Leitthema
Z Epileptol 2019 · 32:135–140
https://doi.org/10.1007/s10309-018-0237-3
Published online: 8 January 2019
© Springer Medizin Verlag GmbH, ein Teil von
Springer Nature 2019
Introduction and terminology
Electrical status epilepticus in sleep
(ESES) is a subclinical electroencephalo-
graphic (EEG) pattern of sleep-induced
spikes and waves (SWs) with a fre-
quency of 1.5–3.5 Hz in children, oc-
cupying 85–100% of slow-wave sleep
([1]; . Fig. 1). When bilateral ESES is
associated with acquired cognitive or
behavioral problems, the child is diag-
nosed with ESES syndrome, of which
Landau–Kleffner syndrome (LKS) and
continuous spike and waves during slow-
wave sleep (CSWS) are the best known
[2–5]. Atypical cases, or ESES spectrum
variants, exist and involve children with
ESES but without developmental arrest
or regression [6] as well as patients with
cognitive decline or behavioral problems
but in whom the sleep-induced SWs do
not exceed the 85% of slow-wave sleep, or
are found only unilaterally (see . Fig. 2).
Aggravation of epileptiform activity
by sleep with spike wave index (SWI)
exceeding 50% but not reaching 85% are
now considered within the spectrum as
well [7]. For the spectrum of ESES syn-
drome abnormalities, see . Fig. 3.
The characteristic EEG pattern usu-
ally resolves spontaneously after puberty.
However, the neurodevelopmental out-
come is often disappointing, with per-
manent deficits in the large majority of
children [8].
The major goal of therapy in ESES
syndrome is to prevent or reduce as-
sociated neurodevelopmental disorders.
However, in the current literature, treat-
ment efficacy is often judged by seizure
control and EEG recordings. Normal-
Floor E. Jansen · Bart van den Munckhof
Department of Child Neurology, Brain Center Rudolf Magnus, University Medical Center Utrecht, Utrecht,
The Netherlands
Treatment in epileptic
encephalopathy with ESES and
Landau–Kleffner syndrome
ization of the EEG pattern of ESES has
been found to improve neurodevelop-
ment but whether this is truly necessary
is unknown [9].
In the past 50 years many treatment
regimens have beenadministered toESES
syndrome patients with variable success
and many questions regarding accurate
treatment remain. There is no general
agreement on whether immunomodu-
lation, benzodiazepines, or antiepileptic
drugs (AEDs) should be used as a first-
choice medication. Nor is there con-
sensus on the duration of treatment after
which cognitive improvement may be ex-
pected, or how long treatment needs to be
continued after improvement is achieved
to prevent relapses. In clinical practice,
there are two major challenges: (1) to es-
tablish the optimal treatment strategy in
patients with ESES syndrome, and (2) to
identify the patients who will benefit most
from therapy, including atypical cases.
The current treatment options in
ESES, as in other types of epilepsies, in-
clude “routine” AEDs, benzodiazepines,
immune modulation therapy, includ-
ing corticosteroids, ketogenic diet, and
surgical treatment. We reviewed the
literature on these different treatment
modalities.
Antiepileptic drugs
Patients are often started on antiepileptic
drugs because the majority present with
clinical seizures and ESES/CSWS is only
diagnosed on average 1–2 years later. The
most widely used AEDs in these patients
include levetiracetam, sodium valproate,
ethosuximide, and sultiame. Their effi-
cacy with respect to seizure control is
likely to be as good as in other epilepsy
syndromes, but effects on neurodevelop-
ment are often disappointing or tempo-
rary. The most frequently applied as first-
line AED in ESES treatment today is lev-
etiracetam [10–14]. Capovilla et al. [10]
observed efficacy in two of three chil-
dren with focal structural epilepsy and
ESES. Neuropsychological or behavioral
improvement was reported in nine of
12 patients and EEG improvement in
seven of 12 children after treatment with
levetiracetam by Aeby et al. [11]. How-
ever, four patients relapsed.
Relapses were also seen by Wang et al.
[12] in two of five initial responders out
of the six patients treated. In a study
by Atkins and Nikanorova [13], leve-
tiracetam was found more effective in
patients with structural etiology com-
pared with unknown or genetic etiol-
ogy. Theydemonstrated a long-lasting re-
sponse (more than 12 months) in eight of
20 patients and three showed a temporary
response (6–12 months). Six of the nine
patients with seizures became seizure free
when levetiracetam was added. Inutsuka
et al. found that treatment with val-
proate alone or in combination with etho-
suximide resulted in long-lasting seizure
control and partial recovery of cognitive
functions in ten out of 15 patients (67%;
[15]). Liukkonen et al. [16] also re-
ported on the efficacy of a combination
of valproate and ethosuximide. Others
observed insufficient improvement after
treatment with valproate [5, 10, 16].
Sultiame has been reported as effective
in small series [17, 18], but may be less
easily accessible in some countries.
Zeitschrift für Epileptologie 2 · 2019 135
 Leitthema
Fig. 1 8 Typical ESES-EEG pattern with SWI >85%
Recently, topiramate was reported as
a successful treatment option in ESES.
At 3 months, 16 of 21 patients showed
clinical and behavioral improvement,
with long-lasting effect in ten patients
[19]. Acetazolamide, not routinely used
in ESES by many, was reported to yield
subjective clinical improvement in five
of six children in whom up to 12 previ-
ous treatments had failed, when studied
retrospectively [20].
Exacerbation with AEDs
Carbamazepine, oxcarbazepine, vigaba-
trin, gabapentin, and phenobarbital can
have negative effects on neuropsycholog-
ical outcome and EEG pattern [21].
High-dosage benzodiazepines have
demonstrated efficacy in reducing epilep-
tiform activity, especially in the short
term. Today, the first-choice benzo-
diazepine is clobazam, which showed
sustained effect in combination with
other AEDs [22]. Treatment with short
136 Zeitschrift für Epileptologie 2 · 2019
cycles ofhigh-dose diazepam (1–3 weeks)
can lead to transient remission, but re-
lapses necessitate repeated cycles [17,
23]. Three of eight patients treated with
oral diazepam 0.75–1 mg/kg/day for
3 weeks, relapsed within 6 months. In
a series by Sanchez Fernandez et al. [24],
29 patients with ESES were treated with
1 mg/kg/day oral diazepam and showed
a decrease in the mean SWI from 77 to
41%. In an earlier report, diazepam was
also administered successfully rectally
in 15 patients [24]. However, negative
results were also observed; valproate and
benzodiazepines were not effective in
nine out of ten patients, and three pa-
tients experienced an adverse behavioral
reaction [5].
Immunomodulating treatment
Because immune-mediated mechanisms
are suspected in epileptogenesis, intra-
venous immunoglobulins (IVIGs) and
corticosteroids have been used as alter-
natives to AEDs and benzodiazepines in
the treatment of refractory seizures.
Corticosteroids
When reviewing the literature, it is noted
that corticosteroids likely offer better and
more long-lasting efficacy than conven-
tional AEDs or benzodiazepines [25].
However, because of the, sometimes
considerable, adverse effects, steroids are
infrequently used as first-choice treat-
ment. Different steroid modalities and
schemes, including oral prednisolone,
adrenocorticotropic hormone (ACTH),
intravenous methylprednisolone, and
oral dexamethasone, have been reported
over the years. Successful long-term
treatment of ACTH and corticosteroids
was observed by Lerman et al.in four
patients [26]. Tsuru et al. reported
on the efficacy of repeated pulses with
intravenous methylprednisolone in two
patients [27]; when continuing treatment
with oral prednisolone, improved lan-

guage performance was maintained. An-
other nine out of ten patients manifested
significant long-lasting improvement in
language, cognition, and behavior after
6 months of treatment with oral pred-
nisolone at 1 mg/kg/day [28]. Only few
and reversible side effects were noted.
Kramer et al. [17] found steroids to show
short-term efficacy in 11 of 17 patients,
but of those 33% eventually relapsed and
22% became steroid dependent. Buzatu
combined the experience of corticos-
teroid treatment in ESES of LKS of two
centers in a large retrospective cohort
of 44 patients [29]. All but two patients
were administered daily hydrocortisone,
in a scheme lasting up to 21 months.
Although relapses occurred (14 of 34),
20 patients (45.4%) were found to be
long-term responders, despite initial
positive response in 34 of 44 patients
(77.2%), with normalization of the EEG
in 21 patients. Treatment efficacy was
related to higher intelligence quotient/
developmental quotient (IQ/DQ) and
shorter CSWS duration. Dexametha-
sone, more frequently used in neuro-
oncology, was studied by Haberlandt et al.
in a number of epilepsy syndromes, in-
cluding epileptic encephalopathy with
ESES [30]. The efficacy of dexametha-
sone was confirmed by Chen et al.
[31], who reported that seven out of
15 patients responded to treatment, al-
though relapses were seen in four when
dexamethasone was discontinued after
1 month. Recently, the electroclini-
cal spectrum and treatment efficacy in
ESES were reported in a large cohort
of 44 Turkish patients [32]. The au-
thors did not observe corticosteroid to
be superior over administration of two
AEDs. When evaluating 147 treatments
in 47 ESES patients, van den Munckhof
et al. [33] found steroid treatment to be
most successful in improving cognitive
performance. Further, improvement
of daily functioning after treatment was
strongly associated with an SWI decrease.
van den Munckhof also pooled the data
of 575 patients in whom 950 treatments
were administered. In total, 81% of
ESES cases showed subjective cognitive
or EEG improvement after treatment
with steroids, rendering steroids more
effective than benzodiazepines and other
AEDs, with efficacy rates of 68 and 49%,
respectively [27].
Intravenous immunoglobins
It been proposed that IVIG, an agent
used in the treatment of various cen-
tral nervous system inflammatory neu-
rological conditions, could block the pre-
sumptive early inflammation-mediated
increased neuronal excitability that leads
to epileptogenesis. In theory, IVIG could
be as or more efficacious than other anti-
inflammatory agents, without the sig-
nificant side effects that accompany the
continuous administration of high-dose
steroids. Over the years treatment reg-
imens with IVIG in LKS or ESES syn-
drome have been published with vari-
able results [17, 34–36]. Arts et al. [34]
performed a prospective study with six
children. Only one of the six patients
showed a positive response to IVIG. An-
other study reported a long-term notable
improvement in only two of 11 patients
[35]. Kramer found three of nine patients
responded to the IVIG, one of these also
used clobazam [17].
Ketogenic diet
The ketogenic diet (KD) is a high fat,
low to adequate protein, low carbohy-
drate diet used for refractory childhood
epilepsy. The data on ketogenic diet
in CSWS/ESES treatment are limited.
Bergqvist et al. [37] were the first to de-
scribe three patients with improvement
of their language performance, behavior,
and seizure frequency after introduction
of the ketogenic diet. A recent review by
Kelley and Kossoff [38] reported results
of the KD in 38 children from six pub-
lications. A mixed outcome was found
with over 50% seizure reduction in 41%
of the patients, cognitive improvement
in 45%, and EEG improvement in 53%
with EEG normalization in only 9%. This
review included one series of 13 patients
in whom the diet was administered in
combination with steroids and only one
of 13 was able to discontinue steroid
treatment [39].
Abstract · Zusammenfassung
Z Epileptol 2019 · 32:135–140
https://doi.org/10.1007/s10309-018-0237-3
© Springer Medizin Verlag GmbH, ein Teil
von Springer Nature 2019
F. E. Jansen · B. van den Munckhof
Treatment in epileptic
encephalopathy with ESES
and Landau–Kleffner
syndrome
Abstract
Epileptic encephalopathy with electrical
status epilepticus in sleep (ESES) is an
age-related syndrome with acquired
neurodevelopmental deficits in relation to
epilepsy with many seizure types. Early and
accurate treatment is aimed at prevention
or reduction of associated cognitive deficits
and, to a lesser extent, control seizures.
Evidence for an optimal treatment strategy
is still lacking. Here, we review the current
literature on treatment efficacy in patients
with epileptic encephalopathy with ESES.
Keywords
Epileptic encephalopathy with ESES ·
Cognition · Treatment efficacy ·
Corticosteroids · Review
Behandlung bei epileptischer
Enzephalopathie mit ESES
und Landau-Kleffner Syndrom
Zusammenfassung
Die epileptische Enzephalopathie mit
elektrischem Status epilepticus im Schlaf
(ESES) ist ein altersabhängiges Syndrom,
bei dem es zu erworbenen neurologischen
Entwicklungsdefiziten im Zusammenhang
mit der Epilepsie und ihren vielen
Anfallstypen kommt. Eine frühe und gezielte
Therapie wird angestrebt, um die mit
dem Syndrom einhergehenden kognitiven
Defizite zu verhindern oder zu reduzieren
und – in geringerem Maße – Anfälle zu
beherrschen. Belege für eine optimale
Behandlungsstrategie stehen noch aus. In
der vorliegenden Arbeit eine Auswertung
der aktuellen Literatur in Bezug auf die
Therapiewirksamkeit bei Patienten mit
epileptischer Enzephalopathie und ESES
dargestellt.
Schlüsselwörter
Epileptische Enzephalopathie mit ESES ·
Kognition · Therapiewirksamkeit ·
Kortikosteroide · Übersicht
Zeitschrift für Epileptologie 2 · 2019 137
 Leitthema

Fig. 2 8 Atypical ESES-EEG pattern with only unilateral SWs

Epilepsy surgery
Regression

The most effective treatment of ESES/

CSWS in patients with strictly or pre-
Typical ESES
dominantly unilateral congenital or
syndrome early-acquired brain lesion is epilepsy
surgery, even in the presence of abundant
Cognition

generalized or bilateral epileptiform dis-

Arrest

Atypical ESES charges on EEG [40]. Different surgical

syndrome techniques such as hemispherectomy,
lobar or multilobar resection, result in
No ESES improvement in 93% of patients. Eti-
Delay

Fig. 3 9 Spectrum
syndrome ology includes hemispheric or lobar
of electrical status
epilepticus in sleep polymicrogyria, or perinatal vascular
Unilateral EA Bilateral SWI 50-85% Bilateral EA (ESES) syndrome. with or without thalamic injury. Bag-
SWI 50-85% or SWI >85% EA epileptiform ac- giatta et al. observed immediate effec-
Unilateral SWI >85% tivity, EEGelectroen- tiveness of the surgical treatment in two
cephalography,
EEG SWI spike wave
patients who underwent hemisphero-
index tomy for ESES/CSWS regarding both
epileptic evolution (no more seizures)
and EEG abnormalities [41]. Postoper-
ative resolution of generalized interictal
discharges and ESES as well as improve-
ment in neuropsychological testing was

138 Zeitschrift für Epileptologie 2 · 2019

Table 1 Treatment recommendations for
clinical practice
Consider epilepsy surgery when the patient
has a unilateral structural abnormality that
causes the epileptic encephalopathy
Consider steroids when there is a history of
regression
In refractory patients (refractory to
clobazam or steroids) consider ketogenic
diet or IVIGs, or sultiame (in cases with
atypical benign focal epilepsy of childhood
[ABFEC]), ethosuximide (in cases with thala-
mic injury)
found in all eight patients reported by
Loddenkemper et al., although two of
eight patients continued to have rare
seizures [42]. The positive effects of
early surgery on neurodevelopmental
outcome was also observed by Fournier-
Del Castillo et al. [43]. They reported
on two cases with highly significant
improvement in DQ/IQ. The most re-
cent report of Jeong et al. [44] showed
complete seizure control in all nine chil-
dren with ESES and resolution of sleep-
aggravated SWs in six of six patients
in whom postoperative EEG recordings
were available, after hemispherotomy.
Although often considered a palliative
treatment, callosotomy was found to be
effective in some children with ESES/
CSWS. Four of nine children showed
reduced SWs propagation or source
strength, six showed a significant im-
provement in seizure control, and three
a significant improvement in IQ [44].
Multiple subpial transection (MST) was
first reported by Morrell as an effective
treatment in non-structural LKS cases
with language regression [45]. This
surgical procedure has been suggested
to also have positive effects in ESES/
CSWS patients with global cognitive
impairment and behavioral problems
[46]. However, since then, few have
confirmed these positive findings, and
whether MST is truly effective is still
under debate.
Conclusion
Epileptic encephalopathy and ESES is
a devastating, under-recognized, age-
related epilepsy syndrome. Early and
accurate treatment are necessary to pre-
vent severe neurodevelopmental deficits.
Here, we reviewed the variety of available
treatments. Many of the studies sum-
marized here are small or retrospective,
and have not used quantitative neu-
ropsychological outcome data. Based
on the current literature, we cannot
make strong recommendations, but
a first choice for surgery in patients
with unilateral structural abnormalities
seems justified (. Table 1). In none-
structural cases, benzodiazepines, es-
pecially clobazam, and corticosteroids
may be effective. An added value of one
over the other has not been established
with certainty but is currently being
examined in a European multicenter
randomized controlled trial, the RES-
CUE ESES (Randomized European trial
of Steroids versus Clobazam Usage for
Encephalopathy with Electrical Status
Epilepticus in Sleep) study.
Practical conclusions
4 Currently, there is no evidence
for rational treatments in patients
with epileptic encephalopathy with
electrical status epilepticus in sleep
(ESES).
4 Prompt and accurate treatment
is mandatory to prevent further
neurodevelopmental decline. Ob-
servational research has shown that
standards antiepileptic drugs AEDs
have limited efficacy.
4 Surgery is the most effective treat-
ment in children with unilateral
structural abnormalities.
4 Both clobazam and steroids can
be found effective in patients with
epileptic encephalopathy with ESES.
Corresponding address
Dr. Floor E. Jansen
Department of Child Neurology, Brain Center
Rudolf Magnus, University Medical Center
Utrecht
Postbus 85090, 3508 AB Utrecht, The
Netherlands
F.E.Jansen@umcutrecht.nl
Compliance with ethical
guidelines
Conflict of interest. F.E. Jansen and B. van den
Munckhof declare that they have no competing inter-
ests.
This article does not contain any studies with human
participants or animals performed by any of the au-
thors.
References
1. Patry G, Lyagoubi S, Subclinical TCA (1971)
“electrical status epilepticus” induced by sleep in
children. A clinical and electroencephalographic
study of six cases. Arch Neurol 24(3):242–252 (Mar)
2. Nickels K, Wirrell E (2008) Electrical status epilep-
ticus in sleep. SeminPediatrNeurol 15(2):50–60
(Jun)
3. Smith MC (1997) Landau-Kleffner syndrome
and continuous spikes and waves during slow
slee. Lippenscot Raven publishers. Epilepsy:
a comprehensive textbook. Engel J. and Pedley TA,
Philadelphia, pp 2367–2377
4. Tassinari CA, Rudolf G, Billard C, Bureau M (2005)
Electrical status Epilepticus during slow sleep
(ESES or CSWS) including acquired epileptic
aphasia (Landau-Kleffner syndrome). John
LibbeyEutotext ltd. Epilepsy Syndromes in infancy,
Childhood and Adolescence. Roger J, Bureau M,
Dravet C, Genton P, and Tassinari CA, London, pp
295–314
5. Scholtes FB, Hendriks MP, Renier WO (2005)
Cognitive deterioration and electrical status
epilepticus during slow sleep. Epilepsy Behav
6(2):167–173 (Mar)
6. Ballaban-Gil K, Tuchman R (2000) Epilepsy and
epileptiform EEG: association with autism and
language disorders. Ment Retard DevDisabil Res.
Rev 6(4):300–308
7. Scheltens-de Boer M (2009) Guidelines for EEG in
encephalopathy related to ESES/CSWS in children.
Scheltens-de Boer M. Epilepsia. 50(Suppl 7):13–17
8. Rossi PG, Parmeggiani A, Posar A, Scaduto MC,
Chiodo S, Vatti G (1999) Landau-Kleffner syndrome
(LKS): long-term follow-up and links with electrical
status epilepticus during sleep (ESES). Brain Dev
21(2):90–98 (Mar)
9. Saltik S, Uluduz D, Cokar O, Demirbilek V, Dervent A
(2005)AclinicalandEEGstudyonidiopathicpartial
epilepsies with evolution into ESES spectrum
disorders. Epilepsia 46(4):524–533 (Apr)
10. Capovilla G, Beccaria F, Cagdas S, Montagnini
A, Segala R, Paganelli D (2004) Efficacy of
levetiracetam in pharmacoresistant continuous
spikes and waves during slow sleep. Acta Neurol
Scand 110:144–147
11. Aeby A, Poznanski N, Verheulpen D, Wetzburger
C, Van Bogaert P (2005) Levetiracetam efficacy in
epileptic syndromes with continuous spikes and
waves during slow sleep: experience in 12 cases.
Epilepsia 46:1937–1942
12. Wang S-B, Weng W-C, Fan P-C, Lee W-T (2008)
Levetiracetam in continuous spike waves during
slow-wave sleep syndrome. Pediatr Neurol
39:85–90
13. Atkins M, Nikanorova M (2011) A prospective study
of levetiracetam efficacy in epileptic syndromes
with continuous spike-waves during slow sleep.
Seizure 20:635–639
Zeitschrift für Epileptologie 2 · 2019 139

14. Larsson PG, Bakke KA, Bjørnæs H et al (2012)
The effect of levetiracetam on focal nocturnal
epileptiform activity during sleep—A placebo-
controlled double-blind cross-over study. Epilepsy
Beh 24:44–48
15. Inutsuka M, Kobayashi K, Oka M, Hattori J, Ohtsuka
Y (2006) Treatment of epilepsy with electrical
status epilepticus during slow sleep and its related
disorders. Brain Dev 28:281–286
16. Liukkonen E, Kantola-Sorsa E, Paetau R, Gaily
E, Peltola M, Granström M-L (2010) Long-term
outcome of 32 children with encephalopathy with
status epilepticus during sleep, or ESES syndrome.
Epilepsia 51:2023–2032
17. Kramer U, Sagi L, Goldberg-Stern H, Zelnik N,
Nissenkorn A, Ben-Zeev B (2009) Clinical spectrum
and medical treatment of children with electrical
status epilepticus in sleep (ESES). Epilepsia
50:1517–1524
18. Wirrell E, Ho AW-C, Hamiwka L (2006) Sultiame
therapy for continuous spike and wave in slow-
wave sleep. Pediatr Neurol 35:204–208
19. Vrielynck P, Marique P, Ghariani S, Lienard F,
de Borchgrave V, van Rijckevorsel K, Bonnier
C (2017) Topiramate in childhood epileptic
encephalopathy with continuous spike-waves
during sleep: A retrospective study of 21 cases. Eur
J Paediatr Neurol 21(2):305–311
20. FineAL,WirrellEC,Wong-KisielLC,NickelsKC(2015)
Acetazolamide for electrical status epilepticus in
slow-wave sleep. Epilepsia 56(9):e134–8 (Sep)
21. Larrieu Jl, Lagueny A, Ferrer X, Jullien J (1986)
Épilepsie avec décharges continues au cours du
sommeil lent. Guérison sous clobazam. Rev EEG
Nuerophysiol Clin 16:383–394
22. Lerman P (1986) Seizures induced or aggravated
by anticonvulsants. Epilepsia. Nov 27(6):706–710
23. Sanchez Fernandez I, Hadjiloizou S, Eksioglu Y
(2012) Short-term response of sleep-potentiated
spiking to high-dose diazepam in electric sta-
tus epilepticus during sleep. Pediatr Neurol
46:312–318
24. De Negri M, Baglietto MG, Battaglia FM, Gaggero
R, Pessagno A, Recanati L (1995) Treatment of
electrical status epilepticus by short diazepam
(DZP) cycles after DZP rectal bolus test. Brain Dev.
Sep 17(5):330–333
25. van den Munckhof B, van Dee V, Sagi L, Caraballo
RH, Veggiotti P, Liukkonen E, Loddenkemper T,
Sánchez Fernández I, Buzatu M, Bulteau C, Braun
KP, Jansen FE (2015) Treatment of electrical status
epilepticus in sleep: A pooled analysis of 575 cases.
Epilepsia 56(11):1738–1746 (Nov)
26. Lerman P, Lerman-Sagie T, Kivity S (1991) Effect of
early corticosteroid therapy for Landau-Kleffner
syndrome. Dev Med Child Neurol 33:257–260
27. Tsuru T, Mori M, Mizuguchi M, Momoi MY (2000)
Effects of high-dose intravenous corticosteroid
therapy in Landau-Kleffner syndrome. Pediatr
Neurol 22:145–147
28. Sinclair DB, Snyder TJ (2005) Corticosteroids for
the treatment of Landau-kleffner syndrome and
continuous spike-wave discharge during sleep.
Pediatr Neurol 32:300–306
29. Buzato M, Bulteau C, Altuzarra C, Dulac O, Van
Bogaert P (2009) Corticosteroids as treatment
of epileptic syndromes with continuous spike-
waves during slow-wave sleep. Epilepsia 50(Suppl
7):68–72
30. Haberlandt E, Weger C, Sigl SB, Rauchenzauner
M, Scholl-Bürgi S, Rostásy K, Karall D (2010)
Adrenocorticotropic hormone versus pulsatile
dexamethasone in the treatment of infantile
epilepsy syndromes. Pediatr Neurol 42:21–27
140 Zeitschrift für Epileptologie 2 · 2019
Lesetipp
31. Chen J, Cai F, Jiang L, Hu Y, Feng C (2016) A Genetik in Neurologie und
prospective study of dexamethasone therapy Psychiatrie
in refractory epileptic encephalopathy with
continuous spike-and-wave during sleep. Epilepsy
Behav 55:1–5 (Feb) Nicht nur Neuro-
32. Gencpinar P, Dundar NO (2016) Tekgul H Electrical logen und Psych-
status epilepticus in sleep (ESES)/continuous
iater werden in
spikes and waves during slow sleep (CSWS)
syndromeinchildren: Anelectroclinicalevaluation Zukunft zuneh-
according to the EEG patterns. Epilepsy Behav mend von den
61:107–111 (Aug)
Erkenntnissen
33. van den Munckhof B, Alderweireld C, Davelaar S,
van Teeseling HC, Nikolakopoulos S, Braun KPJ, der Neurogene-
Jansen FE (2018) Treatment of electrical status tik profitieren.
epilepticusinsleep: ClinicalandEEGcharacteristics
Durch die außer-
and response to 147 treatments in 47 patients. Eur
J Paediatr Neurol 22(1):64–71 (Jan) ordentlichen Fortschritte der genetischen
34. Arts WF, Aarsen FK, Scheltens-de Boer M, Catsman- Forschung sind zahllose Krankheiten, de-
Berrevoets CE (2009) Landau-Kleffner syndrome
ren Ursachen vor der Identifizierung ihrer
and CSWS syndrome: treatment with intravenous
immunoglobulins. Epilepsia 5((Suppl 7):55–58 genetischen Grundlagen bisher unbe-
35. Fayad MN, Choueiri R, Mikati M (1997) Landau- kannt waren, mittlerweile einer raschen
Kleffner syndrome: consistent response to
Diagnose zugänglich. Der Umgang mit
repeated intravenous gamma-globulin doses:
a case report. Epilepsia 38:489–494 genetischen Befunden wird daher für Sie
36. Mikati MA, Saab R, Fayad MN, Choueiri RN im klinischen Alltag zur Routine werden.
(2002) Efficacy of intravenous immunoglobulin
Das Leitthema in Der Nervenarzt 02/2019
in Landau-Kleffner syndrome. Pediatr Neurol
26:298–300 bietet Ihnen Einblicke in grundlegende
37. Bergqvist AGC, Brooks-Kayal AR (1997) Ketogenic Aspekte neuropsychiatrischer Genetik und
diet in the treatment of acquired epileptic aphasia.
hilft Ihnen, Ergebnisse der Forschung und
Ann Neurol 42:504
38. Kelley SA, Kossoff EH (2016) How effective is the insbesondere einzelne Erfolgsmeldungen
ketogenic diet for electrical status epilepticus of im Gesamtkontext einzuordnen.
sleep? Epilepsy Res 127:339–343 (Nov)
39. Ville D, Chiron C, Laschet J, Dulac O (2015)
The ketogenic diet can be used successfully in 4 Verständnis der molekularen Ursachen
combination with corticosteroids for epileptic psychiatrischer Störungen
encephalopathies. Epilepsy Behav 48:61–65
40. WyllieE, Lachhwani DK, GuptaA, ChirlaA, CosmoG,
WorleySetal(2007)Successfulsurgeryforepilepsy 4 Umwelt und Epigenetik
due to early brain lesions despite generalized EEG
findings. Baillieres Clin Neurol 69:389–397
4 Ethische Aspekte
41. Battaglia D, Veggiotti P, Lettori D, Tamburrini G,
Tartaglione T, Graziano A et al (2009) Functional
hemispherectomy in children with epilepsy and 4 Mitochondriale Erkrankungen
CSWS due to unilateral early brain injury including
thalamus: sudden recovery of CSWS. Epilepsy Res
87:290–298 4 Exomdiagnostik in der Neurologie
42. Loddenkemper T, Cosmo G, Kotagal P, Haut J,
Klaas P, Gupta A et al (2009) Epilepsy surgery in
Suchen Sie noch mehr zum Thema?
children with electrical status epilepticus in sleep.
Neurosurgery 64:328–337 Mit e.Med – den maßgeschneiderten Fort-
43. Fournier-Del Castillo C, García-Fernández M, Pérez- bildungsabos von Springer Medizin – ha-
Jiménez M-Á, Ugalde-Canitrot A, Alvarez-Linera J,
ben Sie Zugriff auf alle Inhalte von Sprin-
Ruiz-Falcó M-L et al (2014) Encephalopathy with
electricalstatusepilepticusduringsleep: Cognitive gerMedizin.de. Sie können schnell und
andexecutiveimprovementafterepilepsysurgery. komfortabel in den für Sie relevanten Zeit-
Seizure 23:240–243
schriften recherchieren und auf alle Inhalte
44. Peltola M, Liukkonen E, Granström M, Paetau R,
Kantola-Sorsa E, Valanne L et al (2011) The effect of im Volltext zugreifen.
surgery in encephalopathy with status epilepticus
during sleep. Epilepsia 52:602–609
Weitere Infos zu e.Med finden Sie auf
45. Morrell F, Whisler WW, Smith MC, Hoeppner TJ,
de Toledo-Morrell L, Pierre-Louis SJ, Kanner AM, springermedizin.de unter „Abos“
Buelow JM, Ristanovic R, Bergen D et al (1995)
Landau-Kleffner syndrome. Treatment with
subpial intracortical transection. Brain 118(Pt
6):1529–1546 (Dec)
46. Nass R, Gross A, Wisoff J, Devinsky O (1999)
Outcome of multiple subpial transections for
autistic epileptiform regression. Pediatr Neurol
21:464–470