the first 10 years of life (Blume et al.
1973; Cavazzuti 1980; Dalby 1969; Heijbel et al. 1975;
Livingston et al. 1965). Juvenile absence epilepsy accounts
for approximately 11–20% of patients with absence
epilepsy, and males and females are equally affected
(Wirrell et al. 1996). In short, there are considerably fewer
numbers of patients with juvenile absence epilepsy than
with childhood absence epilepsy, though it is generally
considered to be a more serious disorder. The presence of
concomitant generalized tonic-clonic seizures is associated
with a poorer prognosis (Tovia et al. 2006). With respect to
the etiology, genetic factors are considered to be most
salient. For example, it has been demonstrated that absence
seizures and generalized spike and wave discharges are
both inherited traits (Metrakos and Metrakos 1961). Given
the fact that bilateral synchronous 3 Hz spike and wave
activity is a defining feature of absence seizures, deep
subcortical structures are implicated in the pathophysiolo-
gy; studies have implicated the thalamocortical circuitry in
absence seizures (Gloor and Fariello 1988).
Neuropsychol Rev (2007) 17:427–444
Valproic acid, ethosuximide, and clonazepam have all
proven effective in decreasing seizure frequency (Browne
et al. 1975; Browne 1976, 1978; Bruni et al. 1980; Lund
and Trolle 1973; Sherard et al. 1980). Generally speaking,
cognitive side effects are low with ethosuximide and
valproic acid and a recent study suggests that children with
absence epilepsy being treated with these medications
experience improved attention, visual memory, and fine
motor skills (Siren et al. 2007). Whereas many patients with
childhood absence epilepsy will eventually enter remission,
those with juvenile absence epilepsy will generally require
lifelong treatment. Some suggest remission rates in approx-
imately 80% of childhood absence epilepsy cases. Howev-
er, a number of patients, 44% in one study, with unremitting
childhood absence epilepsy will develop juvenile myoclon-
ic epilepsy (Wirrell et al. 1996).
Despite remission, however, the psychosocial outcome
in children with childhood absence epilepsy may be poor.
One study followed such children into adulthood and
compared them to a group of controls with a history of
juvenile rheumatoid arthritis. In comparison to the arthritis
group, those with a history of absence epilepsy were more
likely to drop out of high school, have unplanned
pregnancies, and abuse substances (Wirrell et al. 1997).
In 2001, a sample of children with childhood absence
epilepsy was studied neuropsychologically (Pavone et al.
2001). The sample included six boys and ten girls that
ranged in age from 6 to 16 years, with a median age of onset
of 5.3 years. These children were compared to a control
group matched for gender and had comparable age and
socioeconomic status (Pavone et al. 2001). To assess
cognitive function, patients and controls were administered
the Italian version of the Wechsler Intelligence Scale for
Children—Revised, as well as the Raven’s Progressive
Matrices, Rey Complex Figure, and the Test of Memory
and Learning. The results showed that 81% of the patient
group had IQ scores in the average range, with three
patients having scores in the borderline range. Overall,
however, patients had lower full scale IQ’s than did the
matched controls (Pavone et al. 2001). To assess more
specific neuropsychological domains, composite indices
were created by calculating norm-referenced scores for
each task, and percentile ranks were averaged for each
domain. Domains included general cognition, language,
visual spatial skills, and memory. Overall, the patients
showed a lower level of general cognition, visual spatial
skills, nonverbal memory, and delayed recall. The authors
concluded that childhood absence epilepsy patients show
slight, but statistically significant, deficits in global cogni-
tive function, visual spatial functions, and visual memory.
Verbal skills and verbal memory were less affected (Pavone
et al. 2001). The finding that general intellectual function-
ing is lowered in children with generalized idiopathic
429
epilepsy syndromes is consistent with the results of later
work (Nolan et al. 2003). However, the latter study
considered children with generalized idiopathic epilepsy
as a group and did not consider childhood absence epilepsy
specifically. Thus, direct comparisons between these studies
are difficult.
Likewise, Jambaque et al. (1993) studied a large group
of children with various seizure types. These included 18
patients with generalized idiopathic epilepsies, 12 of
whom had childhood absence epilepsy. As with the results
reported above, the group of children with generalized
idiopathic epilepsy performed poorly relative to controls
on tasks of visual memory, whereas verbal memory was
intact (Jambaque et al. 1993). Again, however, this study
did not consider children with childhood absence epilepsy
alone; the generalized idiopathic epilepsy group also
contained three children with juvenile absence epilepsy
and three children with generalized tonic-clonic seizures
on awakening.
Nolan et al. (2004) studied memory function in children
with mixed seizure syndromes, including childhood ab-
sence epilepsy, FLE, and TLE. According to the authors,
this was the first prospective study of memory functioning
in children with epilepsy where cases were strictly
classified according to the criteria of the ILAE (Nolan et
al. 2004). The mean age of onset for the group of 13 with
childhood absence epilepsy was 5.5 years, and they aver-
aged 9.5 years at assessment. To assess memory, the children
were administered five subtests from the Wide Range
Assessment of Memory and Learning (WRAML), as well
as the Rey Complex Figure. Though children with absence
epilepsy showed the least degree of memory impairment in
comparison to the other seizure types, they did show
impairment relative to normative data. Specific weaknesses
were seen on the finger windows subtest of the WRAML, a
test requiring sustained visual attention and working mem-
ory, as well as on the Rey Complex Figure, which suggests
fairly specific visual-spatial skill deficits consistent with
prior research.
Another investigation studied children with absence
epilepsy whose onset occurred prior to the age of 3 years
(Chaix et al. 2003). Ten cases were identified, including
seven girls and three boys: five cases presented with simple
absences, and five cases with complex absences (i.e., with
automatisms or eyelid myoclonia, or myoclonic jerks). The
authors concluded that the overall prognosis for children
with early onset absence epilepsy is relatively poor in
comparison to what is seen in childhood absence epilepsy
more generally. However, this investigation suffers from
limitations that make firm conclusions difficult. First and
foremost, the assessment was not standardized across
participants, with different measures used in different cases.
Moreover, two cases received no formal testing. Equally
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important is that global IQ measures were used, rather than
tests of specific neuropsychological functions; tests
employed may not have had sufficient sensitivity to detect
subtle deficits.
Juvenile Myclonic Epilepsy
Juvenile myoclonic epilepsy, which has also been termed
“impulsive petit mal,” generally begins between ages 12
and 18 and requires lifelong treatment. It is the most
common primary generalized epilepsy syndrome in adoles-
cence, accounting for between 4 and 10% of all epilepsies.
This syndrome is often associated with myoclonic jerks of
the neck, shoulders, and arms (Janz 1985). Most also
experience generalized tonic-clonic seizures, and as many
as 40% experience absences (Asconape and Penry 1984).
Seizures are often brought on by sleep deprivation, stress,
alcohol, or illicit drug use. Photosensitivity is common in
this syndrome as well, as it occurs in about 30% of patients
(Wolf and Goosses 1986). The characteristic EEG findings
in this syndrome include high amplitude generalized
symmetrical and synchronous 4–6 Hz polyspike-wave com-
plexes. There is a genetic contribution to this syndrome and
it has been mapped to chromosome 6p21.3 (Delgado-
Escueta et al. 1989).
Despite the ubiquity of juvenile myoclonic epilepsy, the
diagnosis is often missed. Several studies documented
unusually long period between initial seizures and correct
diagnosis, ranging from 8.3 to 15 years across studies
(Grunewald et al. 1992; Panayiotopoulos et al. 1991;
Vazquez et al. 1993). Delayed diagnosis has been attributed
to factors including a patient’s failure to report myoclonic
jerks to their physicians, or a physician’s failure to
specifically inquire about myoclonic jerks.
As with other generalized idiopathic epilepsies of
childhood, valproic acid, ethosuximide, and clonazepam
are all used to treat juvenile myoclonic epilepsy (Browne et
al. 1975; Browne 1976, 1978; Bruni et al. 1980; Lund and
Trolle 1973; Sherard et al. 1980), though valproic acid is
considered a better choice in patients with both absences
and generalized tonic-clonic seizures, as seen in this
syndrome (Benbadis 2005). Lamotrigine and topiramate
have shown some success, but are not yet specifically
approved for use in juvenile myoclonic epilepsy (Beran et
al. 1998; Cross 2002; Frank et al. 1999). Interestingly, one
study showed greater attention, short-term memory, and
processing speed deficits in juvenile myoclonic epilepsy
patients that were treated with topiramate versus valproic
acid (de Araujo Filho et al. 2006).
Several studies have documented the cognitive and
psychological difficulties in this syndrome. For example,
Devinsky et al. (1997) evaluated 15 juvenile myoclonic
epilepsy patients and compared the results of neuropsycho-
Neuropsychol Rev (2007) 17:427–444
logical tests of executive functions to those obtained by a
group of TLE patients matched for IQ. Variable perfor-
mances were seen across juvenile myoclonic epilepsy
patients, with some showing no cognitive difficulties and
others showing significant impairment. Overall, high
frequencies of impaired performances were seen on tests
of concept-formation/abstract reasoning, speed of cogni-
tion, planning, and organization. Significant differences
between patient groups were seen on tests of mental
flexibility and concept formation (Devinsky et al. 1997).
A later study compared the neuropsychological perform-
ances of 50 patients with juvenile myoclonic epilepsy to 50
age, education, and gender matched controls. This study
showed more widespread deficits than earlier work. Patients
had significantly poorer performances on tests of attention,
inhibition, working memory, processing speed, and mental
flexibility, in addition to deficits in verbal and visual
memory, naming, and verbal fluency. Additionally, duration
of epilepsy was associated with greater cognitive impair-
ment, but not for patients with more education (Pascalicchio
et al. 2007).
Trinka et al. (2006) administered the Structured Clinical
Interview for DSM-IV Axis I (SCID-I) and the Structured
Clinical Interview for DSM-IV Axis II (SCID-II) to 21
males and 22 females with juvenile myoclonic epilepsy.
Thirty-five percent had a comorbid Axis I psychiatric
disorder, most commonly with anxious features, and 23%
had comorbid personality disorders. These numbers are
slightly higher than community estimates, which show 27%
incidence rate for Axis I disorders and 13.4% rate of
personality disorders (Trinka et al. 2006). However, the
rates of mood and personality disorders in juvenile
myoclonic epilepsy appear to be lower than that seen in
other seizure types, such as TLE (Perini et al. 1996).
To summarize, of the three generalized idiopathic
epilepsy syndromes discussed here, childhood absence
epilepsy tends to have the most favorable outcome. A fair
number of these patients will experience remission after a
period of time, but some may go on to experience other
seizure types. From a cognitive perspective, children with
childhood absence epilepsy may have subtle deficits in
attention and visual memory skills. Limited information is
available regarding the outcomes of juvenile absence
epilepsy, but it is generally considered to be a less benign
form of epilepsy. In juvenile myoclonic epilepsy, frontal
deficits may predominate, but there is clear evidence of
memory and language deficits in these individuals as well.
Focal Epilepsies
The focal epilepsies are characterized by seizures in which
onset of the initial electrical activation occurs in a specific
Neuropsychol Rev (2007) 17:427–444
(i.e., focal) area of the brain. Based on their etiology, partial
seizures are distinguished as (1) idiopathic; (2) symptom-
atic; or (3) cryptogenic (i.e., probably symptomatic). Partial
seizures predominate in these patients. Overall, partial
seizures account for approximately 40–60% of children
with childhood epilepsy, and complex partial seizures
appear to be more prevalent in children than simple partial
seizures (Cowan 2002).
Seizure syndromes classified under the heading of focal
idiopathic epilepsies of childhood, also termed the “benign”
focal epilepsies, are among the most common epilepsies in
children between ages three and 12. The two most
commonly recognized and studied focal idiopathic epilep-
sies are benign epilepsy with centrotemporal spikes (also
termed Benign Rolandic Epilepsy) and childhood epilepsy
with occipital paroxysms. Focal symptomatic (or presum-
ably symptomatic) epilepsy refers to epilepsies in which
there is a known or suspected underlying lesion, although it
should be noted that in many cases no underlying
pathology is ever identified. TLE and FLE are the primary
syndromes included under the classification of symptomatic
or probably symptomatic epilepsy. Little information is
available regarding incidence and prevalence rates specific
to temporal and frontal lobe epilepsy, as these patients are
often grouped together in epidemiological studies. The total
prevalence rates for simple partial seizures, complex partial
seizures, and secondary generalized seizures in children
have been reported as 2–12, 8–31, and 7–29% respectively
(Cowan 2002).
The treatment of partial seizures in childhood involves
the use of first generation and recently introduced anti-
epileptic drugs, as well as nonpharmacologic options
including the ketogenic diet, vagus nerve stimulation, and
surgical intervention. First line antiepileptic therapies for
partial seizures include carbamazepine and valproic acid,
whereas phenobarbital and phenytoin are usually consid-
ered as last choice drugs because of their adverse event
profiles; phenobarbital, topiramate, and zonisamide have
been associated with the most cognitive side effects
(Coppola 2004).
Benign Epilepsy of Childhood with Centrotemporal Spikes
(Benign Rolandic Epilepsy)
Benign Rolandic Epilepsy is the most common epilepsy
syndrome of childhood, representing about 15% of all
childhood seizure disorders (Sidenvall et al. 1993). Onset is
typically between ages 3 and 13 years, with a peak around
ages 8–10. Males are more frequently affected. A genetic
basis for BRE has been suggested by twin studies (Eeg-
Olofsson et al. 1982), and it has been linked to chromo-
some 15q14 (Neubauer et al. 1998). Though there was an
early suggestion of an autosomal inheritance pattern, later
431
studies suggest a more multifactorial inheritance, and more
recent work has shown that non-inherited factors are more
important than once believed (Vadlamudi et al. 2006). The
characteristic EEG findings involve high voltage centro-
temporal spikes followed by slow waves. These tend to be
activated by sleep and may shift from side to side. Seizures
seen in rolandic epilepsy frequently affect the oropharyn-
geal muscles and are often described as brief, simple
partial, hemifacial motor seizures, but they may evolve into
generalized tonic-clonic seizures. Treatment with antiepi-
leptic medications is not always warranted because the
seizures tend to be infrequent, typically occur at night, and
often spontaneously remit by the mid-teenage years. That
being said, there are certain circumstances under which
treatment is favored, including a young age of onset, a short
period of time between the first three seizures, and daytime
seizures (Bourgeois 2000). The typical treatment, when
warranted, is valproic acid or carbamazepine, although it
should be noted that carbamazepine may adversely affect
verbal memory in children treated for BRE (Seidel and
Mitchell 1999). Levitiracetam has also been approved for
use in children with BRE (Bello-Espinosa and Roberts
2003).
Despite its status as a benign syndrome, there is
increasing evidence to suggest that these children have
neuropsychological impairments that may be overlooked. A
1999 study in Turkey suggested deficits in vocabulary,
prosody, motor skills, and frontal functions. In this
investigation, 20 children diagnosed with BRE were
compared to 15 controls of comparable age and gender
distribution. The patient group was more frequently
impaired on tests of vocabulary, dyspraxia in the leg to
imitation, dysprosody, response inhibition on a go/no-go
task, and motor deficits of the upper extremities. Although
this study suggested that deficits in children with BRE
might indeed exist, as acknowledged by the authors, a
major weakness of this study is the lack of standardized
neuropsychological measures in Turkish (Gunduz et al.
1999).
Using a more comprehensive neuropsychological bat-
tery, Croona et al. studied 17 children with BRE and
compared performances to age, gender, and estimated
intelligence matched controls. Intellectual ability was
assessed by Raven’s Progressive Matrices. A variety of
tasks were used to examine attention span, verbal memory,
visual memory, and executive functioning. Moreover,
parents and teachers completed questionnaires regarding
the children’s cognitive function and academic achievement
abilities. Patients and controls performed equally well on
numerous tasks, including digit span, block span, Trailmak-
ing Test, the Rey Complex Figure delayed recall, and the
Spatial Learning Task. However, the patient group per-
formed more poorly than controls on tests of verbal fluency,
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verbal memory, and planning. Moreover, the parents of
patients reported more difficulties with respect to distract-
ibility, concentration, mood swings, impulsivity, under-
standing instructions, etc. (Croona et al. 1999).
Northcott et al. studied 16 girls and 26 boys with BRE
(Northcott et al. 2005). The mean age at evaluation was
8.5 years, with seizure onset ranging from 3 to 11. The
patients were administered a comprehensive battery of
neuropsychological tasks, and one sample z-tests were used
to compare performances to normative populations. EEG
features were also assessed, including spike frequency,
trains (i.e., runs of discharges of 5s or more), and laterality.
Correlational analyses assessed the relations between EEG
variables and cognitive functions (Northcott et al. 2005).
The sample had higher than expected performances on tests
of general intellectual functioning and general language.
Further, academic achievement (i.e., basic reading, spelling,
and mathematics) was not impaired relative to normative
values. However, both verbal and visual memory indices
were below expected levels, as were more specific language
functions (e.g., phonological awareness). Thus, in contrast
to prior work (Croona et al. 1999), this study showed more
generalized memory deficits, possibly due to improved
statistical power. EEG features were minimally associated
with cognitive variables, but there was no relation between
spike burden and laterality (Northcott et al. 2005).
Interestingly, a subset of this sample was followed
longitudinally. Twenty-eight participants underwent re-
evaluation, and differences were evaluated via t-tests.
Improvement was observed in verbal memory and receptive
language, whereas visual memory performance and phono-
logical awareness did not improve (Northcott et al. 2006).
Thus, despite being considered an epilepsy syndrome
with a benign course, individuals with BRE may present
with clear neuropsychological deficits despite overall intact
intelligence. These include deficits in aspects of language
and memory, which is not surprising given the character-
istic epileptiform discharges in the centrotemporal regions.
There may be some degree of motor and executive function
impairment in this group as well.
Childhood Epilepsy with Occipital Paroxysms
Occipital lobe epilepsy accounts for 6–8% of individuals
with focal epilepsy (Manford et al. 1992) and is more
common in children than in adults (Sveinbjornsdottir and
Duncan 1993). There are two types of occipital lobe
epilepsy currently recognized by the ILAE, including early
onset benign childhood occipital lobe epilepsy (Panayioto-
poulos type) and late onset childhood occipital lobe
epilepsy (Gastaut type; Engel 2006). The former generally
has a more favorable outcome. Though there are few
studies on this syndrome, several neuropsychological
Neuropsychol Rev (2007) 17:427–444
studies have found cognitive impairments in children with
benign childhood occipital epilepsy (Chilosi et al. 2006;
Germano et al. 2005; Gulgonen et al. 2000).
In one study, children with occipital lobe epilepsy
performed significantly lower than controls (matched for
age, sex, and socioeconomic status) on measures of
intellectual functioning, particularly with respect to perfor-
mance IQ (Gulgonen et al. 2000); however, another study
found verbal IQ to be differentially impacted relative to
controls, with no difference found between groups on
performance IQ (Germano et al. 2005). In both studies, the
children with epilepsy showed significantly reduced per-
formances across a wide variety of cognitive tasks,
including attention, memory, visuospatial skills, language
skills, and motor skills, and in the Gulgonen et al. (2000)
study, group differences remained significant (or reached
significance) when intellectual functioning was controlled
for. Gulgonen et al. (2000) did not find differences between
groups with respect to academic achievement, visuomotor
skills, or executive functioning, whereas Germano et al.
(2005) found reduced reading, writing, and calculation
abilities in their epilepsy group; in the latter study,
performances on several lexical tasks were associated with
visual memory and graphomotor skills.
Temporal Lobe Epilepsy
Temporal Lobe Epilepsy is among the most common types
of epilepsy in both adults and children, and the associated
cognitive profile is undoubtedly the most studied of the
epilepsy syndromes (Deonna et al. 1986). Complex partial
seizures are the most common type of seizure associated
with TLE, although simple partial seizures do occur, and
complex partial seizures often lead to secondarily general-
ization (Cowan 2002). Auras often precede temporal lobe
seizures and may involve somatosensory symptoms, in-
cluding epigastric rising sensation, olfactory symptoms, and
emotional symptoms (e.g., feeling of fear); many patients
report experiencing a “funny feeling” prior to the onset of
the seizure. The actual complex partial seizure generally
consists of behavioral arrest, unresponsiveness and staring,
stereotyped automatisms, and tonic motor phenomena
(Bourgeois 1998). Auras and seizures can present very
differently in infants and young children.
Temporal Lobe Epilepsy in children usually begins
during the school years, and when temporal lobe seizures
are seen in young children there is often an underlying
lesion (Jambaque 2001). Onset in childhood is generally
associated with a poorer cognitive outcome than in adult
onset cases. Further, these children are at a greater risk of
developing learning disabilities, which may have implica-
tions for success later in life (Jambaque et al. 1993;
Williams et al. 1996). Lower age of onset has been
Neuropsychol Rev (2007) 17:427–444
associated with lower performance IQ, and patients with
later onset performed better when copying the Rey
Complex Figure (Jambaque et al. 2007). Persistent seizures
in childhood might slow down the rate of cognitive and
psychological development (Oguni et al. 2000). Children
with longer duration of epilepsy have been shown to have
lower IQs than children with a shorter seizure history
(Robinson et al. 2000), and long-term effects of intractable
seizures may be more deleterious for children than adults
(Bjornaes et al. 2001). Not surprisingly, the most prominent
deficits pertain to memory, which may be seen in the
context of average intellectual functioning. However,
impairments are not always limited to memory, and several
factors contribute to the progression of broader deficits,
including age at seizure onset and level of seizure control
(Hermann et al. 2002).
In a cross-sectional investigation of patients with TLE
(early versus late onset) and controls, the early onset group
(onset before age 14) performed more poorly than the late
onset group and controls on all measures of intelligence and
memory (Hermann et al. 2002). There were fewer differ-
ences between the late onset group and controls, despite the
fact that this group had epilepsy for an average of 16 years.
Further, neuroimaging showed that the early onset group
had smaller total cerebral tissue volume and smaller
hippocampal volume relative to late onset patients and
controls. Within the early onset patients, duration of
epilepsy was directly related to cognition when age of
onset, gender, and education were covaried. The authors
concluded that onset of TLE in childhood adversely affects
the development of brain structures and function, which
often extends outside the primary epileptogenic region.
Childhood onset TLE has also been associated with
reduction in corpus callosum volume when compared to
both late onset patients and healthy controls (Hermann et al.
2003). Smaller corpus callosum volume was linked to
poorer performances on nonverbal problem solving tasks,
immediate memory, speeded complex motor ability, and
fine motor dexterity.
Whereas several adult studies have shown dissociation
between verbal and visual memory impairment as a
function of side of focus, child studies have been less
consistent. Several child studies have found either no
relationship between the side of seizure focus and material
specific memory performances (Camfield et al. 1984; Lendt
et al. 1999), equivalent deficits in verbal memory perfor-
mance regardless of seizure side (Igarashi et al. 1995), or
lower verbal memory performances in left-TLE patients in
the absence of concomitant visual memory impairments in
right-TLE patients (Clusmann et al. 2004). However, some
have indeed shown modality specific impairments (Cohen
1992; Jambaque et al. 1993, 2007). Beardsworth and Zaidel
found children with right-TLE to be differentially impaired
433
in memory for faces relative to left-TLE patients and
controls (1994). In a study that used proton magnetic
resonance spectroscopy in children with intractable TLE,
patients with left temporal pathology showed more im-
paired verbal learning than patients with right-sided
pathology (Gadian et al. 1996); the authors did not
administer a visual memory task. This investigation also
showed poorer general verbal abilities associated with left
temporal pathology, while right-sided pathology was
associated with a loss of nonverbal cognitive functions.
Forty-five percent had bilateral pathology; the authors
noted that without sophisticated imaging, undetected
pathology could account for contradictions in the literature.
In contrast, an earlier study did not find differences
between cognitive profiles of children with “pure right”
versus “pure left” temporal lobe seizure foci, and few
participants showed evidence of cognitive dysfunction at all
(Camfield et al. 1984). However, the majority had excellent
seizure control, and very few had severe protracted TLE.
Further, they did not account for handedness or language
lateralization. Subsequent studies have suggested a moder-
ating effect of language lateralization to overall intellectual
functioning. Children with atypical language dominance
may perform more poorly on both verbal and nonverbal IQ
subtests (Billingsley and Smith 2000). Gleissner et al.
(2003) compared neuropsychological performances of
children with left-sided focal epilepsy and either atypical
language representation (i.e., right or bilateral) or left-
hemisphere language representation patients matched for
age. Among the atypical language group, there were more
patients with atypical hand dominance, extratemporal
lesions, and earlier onset of epilepsy. Consistent with a
“crowding effect,” patients with atypical language domi-
nance demonstrated significantly lower performance in
visual memory, yet there was a trend in this group to show
better verbal memory performance than seen in the typical
language dominance group. These findings highlight the
importance of considering the possibility of language shift
in presurgical neuropsychological workups in patients with
early left-hemisphere damage. In cases where atypical
language dominance is established, an extratemporal focus
should be considered. In the absence of data on language
lateralization, handedness should be considered when
interpreting neuropsychological profiles of these patients.
Attention impairments are widespread in children with
epilepsy, regardless of seizure focus, and such deficits may
be a leading cause of learning difficulties in these patients
(Dunn and Kronenberger 2005; Schubert 2005). Though
improvements in attention are often seen after temporal
lobe surgery (Clusmann et al. 2004; Jambaque et al. 2007;
Lendt et al. 1999), children who undergo resective surgery
may not improve to levels seen in normally developing
children (Billingsley et al. 2000). With respect to treatment,
434
evidence clearly indicates that stimulants are safe and
effective in treating the attention deficits seen in children
with epilepsy (Dunn and Kronenberger 2005).
Executive function impairments have also been seen in
children with TLE that were administered a modified
version of the Wisconsin Card Sorting Test (WCST;
Igarashi et al. 2002). Children with TLE and hippocampal
atrophy performed more poorly (i.e., fewer categories
achieved and more perseverative errors) than those without
visible hippocampal damage, though impairment was less
severe than that seen in children with FLE. Interestingly,
seizure onset prior to 40 months of age was associated with
better performance, which was attributed to greater brain
plasticity in younger children.
Language deficits, such as deficits in naming and
vocabulary, have been seen in children with left-TLE
(Jambaque et al. 1993), and earlier onset may be associated
with developmental language problems (Jambaque 2001).
Jambaque and colleagues found post-operative naming to
be higher in children with later epilepsy onset, despite the
fact that post-operative gains in Verbal IQ were associated
with earlier age of onset, a finding that that may point to
a specific risk to the development of semantic knowledge
in younger patients (Jambaque et al. 2007). Reading
development may be problematic in children with TLE
(Williams et al. 1996), and these patients show greater
reading difficulties than do children with idiopathic
generalized epilepsy.
The role of the temporal lobes (especially the right
temporal lobe) in social and emotional perception is well
established in the literature, but few studies have examined
this in children with TLE. One study showed that right-TLE
patients with early-onset seizures (before age five) were
selectively impaired in recognizing fearful faces when
compared to right-TLE patients with late-onset seizures
and controls. Those with early onset seizures were also
impaired in their ability to recognize expressions of sadness
and disgust relative to controls, whereas there was not a
significant difference between controls and the late-onset
seizure group (Meletti et al. 2003). In another study that
compared the ability to perceive emotional gesturing and
prosody in children with right- and left-TLE, only the right-
TLE group was significantly impaired relative to healthy
controls (Cohen et al. 1990). There was no significant
difference between the left- and right-TLE groups, or
between the left-TLE group and controls.
Surgery is recognized as a safe treatment option for
adults with intractable TLE, but neurologists tend to be
more conservative in considering this in children, despite
mounting evidence of positive outcomes in children who
received surgery after poor management with medication.
Patients that received resective surgery as a child have been
Neuropsychol Rev (2007) 17:427–444
shown to have better long-term socioeconomic outcome
and quality of life (Keene et al. 1998a), as well as overall
satisfaction with the surgery (Keene et al. 1998b), when
seizures were reduced by 50% or more (Engel Class I–III).
Further, studies have shown that cognitive functioning
tends to remain stable or improve after surgery, especially
when it is undertaken early in the disease course (Jambaque et
al. 2007). The risk for decline in intelligence level after
temporal lobe surgery is low (Kuehn et al. 2002; Westerveld
et al. 2000; Williams et al. 1998), but there is evidence that
higher preoperative functioning is a risk factor for postop-
erative decline (Szabo et al. 1998). Risk factors for decline
may also include older age at time of surgery and the
presence of a structural lesion other than mesial temporal
sclerosis on imaging.
Children who underwent left temporal lobectomy not
only maintained presurgical verbal intellectual functioning
but also were found to improve significantly in nonverbal
intellectual functioning (Jambaque et al. 2007; Westerveld
et al. 2000). Children who have undergone right temporal
lobectomies typically do not show significant changes in
intellectual functioning (Jambaque et al. 2007; Westerveld
et al. 2000). In comparing adults and children who have
undergone temporal lobectomies in the context of similar
pathology, adults tend to show less recovery of memory
skills (Gleissner et al. 2005). Generally speaking, post-
operative declines may be seen at shorter retest intervals,
but improvement is generally observed at longer follow-up
(Adams et al. 1990; Szabo et al. 1998; Williams et al.
1998). In an investigation of children and adolescents who
received a selective surgical procedure that spares the
lateral temporal cortex (transparahippocampal selective
amygdalohippocampectomy), no changes in cognitive
functioning were seen post-operatively, with the exception
of significant improvement in rote verbal memory scores
among patients who underwent right-sided surgery (Robinson
et al. 2000). In this study, high premorbid functioning was
not associated with post-operative decline, as has been seen
after anterior temporal lobectomy (Chelune et al. 1998;
Hermann et al. 1995; Szabo et al. 1998); therefore this may
be a better surgical option for children, particularly in
children who show strong verbal memory skills prior to
surgery.
The intracarotid amobarbital procedure (Wada test),
which can establish language dominance and lateralized
memory functions, has shown utility in predicting post-
surgical memory improvement in children. In one study,
verbal memory was improved for children who showed
asymmetries in the predicted direction (i.e., memory after
the injection on the side ipsilateral to surgery better than
memory after contralateral injection), whereas children who
did not show such memory asymmetries showed a
Neuropsychol Rev (2007) 17:427–444
significant decline in verbal memory post-surgically (Lee et
al. 2005).
To summarize, there are both similarities and differences
between the profiles of children and adults with TLE.
While onset later in life is linked primarily with memory
deficits, an earlier onset appears to have a more widespread
impact on brain structure and function. With respect to
memory, although several adult studies have found modal-
ity specific memory deficits depending on the side of
pathology, results from child studies are far from conclusive
in this regard, perhaps due to the greater plasticity in
children. Language lateralization further complicates this
issue. Regarding treatment of TLE in childhood, the
preponderance of evidence suggests that not only is surgery
a safe and effective treatment for reducing seizures in
children, but it also appears to be associated with positive
cognitive outcomes. However, high pre-operative verbal
memory skills have been associated with greater post-
operative declines, though it is worth noting that the effect
of prolonged seizures may ultimately be more devastating
in the long run. A better outcome can generally be expected
when surgery is undertaken early in the disease course.
Neuropsychologists consulted during pre-surgical workups
should be aware of issues contributing to post-operative
outcomes.
Frontal Lobe Epilepsy
Frontal lobe epilepsy accounts for approximately 30% of
partial epilepsy, and it represents the largest subgroup of
extratemporal lobe epilepsy. Frontal lobe seizures often
have a bizarre clinical presentation, in the context of little or
no interictal and ictal EEG abnormalities, and they are often
mistaken for nonepileptic seizures (Arunkumar et al. 2001).
Most neuropsychological studies of FLE in children have
been single case studies (Hernandez et al. 2001). In a study
that compared neuropsychological performances of children
with symptomatic TLE and FLE, a differential impairment
in concept formation on a modified (i.e., simpler) WCST
was seen in the latter (Igarashi et al. 1995), a finding that
was confirmed in a subsequent study (Igarashi et al. 2002).
However, a study that used the original version of the
WCST did not find children with asymptomatic FLE to be
impaired relative to controls with respect to number of
categories completed or in the number of perseverative
responses (Riva et al. 2005), despite deficits on other
executive measures, including phonemic (but not semantic)
fluency and design fluency. Mean Full Scale IQ was
average in the latter study, while lower IQ scores were
reported in the Igarashi studies, and this may account for
the discrepant findings. Riva et al. administered the
California Verbal Learning Test in addition to traditional
435
measures of executive functioning, and though this sample
was not impaired relative to controls, age at seizure onset
and duration of epilepsy were related to the total number of
words recalled, use of semantic clustering strategies, short
delay free recall, and long delay free and cued recall. In
another study that compared children with idiopathic FLE
and TLE on measures of attention, executive functioning,
memory (verbal and nonverbal), and adaptive functioning,
FLE patients showed greater deficits in planning and
executive functions in the context of intact memory
performances; the opposite pattern was seen in the TLE
group (Culhane-Shelburne et al. 2002). Furthermore, 50%
of the variance in the Vineland Adaptive Behavior
Composite was accounted for by performances on two
measures of executive functioning (Stroop—Color/Word
and Tower of London—Rule Violations).
Profiles of children with FLE, TLE, and generalized
epilepsy (typical absence) were compared on tests of motor
ability and executive functioning (Hernandez et al. 2002).
Groups were matched for age, IQ, age at seizure onset, and
duration of epilepsy. The groups did not differ with respect
to IQ (full scale, verbal, or performance), but children with
FLE showed deficits on tasks of motor coordination,
response generation, impulse control, and planning ability
relative to the other seizure groups. With regard to motor
skills, deficits were particularly apparent during tasks
involving bimanual coordination and asymmetrical move-
ments, with younger children (ages 8–12) showing more
pronounced deficits. In a related study on the same cohort
of children, performances on measures of attention,
processing speed, and memory (verbal and nonverbal), as
well as parent ratings of behavior were compared (Hernandez
et al. 2003). All three groups were impaired relative to
controls on attention and memory tasks, but attention
problems were more prominent in the FLE group, particu-
larly with respect to sustained attention and complex
working memory on a continuous performance test. This
group also displayed differential impairments in perceptual
organization and self-regulation of behavior across several
tasks. On a list-learning task, more intrusion errors were
made and these patients were more prone to interference
than the other patient groups, despite comparable overall
learning and recall between groups; all three groups were
deficient relative to healthy controls. The FLE and TLE
groups both had difficulty reproducing a complex figure
relative the generalized epilepsy group and controls. Recall
difficulties in the FLE group were due to organizational
difficulties when copying the design, whereas the TLE
group’s difficulties appeared to be due to impaired encoding.
Thought problems, behavior problems, and social adjustment
problems were reported more frequently in children with
FLE than in the other two groups on behavior rating scales.
436
The authors noted that the number of antiepileptic drugs
taken by children did not appear to affect attention, memory,
and behavior significantly.
Another group examined performances of children with
TLE, FLE, and controls on a measure of preparatory
attention (Auclair et al. 2005). They found that the FLE
group showed a higher mean slope of response time to a
target as a function of distractor probability (i.e., a
distractor was occasionally presented prior to the target)
compared to children with TLE and control subjects.
Children with TLE did not differ from controls in their
response time slope. A high incidence of attention deficit
and hyperactivity, learning difficulties, and behavioral
problems has been observed in children with FLE, even
when seizures are well-controlled (Prevost et al. 2006).
Cohen and Le Normand (1998) conducted yearly
evaluations of receptive and expressive language skills in
a small sample of young children with left frontal simple
partial seizures and compared results to controls. Analyses
of individual language trajectories revealed a clear dissoci-
ation in linguistic performance between comprehension and
production. Linguistic comprehension gradually improved
to reach normal performance levels by age seven, whereas
production, even at later stages, remained quite poor
(Cohen and Le Normand 1998). Children with FLE
exhibited significant deficits on phonological processing
tasks when compared to children with TLE and generalized
absence seizures (Vanasse et al. 2005).
Surgical intervention is also a viable option for patients
with FLE, though the likelihood of becoming seizure-free
post-surgically is lower in this group in comparison to
temporal lobe surgery patients, and there is increased risk of
damage to eloquent cortex. Lendt and colleagues investi-
gated the neuropsychological function of children with FLE
before and 1 year after surgery (Lendt et al. 2002), and
compared their performance to children who underwent
temporal lobe surgery for seizures. The FLE group
demonstrated higher intellectual functioning prior to sur-
gery but were more impaired in manual motor coordination
than the TLE group. Post-surgically, both groups improved
in attention, processing speed, short- and long-term
memory, and manual motor coordination (although the
latter was not significant). There was no improvement in
executive functions in either group. Post-operative
improvements did not depend on complete seizure relief
after surgery. Age at surgery and side of resection did not
moderate the effects that were seen. Only two patients
showed a decline in language, executive, or motor
functions; no factors could be identified that accounted for
these changes. Overall, outcome was favorable in this
group, but the small sample size and associated poor
statistical power may explain the lack of group differences
in the cognitive outcome of FLE and TLE patients. The
Neuropsychol Rev (2007) 17:427–444
authors point out an interesting caveat to these results:
future deficits may emerge as the frontal lobes continue to
develop into adolescence and adulthood (a phenomenon
that has been reported after frontal lesions), and they
underscored the need for longitudinal studies that utilize
longer retest intervals.
In summary, FLE in children appears to be associated
primarily with deficits in executive functioning, attention,
and motor skills, often in the context of normal intellectual
function. Executive deficits are believed to be the primary
cause of memory impairments in this group. Further, social
and behavioral problems are common. While surgery has
been shown to improve attention, processing speed, and
memory in children with FLE, the benefit to overall
executive functioning and/or behavior is less clear. How-
ever, few studies have examined this issue and results are
limited by small sample sizes.
Epileptic Encephalopathies
Epileptic encephalopathies are a group of syndromes
characterized by deterioration of sensory functions, motor
functions, and/or cognition as a result of epileptic activity,
including frequent seizures and/or prominent interictal
paroxysmal activity (Nabbout and Dulac 2003). In most,
there is either a regression of cognitive development or a
failure to attain developmental milestones. The long-term
cognitive and behavioral outcome is often poor. Despite
commonalities between syndromes, studies have attempted
to differentiate the cognitive profiles of disorders; some have
distinct patterns of strengths and weaknesses. Several
syndromes present with such profound developmental delay
that they are rarely seen by pediatric neuropsychologists for
assessment, and neuropsychological studies are virtually
absent. Only those syndromes for which neuropsychological
studies were available will be discussed below.
Dravet’s Syndrome (Severe Myoclonic Epilepsy of Infants)
The incidence of Dravet’s syndrome is estimated to be one
in 40,000 children under the age of seven (Hurst 1990).
Onset occurs within the first year of life in normally
developing children. A family history of epilepsy or febrile
convulsions have been noted in up to 64% of cases
(Ohtsuka et al. 1991) and recent investigations have found
mutations in the SCNA1 gene in approximately 35% of
children with Dravet’s syndrome (Oguni et al. 2005). There
is molecular evidence to suggest Dravet’s syndrome may be
on the spectrum of generalized epilepsy with febrile
seizures “plus,” along with myoclonic astatic epilepsy
(discussed below; Scheffer et al. 2001). Dravet’s syndrome
is characterized by frequent prolonged seizures that often
Neuropsychol Rev (2007) 17:427–444
lead to recurrent status epilepticus. Seizure focus tends to
switch sides from one event to the next, and they are
refractory to antiepileptic medications (Wirrell et al. 2005).
Myoclonic seizures appear between 1 and 4 years and are
often accompanied by absence and focal seizures. A recent
study that assessed the intellectual functioning of 53
patients with Dravet’s syndrome found mild developmental
delay in 18 children (34%), moderate delay in 22 children
(41.5%), and severe delay in 14 children (26%; Caraballo
and Fejerman 2006). Hyperactivity was observed in the
majority (85%). Interpersonal relationships rarely exceed
the developmental level of 2 years of age, and these patients
sometimes exhibit autistic traits (Casse-Perot et al. 2001).
Wolff et al. (2006) conducted a prospective longitudinal
neuropsychological study in 14 patients with Dravet’s
syndrome in order to elucidate the nature of the cognitive
and behavioral profiles associated with this syndrome. The
authors administered a battery of 50 neuropsychological
tests, and scores were used to calculate a global develop-
mental quotient, as well as a quotient for each domain.
Before the onset of seizures, psychomotor development and
behavior were reportedly normal in all of the children. The
age at first assessment ranged from 11 months to 12 years.
Four children underwent neuropsychological testing in the
first 2 years of life, and their global developmental quotients
were found to be only “slightly deficient” at that time.
Longitudinal data indicated that after the age of four, the
developmental quotient tended to decrease until age six,
after which it remained relatively stable at a low level.
Severe mental retardation, hyperactivity, poor relational
capacity, and gestural stereotypies were evident in most
children even before the age of six. Subtest results revealed
poor visuomotor skills in all children over 4 years. Lan-
guage results were more heterogeneous, yet language skills
were better than visuospatial skills in only three children;
these three patients showed a more favorable outcome
overall. A trend was noted whereby children with fewer
convulsive seizures (<5 per month) performed better than
those with higher seizure frequency.
West Syndrome
Also known as infantile spasms, West syndrome is
diagnosed in children who exhibit the triad of infantile
spasms, psychomotor deterioration, and hypsarrhythmia.
Onset of seizures generally occurs between three and
12 months of age. Causative factors include neurological
insult (e.g., infection or hypoxia-ischemia), cortical malfor-
mations, neurocutaneous syndrome (e.g., tuberous sclerosis
complex and Sturge–Weber syndrome), chromosomal or
single gene disorder, or an inborn error of metabolism
(Wirrell et al. 2005). The incidence rates of West syndrome
have been estimated at 2–4.5 per 10,000 live births per
437
year, while the prevalence rates for children 10 years of age
or older are about 1.5–2 per 10,000 (Cowan 2002).
Approximately 50–70% of children with infantile spasms
will develop other seizure types (Cowan 2002), with
Lennox–Gastaut syndrome comprising approximately 20–
50% of these patients (Appleton 2001). At least a third of
the cases of West syndrome are idiopathic (Nabbout and
Dulac 2003), and these patients generally show a more
favorable outcome (Guzzetta 2006).
Early signs of cognitive impairment include deterioration
of responsiveness and sensory abilities, as well as poor
social contact, which is often the reason that parents seek
medical attention (Guzzetta 2006). Impaired eye-hand
coordination was found to be deficient when infants were
evaluated, even in idiopathic patients who appear to be
functioning normally otherwise (Rando et al. 2005).
Impaired visual functioning involving acuity, ocular motil-
ity, visual field, visual attention, and visual scanning skills
have been found in several studies with infants (Guzzetta et
al. 2002; Jambaque et al. 1993; Rando et al. 2004).
Guzzetta (2006) reported an associated deficit in auditory
attention, and visual and auditory functions were both
correlated with cognitive competence on the Griffith Scales
of Infant Development. In general, long-term cognitive
outcome appears to depend on the underlying causes (e.g.,
lesion characteristics); however, about 80% of individuals
with the syndrome present with mental retardation (Guzzetta
2006; Koo et al. 1993; Matsumoto et al. 1981). Patients
with the idiopathic form of the disease whose spasms had
completely resolved within the first year were evaluated
immediately after spasms ceased and subsequently under-
went two follow-up evaluations (4–6 years later and 6–
8 years later; Gaily et al. 1999). Deficits were seen in
attention, learning, and memory, even in cases where
intellectual functioning was normal. Perception and fine
motor skills at the first assessment (8–15 months after
cessation of infantile spasms) strongly predicted later
cognitive functioning. In an earlier study that also focused
on the idiopathic form, preserved visual tracking during
infancy was associated with a more favorable outcome
(Dulac et al. 1993). Results from a longitudinal study that
followed 214 patients with West Syndrome for 20–35 years
(or until death) showed normal or only slightly impaired
intellectual outcome in a quarter of the patients (Riikonen
2001), although they too noted that specific cognitive
deficits were seen in some patients with normal intelli-
gence. Interestingly, in contrast to what is generally
reported in the literature, only a third of these patients
were found to have the idiopathic form of the syndrome,
but this difference might represent diagnostic errors. In
most cases there was no change in intellectual level as
compared to earlier evaluations at a mean age of 8 years,
and those that were ultimately placed in lower IQ categories
438
all had severe drug-resistant epilepsy. Behavioral disorders,
often accompanied by autistic symptomology, have been
noted as a potential outcome in approximately 15% of
patients with West syndrome, although the incidence rate
increases to 70% in patients with tuberous sclerosis
complex (Guzzetta 2006).
Lennox–Gastaut Syndrome
Lennox–Gastaut syndrome is very rare, with an estimated
incidence rate of 1–2 per 100,000 in children from birth to
14 years of age; the estimated prevalence rate is much
higher, at 1.3–2.6 per 10,000 (Cowan 2002). The peak age
of onset of seizures ranges from 2 to 8 years of age (Wirrell
et al. 2005), and approximately 20–60% of children with
Lennox–Gastaut syndrome have a history of infantile
spasms. The syndrome usually results from focal, multifo-
cal, or diffuse brain damage, although idiopathic cases are
reported and usually present with a later age of onset
(Nabbout and Dulac 2003). Children with Lennox–Gastaut
syndrome almost always present with both tonic and
akinetic seizures, and slow generalized spike-and-wave
discharges on EEG. The outcome for these children is
generally poor, with an estimated 91% exhibiting mental
retardation, often times profound (Cowan 2002). Further,
there is strong evidence to suggest that intellectual
functioning continues to deteriorate with age (Oguni et al.
1996), and one study reported that of patients who had
normal intelligence at baseline, only 26% of these patients
(33% of idiopathic, 17% indeterminate, and 20% of
symptomatic) had normal or borderline normal intelligence
at long-term follow-up (Goldsmith et al. 2000). Addition-
ally, many patients with Lennox–Gastaut syndrome develop
psychiatric and behavioral problems over time (Besag
2006), and long-term follow-up of patients has revealed
perseverative behavior, psychomotor slowing, and apathy
(Kieffer-Renaux et al. 2001). Poorer outcomes are predicted
when onset occurs prior to 3 years of age and there is a prior
history of West syndrome (Wirrell et al. 2005). One
retrospective study found that age of onset was a better
predictor of outcome than whether or not the disorder was
idiopathic in nature (Goldsmith et al. 2000); however, late
onset patients rarely had a history of infantile spasms, and
normal or borderline normal outcomes were seen in a
greater proportion of patients with idiopathic Lennox–
Gastaut syndrome than in symptomatic or indeterminate
forms. This effect disappeared when patients with mental
retardation at the onset were excluded from analyses. Vagus
nerve stimulation has been successful in improving seizure
frequency in some patients and may also lead to slight
improvements in cognition, behavior, and mood. However,
there is insufficient evidence to determine whether such
gains are maintained over time (Majoie et al. 2001, 2005).
Neuropsychol Rev (2007) 17:427–444
Myoclonic Astatic Epilepsy
Myoclonic astatic epilepsy is a syndrome that begins
between the ages of 2 and 5 years in otherwise normally
developing children (Nabbout and Dulac 2003). Genetic
factors are believed to play a role in its development, and
there is molecular evidence to suggest that it may be on the
spectrum of generalized epilepsy with febrile seizures
“plus,” along with Dravet syndrome (Scheffer et al.
2001). Seizures begin as generalized tonic-clonic, but are
eventually followed by myoclonic astatic seizures of
increasing frequency. Neuropsychological outcome in chil-
dren with myoclonic astatic epilepsy is quite variable and
depends on the course of the seizure disorder (Kaminska et
al. 1999; Oguni et al. 2002). Patients who present with
developmental delay are sometimes misdiagnosed, because
they share features of patients with idiopathic Lennox–
Gastaut syndrome. However, several studies have argued
that these are indeed two separate entities. Kaminska et al.
(1999) examined the clinical profiles and outcome of 72
children with idiopathic generalized epilepsy and found that
they were able to classify them into three distinct groups:
(1) myoclonic astatic epilepsy group with favorable
outcome; (2) myoclonic astatic epilepsy group with
unfavorable outcome; and (3) Lennox–Gastaut syndrome
group. The myoclonic astatic epilepsy groups both pre-
sented initially with similar cognitive abilities (mean IQ of
85 during the first 2 years). However, the second group
showed a deterioration in cognitive functioning (IQ <50 in
83% of patients), apparently due to persisting seizures that
were distinct from the pattern seen in the first group. In
contrast, seizures remitted entirely in the first group and
cognitive functioning was found to be only mildly impacted
at least 1 year after the last myoclonic event. Unlike either
of the myoclonic astatic epilepsy groups, the Lennox–
Gastaut group presented with a different seizure profile and
had mental retardation from the outset. Other studies have
failed to find a specific neuropsychological pattern associ-
ated with myoclonic astatic epilepsy but suggest that
cognitive and behavioral functioning are primarily affected
by the duration and frequency of epileptiform activity
(Filippini et al. 2006; Stephani 2006). Filippini and
colleagues described a representative patient (from a group
of seven) with the transitory form of myoclonic astatic
epilepsy who showed cognitive and behavioral disturbances
that remitted entirely with successful pharmacological
treatment of seizures.
Continuous Spike-and-waves During Slow-wave Sleep/
Landau–Kleffner Syndrome
Continuous spike-and-waves during slow-wave sleep
accounts for less than 1% of childhood onset epilepsies
Neuropsychol Rev (2007) 17:427–444
(Kramer et al. 1998), yet it has received considerable
attention in the literature due to its dramatic effects on
cognition and behavior. Peak age of onset is between 5 and
7 years, and there is a slight male preponderance (McVicar
and Shinnar 2004). Although most patients are reported to
have normal development prior to onset of symptoms, pre-
existing neurological abnormalities are reported in approx-
imately one third of patients. Patients with continuous
spike-and-waves during slow-wave sleep often have partial
and generalized seizures at the onset of the epilepsy
syndrome, yet the onset of cognitive deterioration leads to
a more comprehensive workup that reveals generalized
spike-wave discharges that occupy >85% of slow-wave
sleep, which is referred to as electrical status epilepticus in
slow-wave sleep (Camfield and Camfield 2002). Global
intellectual functioning, language, temporo-spatial disori-
entation, motor function, and behavior are all affected
(Tassinari et al. 2000). In their review of literature on
electrical status epilepticus in slow-wave sleep, Tassinari et
al. (2000) hypothesized that long-term and stable cognitive
impairment is the direct consequence of the status of
continuous spike-and-waves during sleep. They cite several
factors that are supported in the literature, including (1) a
close temporal association between the presence of status
epilepticus during sleep and neuropsychological regression;
(2) a parallel between duration of electrical status epilepti-
cus in slow-wave sleep and the final neuropsychological
outcome; and (3) the strict association between the pattern
of neuropsychological deficits and the location of the
interictal focus. Seizures are not easily controlled with
medication, although most patients’ seizures remit sponta-
neously in adolescence (McVicar and Shinnar 2004;
Scholtes et al. 2005).
The most well studied disorder associated with contin-
uous spike-and-waves during slow-wave sleep is Landau–
Kleffner syndrome, which is characterized by a dramatic
regression of language functions in children who have
already developed normal speech, and relative sparing of
other cognitive functions (Landau and Kleffner 1957;
Rotenberg and Pearl 2003). The peak onset is between 3
and 8 years of age, while language regression in children
under 2 or 3 years usually occurs in the context of a more
global autistic regression (McVicar and Shinnar 2004).
Clinical seizures are present in approximately 80% of
children with Landau–Kleffner syndrome, although they are
not necessary to make the diagnosis; in addition to
electrical status epilepticus in slow-wave sleep, these
patients often show prominent epileptiform activity in the
temporal lobes, usually bilaterally (Deonna 1991; McVicar
and Shinnar 2004). The incidence of Landau–Kleffner
syndrome is unclear, but it is thought to be quite rare. Its
pathology also remains unclear; however, one recent
imaging study that compared the volumes of superior
439
temporal areas in a small sample of these patients without
specific anatomic abnormalities to controls found bilateral
volume reduction in the patient group (Takeoka et al.
2004).
The language disorder of Landau–Kleffner syndrome is
characterized by a severe deficit in comprehension, often
referred to as a verbal auditory agnosia, though it often
progresses to include language production as well. A deficit
in phonemic discrimination has been described as the basic
disorder underlying the deterioration of receptive language
(Van Hout 2001). There has been considerable dispute in
the literature regarding whether Landau–Kleffner syndrome
is but one manifestation of continuous spike-and-waves
during slow-wave sleep, or whether it should be included as
an independent syndrome. While language deficits in
continuous spike-and-waves during slow-wave sleep are
often quite severe and long-standing, there is evidence to
suggest that these patients show a profile that is distinct
from Landau–Kleffner patients, which involves impair-
ments in lexical and syntactic skills in the context of spared
comprehension abilities (Debiais et al. 2007).
Unlike the poor outcomes typically seen in continuous
spike-and-waves during slow-wave sleep, the prognosis of
Landau–Kleffner is much more variable (Bishop 1985;
Deonna et al. 1989; Veggiotti et al. 2002). Although the
underlying seizure disorder is almost always treated
successfully with antiepileptic medication, many patients
continue to show language deficits (often severe) into
adulthood, despite treatment (Deonna 1991). Studies have
shown that a younger age at onset of the language
regression is associated with a poorer language outcome
(Bishop 1985), though one study found duration of
electrical status epilepticus in slow-wave sleep to be a
better predictor of outcome than age, with full recovery of
language functions occurring exclusively in patients who
had electrical status epilepticus in slow-wave sleep for less
than 3 years (Robinson et al. 2001). The results of the latter
study are limited by a small sample. Plaza et al. (2001)
described a child who was diagnosed with Landau–Kleffner
syndrome at 28 months (a right temporal focus was
identified on EEG) who ultimately recovered language
skills and acquired reading and spelling abilities. He
showed complete left extinction in dichotic listening and a
pattern of performance on memory tasks whereby very poor
recall was observed when verbal information was presented
auditorily, yet performances were intact when such infor-
mation was presented visually. The authors suggested that
early language regression might have been due to impaired
auditory perception, which he overcame by using compen-
satory strategies that allowed the development of phono-
logical skills from predominantly visual input.
Most studies of Landau–Kleffner syndrome have ex-
cluded patients whose receptive language was compro-
440
mised before the diagnosis was made and have instead
focused on the relationship of seizure control to language
recovery (Klein et al. 2000). Klein and colleagues investi-
gated the effect of premorbid language skills and behavior,
along with clinical variables, on long-term language
recovery. They found that premorbid language and behavior
were more predictive of language recovery than persistence
or absence of seizures. Results from this study underscored
the importance of assessing premorbid language and
behavior when making predictions regarding long-term
outcome.
Concluding Remarks
The neuropsychological literature with respect to seizure
disorders is expansive, and it is well established that
epilepsy in childhood is often associated with lowered
general intellectual functioning and specific cognitive
impairments. However, many studies have addressed
seizure disorders in general, and do not identify specific
epilepsy syndromes as outlined by the ILAE. The existing
research on specific syndromes suggests that each may
have very different cognitive outcomes, but more informa-
tion is needed. Better characterization of the deficits unique
to a given syndrome will aid clinicians in tailoring
neuropsychological batteries to assess functioning in a
given child. Future studies should strive to utilize well-
characterized groups using strict ILAE classification guide-
lines. Given the low base rates of some syndromes, this
may require collaborative multicenter efforts. Moreover,
longitudinal studies that assess cognitive changes over time
are warranted. In such studies, investigators should examine
not only the natural progression of the cognitive profiles
relevant to each syndrome, but also the pattern of changes
subsequent to specific treatments (e.g., surgery, vagus nerve
stimulator, etc.). As newer antiepileptic agents become
available, studies should seek to elucidate the cognitive side
effects or clarify their role in improving cognition.
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