Epilepsia, 50(Suppl.
7):9–12, 2009
doi: 10.1111/j.1528-1167.2009.02210.x
FIFTY YEARS OF LANDAU-KLEFFNER SYNDROME
Atypical rolandic epilepsy
Natalio Fejerman
Department of Neurology, Hospital de Pediatria JP Garrahan, Buenos Aires, Argentina
SUMMARY
Typical benign rolandic epilepsy (BRE) is a fre-
quent and well-delineated epileptic syndrome in
childhood. Mild cognitive and behavioral difficul-
ties are increasingly recognized in the course of
BRE and should not be considered as atypical fea-
tures. Atypical features are recognized on elec-
troclinical grounds. These features, particularly
early age at onset and frequent spikes or spike-
wave discharges, seem to be risk factors for neu-
ropsychological deficits but also for an atypical
Benign childhood epilepsy with centrotemporal spikes
(BCECTS), or benign rolandic epilepsy (BRE), is the most
frequent benign focal epilepsy in childhood and represents
about 20% of epilepsy syndromes in children younger
than 15 years of age (Watanabe, 2004; Dalla Bernardina
et al., 2005; Panayiotopoulos, 2005; Fejerman, 2008).
Typical clinical features are age of onset between 4 and
10 years and seizures related to sleep of short duration
(30–120 s) having suggestive semiology (i.e., orofacial
motor signs). Typical electroencephalography (EEG)
findings are normal background and interictal epileptic
discharges located in centrotemporal areas. Drowsiness
and sleep increase the rate of discharges, keeping the same
morphology as during wakefulness. Extreme discrepan-
cies between rarity of seizures and the activity of the EEG
foci are common.
Neuropsychological performance in children with BRE
was considered as normal and behavioral problems as less
frequent than in other forms of childhood epilepsy
(Heijbel & Bohman, 1975; Fejerman & Medina, 1986;
Lerman, 1998). However, since 1997, a wide spectrum of
neuropsychological deficits leading to academic under-
achievement was reported, as language impairments,
reading and spelling difficulties, attention deficits, and/or
Address correspondence to Dr Natalio Fejerman, Araoz 2867 3 ‘‘A,’’
1425 Buenos Aires, Argentina. E-mail: natalio@fejerman.com
Wiley Periodicals, Inc.
ª 2009 International League Against Epilepsy
9
evolution of BRE. Atypical evolutions of BRE are
defined by the appearance of severe neuropsycho-
logical impairments and continuous spike-and-
waves during slow sleep (CSWSS). The clinical
expressions of these situations correspond to the
syndromes known as atypical benign focal
epilepsy of childhood (ABFEC), status of BRE,
Landau-Kleffner syndrome (LKS), and CSWSS
syndrome, which may be part of a continuum
related to BRE.
KEY WORDS: Continuous spike-wave, Neuropsy-
chological impairments.
spatial perception deficits (Weglage et al., 1997; Staden
et al., 1998; Deonna et al., 2000; Monjauze et al., 2005;
Pinton et al., 2006; Volkl-Kernstock et al., 2006; Deltour
et al., 2007; Riva et al., 2007). Interestingly, the prevalence
of reading disabilities and speech sound disorders was
reported as higher in patients with BRE (but also in their
siblings) than in healthy controls (Clarke et al., 2007).
The relationship between cognitive deficits and EEG
abnormalities remains controversial in BRE. Authors
found a special pattern of difficulties in memory and pho-
nologic awareness with no correlation with EEG features
(Northcott et al., 2005). On the other hand, a relation
between the importance of cognitive impairments and
EEG abnormalities was found in several studies. Longitu-
dinal neuropsychological and EEG studies documented
that aggravation of paroxysmal EEG activity was corre-
lated with transient cognitive difficulties (Deonna, 2000;
Deonna et al., 2000; Baglietto et al., 2001). A prospective
detailed neuropsychological and EEG study of 35 children
with BRE showed that neuropsychological impairments
were clearly correlated with some EEG patterns (Massa
et al., 2001). This was confirmed by a study showing that
slow-wave focus during wakefulness, high number of
spikes in the first hour of sleep, and multiple asynchronic
spike-wave foci were associated with educational and
behavioral impairment (Nicolai et al., 2007). A more
recent study found specific learning disabilities associated
with marked increase in epileptiform discharges during
sleep in 9 of 20 patients with BRE (Piccinelli et al., 2008).
10
N. Fejerman
Atypical Features in Benign
Rolandic Epilepsy
Atypical features in BRE can be seen on seizure charac-
teristics (daytime-only seizures, postictal Todd paresis,
prolonged seizures, or even status epilepticus) or in EEG
features (atypical spike morphology, unusual location,
absence-like spike-wave discharges, or abnormal back-
ground) (Aicardi, 2000). Early age of onset of seizures
seems to be one of the most important items among atypi-
cal features (Kramer et al., 2002; Saltik et al., 2005, You
et al., 2006; Fejerman et al., 2007a). In a recent retrospec-
tive study of 126 case-series, atypical features were found
in almost half of the patients (Datta & Sinclair, 2007).
A follow-up study of patients with BRE reported a
higher percentage of learning and behavioral disabilities
in the group with atypical features (Verrotti et al., 2002).
In a recent prospective study of 44 children with BRE
divided into typical group (n = 28) and atypical group
(n = 16) on the basis of EEGs showing features as slow
spike-wave focus, synchronous foci, or generalized 3-Hz
spike-wave discharges, the atypical group had significant
lower full scale IQ and verbal IQ (Metz-Lutz & Filippini,
2006).
Atypical Evolutions of BRE
The concept of atypical evolutions does not include the
cases of BRE with atypical features, but refers to the pres-
ence of severe neuropsychological impairments that may
become persistent. These cases show on EEG continuous
spike-and-waves during slow sleep (CSWSS), which
seems to be a kind of bilateral secondary synchrony. The
reasons that some children develop this EEG pattern are
yet not understood. In some cases, certain antiepileptic
drugs (AEDs) seemed to be responsible (Shields &
Saslow, 1983; Caraballo et al., 1989; Prats et al., 1998;
Fejerman et al., 2000). These conditions correspond to the
syndromes known as atypical benign focal epilepsy of
childhood (ABFEC), status of BRE, Landau-Kleffner syn-
drome (LKS), and CSWSS syndrome (Tassinari et al.,
2005). For example, the cases of ABFEC described by
Aicardi and Chevrie (1982) showed atonic fits leading to
daily falls, and all of our cases presented important learn-
ing difficulties during the periods of CSWSS (Fejerman
et al., 2007b). Status lasting days or weeks including
motor facial seizures and anarthria with persistent drool-
ing constitutes another complication of BRE (Fejerman &
Di Blasi, 1987; Fejerman et al., 2000). Both complications
have shown an ultimate good prognosis. However, when
aphasia or global deterioration occurs, as in LKS and
CSWSS syndrome, the risk of permanent language or neu-
ropsychological dysfunction is clearly present (Fejerman,
1996; Fejerman et al., 2000, 2007b) (Fig. 1).
Epilepsia, 50(Suppl. 7):9–12, 2009
doi: 10.1111/j.1528-1167.2009.02210.x
Figure 1.
Benign focal epilepsies of childhood (BFEC) and dis-
orders related to CSWSS.
Epilepsia ILAE
Are these four conditions—ABFEC, status of BRE,
LKS, and CSWSS syndrome—independent syndromes or
part of a continuum related to BRE? Obviously, evidence
of these situations registered during the follow-up of a sig-
nificant number of patients with BRE is not sufficient to
generalize it to all the cases.
Is there a relation between atypical features and what
here are considered as atypical evolutions of BCECTS?
It is clear that not all the patients with BCECTS show-
ing atypical clinical and EEG features evolved into
ABFEC, status of BRE, LKS, or CSWSS syndrome. How-
ever, in a series of 43 children with ABFEC, a majority
started at early ages (Hahn et al., 2001). In our series of 39
idiopathic cases of ABFEC, status of BRE, LKS, and
CSWSS syndrome appearing after the onset of BCECTS,
25 of them started at ages of 4 years or younger (Fejerman
et al., 2007b). There is no clear explanation of this
repeated reported observation (Kramer et al., 2002; You
et al., 2006).
There are patients with BCECTS who have prolonged
intermittent drooling and oromotor dyspraxia associated
with a marked increase in centrotemporal spikes during
the episodes (Roulet et al., 1989); persistent slurred speech
as a single phenomenon was also reported (Kramer,
2008). Should we consider these cases as having atypical
features or atypical evolutions of BCECTS? I support the
latter interpretation, but even if not accepted as such, the
therapeutic decision should follow the criteria presented
in the following text.
How to Prevent the Atypical
Evolutions of BCECTS
According to present evidence, the risk of atypical evo-
lutions of BRE due to AEDs is low (Corda et al., 2001).

Nevertheless, atypical features on electroclinical grounds
(mainly EEG abnormalities and early age at onset) should
be considered as risk factors (Fejerman et al., 2000;
Kramer et al., 2002; Saltik et al., 2005). When these risks
are evident, the recommendations may be to avoid using
classic AEDs (phenobarbital, phenytoin, and carbamaze-
pine) and some of the new AEDs such as oxcarbazepine,
lamotrigine, topiramate, levetiracetam (Fejerman et al.,
2007b; Catania et al., 1999; Montenegro & Guerreiro,
2002; Caraballo, personal communication), and starting
treatment with sulthiame or benzodiazepines. In patients
without risks, a good alternative to discuss with parents is
not to use any medication.
Conclusions
Excluding patients with atypical evolution to CSWSS
and severe language or behavior impairments, which
account for approximately 5% of patients with BRE in our
tertiary epilepsy center for children, we have to be cau-
tious with conclusions. Even when a significant number of
children with BRE show some learning difficulties, most
of them are able to attend normal schools. Besides, in most
of the mentioned studies, the impact of AEDs and their
blood levels on neuropsychological findings were not
studied (Aicardi, 2007; Fejerman et al., 2007a). Further-
more, external factors such as parental emotion may have
a major impact on quality of life (Connolly et al., 2006).
Acknowledgment
I confirm that I have read the journal’s position on issues involved in
ethical publication and affirm that this report is consistent with those
guidelines.
Disclosure: The author declares no conflicts of interest.
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