Original Articles
Corticosteroids for the Treatment of Landau-
Kleffner Syndrome and Continuous Spike-
Wave Discharge During Sleep
D. Barry Sinclair, MD and Thomas J. Snyder, PhD
Landau-Kleffner syndrome and its variants such as con-
tinuous Spike-Wave Discharge during Sleep (CSWS) are
progressive epileptic encephalopathies of childhood. The
treatment of this unusual group of patients is controver-
sial. We describe our experience in treating patients with
Landau-Kleffner syndrome and CSWS with corticoste-
roids. The patients received Prednisone 1 mg/kg/day for 6
months, 1 year, then yearly. Follow-up was for 1-10 years
(mean 4 years). Ten patients, 3 females, 7 males were
studied. Age of onset ranged from 2 to 11 years (mean 7.5
years). Eight patients manifested Landau-Kleffner syn-
drome, and two had CSWS. Most patients had seizures
(8/10); however, two patients did not have clinical sei-
zures. MRI was normal in all patients. SPECT scan was
abnormal in four patients, normal in three, and not
available in three. All but one patient manifested signifi-
cant improvement in language, cognition, and behaviour,
which continued after the corticosteroid trial. Side effects
were few (4/10) and transient and consisted of weight gain
(2), behavioral change (1), and hypertension (1). Cortico-
steroids are a safe and effective treatment for patients
with Landau-Kleffner syndrome and CSWS. Most pa-
tients had improvement in language, cognition, and be-
haviour after treatment. Side effects are few and revers-
ible, and benefits appear long lasting. Corticosteroids
should be considered as a treatment option in children
with Landau-Kleffner syndrome and CSWS. © 2005
by Elsevier Inc. All rights reserved.
Sinclair DB, Snyder TJ. Corticosteroids for the treatment of
Landau-Kleffner syndrome and continuous spike-wave dis-
charge during sleep. Pediatr Neurol 2005;32:300-306.
From the Comprehensive Epilepsy Program, University Of Alberta,
Edmonton, Alberta, Canada.
300 PEDIATRIC NEUROLOGY Vol. 32 No. 5
doi:10.1016/j.pediatrneurol.2004.12.006 ● 0887-8994/05/$—see front matter
Introduction
Landau-Kleffner syndrome and its variants such as
continuous spike-wave discharge during sleep or electrical
status epilepticus during sleep are epileptic encephalopa-
thies of childhood. Landau-Kleffner syndrome is an ac-
quired epileptic aphasia presenting as progressive loss of
speech in a previously well child with an abnormal and
usually continuously epileptic electroencephalogram (with
or without seizures) [1-3]. Continuous spike-wave discharge
during sleep is similar to Landau-Kleffner syndrome, al-
though language is preserved in continuous spike-wave
discharge during sleep and the patients demonstrate deterio-
ration in cognition or behavior with an epileptic electroen-
cephalogram, rather than speech or language problems [4,5].
The treatment of this unusual group of patients is contro-
versial and includes antiepileptic drugs, surgery, intrave-
nous immunoglobulin, and corticosteroids [6]. The present
report describes our experience in treating patients with
Landau-Kleffner syndrome and its variants with cortico-
steroids.
Methods
Patients were diagnosed with Landau-Kleffner syndrome or continuous
spike-wave discharge during sleep after language regression (Landau-
Kleffner syndrome) or cognitive/behavioral deterioration with intact lan-
guage (continuous spike-wave discharge during sleep) with the characteristic
epileptic electroencephalogram (Fig 1) usually with continuous spike-wave
discharge during sleep. Patients were then admitted to our pediatric epilepsy
monitoring unit for 24-hour continuous video electroencephalographic mon-
itoring, magnetic resonance imaging, single-photon emission computed
tomography scan (interictal), speech/language assessments, and neuropsy-
chological testing. Neuropsychological testing included tests of intellectual
functioning (Wechsler Intelligence Scale for Children–Third Edition;
Wechsler Preschool and Primary Scale of Intelligence–Revised), tests of
Communications should be addressed to:
Dr. Sinclair; Director, Division of Pediatric Neurology; 2C3 Walter
Mackenzie Health Sciences Center; University of Alberta;
Edmonton, AB T6G 2R7; Canada.
Received August 3, 2004; accepted December 28, 2004.
© 2005 by Elsevier Inc. All rights reserved.
Figure 1. Initial electroencephalogram demonstrating continuous spike-wave discharge during sleep (Patient 8).
attention (attention span and sustained attention), and motor skills (tapping
speed, coordination, perceptual-motor speed). Extensive language assess-
ment was performed in some patients examining general knowledge,
expressive vocabulary, word comparison, and word reading. In addition,
memory and learning for pictures, design stories, and word lists were tested
and behavior examined at both home and school. All patients were taking
antiepileptic drugs, either valproic acid or carbamazepine. These medica-
tions were not changed during the course of the trial. After confirmation of
the diagnosis, the patients received prednisone 1 mg/kg/day for 6 months.
The patients were then reassessed at 6 months, 1 year, then yearly.
Reassessment post-therapy included a clinic visit, repeat electroencephalo-
gram, and speech, language, and neuropsychological testing. Follow-up time
was for 1-10 years (mean 4 years).
Results
Ten patients, 3 females and 7 males, were studied (Table
1). Age of onset ranged from 2 to 11 years (mean 7.5 years).
Eight patients manifested Landau-Kleffner syndrome, and
two patients had continuous spike-wave discharge during
sleep. Most patients had seizures (8/10); however, two
patients did not have clinical seizures, only an abnormal and
epileptic electroencephalogram. The electroencephalography
overall was abnormal in all 10 patients. Abnormalities
included focal epileptic abnormalities in two, continuously
generalized epileptic discharge during sleep in seven, and one
patient had both focal and generalized epileptic discharge
during sleep. The magnetic resonance imaging was normal in
all patients. The single-photon emission computed tomogra-
phy scan (interictal) was abnormal in five patients, normal in
three, and not available in two due to technical problems. The
abnormal single-photon emission computed tomographic
scan results revealed a focal abnormality in the temporal lobe
in the language dominant hemisphere. All but one patient had
significant improvement in language cognition and behavior
on post-treatment, speech/language, and neuropsychological
reevaluations. One patient (#1), although manifesting signif-
icant improvement, never regained normal language skills,
although she managed to complete high school and is now
employed. We believe the long period of time (several years)
between onset of her symptoms and diagnosis and treatment
may be responsible for this outcome. The single patient (#5)
who failed to demonstrate improvement in language skills
was found to have improvement in motor speed and percep-
tual-motor speed. This improvement in language function
observed in the patients in this study persisted long after the
steroids had been discontinued, and no patient has relapsed to
date. Side effects were few and transient (4/10) consisting of
weight gain (2), worsening behavior (1), and hypertension (1).
Neuropsychology
Ten children were included in this study. Five had
neuropsychological assessments before and after treatment
with steroids, and five were assessed pretreatment only
with no formal follow-up testing. These assessments were
primarily made for clinical purposes, i.e., treatment and
Sinclair and Snyder: Treatment of Landau-Kleffner Syndrome 301
Table 1. Patient information

Patient Age/Sex Diagnosis Seizures EEG MRI

1 11 yr female LKS Complex partial Focal epileptic lt. temp. lobe Normal

2 2 yr male LKS Absence CSWS Normal

3 7 yr male LKS 0 Epileptic lt. parietal Normal

4 4 yr female LKS Absence CSWS Normal

5 11 yr male LKS gen. tonic-clonic CSWS Normal

6 9 yr female LKS Absence CSWS Normal

7 7 yr male LKS Absence CSWS Normal

8 6 yr male LKS gen. tonic-clonic Epileptic rt. temp., CSWS Normal

9 8 yr male LKS Absence, gen. tonic-clonic CSWS Normal

10 10 yr male CSWS 0 sz. CSWS Normal

Abbreviations:
abn. ⫽ Abnormal n/a ⫽ Not available
CSWS ⫽ Continuous spike-wave discharge of sleep rt. ⫽ Right
EEG ⫽ Electroencephalography SPECT ⫽ Single-photon emission computed tomography
gen. ⫽ Generalized sz. ⫽ Seizures
LKS ⫽ Landau-Kleffner syndrome temp. ⫽ Temporal
lt. ⫽ Left wt. ⫽ Weight
MRI ⫽ Magnetic resonance imaging

educational planning, and consequently varied from case
to case according to the age of the child, additional
assessments conducted, e.g., speech pathology, extent of
verbal communication, and the child’s cooperation and
behavior. Because of this variability, comprehensive
group data were limited and statistical analyses were
confined to a small number of tests. Nonetheless, there is
merit in reporting the results for a subset of relatively
homogeneous children to determine if pulsing with ste-
roids resulted in meaningful short-term changes in behav-
ior, cognition including language/communication, and
quality of life. This report presents details on four of the
eight relatively homogeneous children who met the Inter-
national Classification of Diseases, Tenth Revision diag-
nostic criteria for Landau-Kleffner syndrome and were
assessed before and immediately after treatment with
corticosteroids. These children had normal nonverbal in-
telligence, relatively normal language development dis-
rupted by rapid language regression, and paroxysmal
electroencephalographic abnormalities. Age of onset, du-
ration of electroencephalographic abnormality before
treatment, and demographic information are listed in Table
1, along with seizure type, antiepileptic drug treatment,
age at time of corticosteroid treatment, and neuroimaging
results. One male (#6) who had pre-steroid and post-
steroid neuropsychological assessments was not included
because he did not have normal nonverbal intelligence.
Two patients (#1, #7) had more than one post-treatment
assessment, and both their immediate and long-term out-
comes are therefore reported.
Ages of children at the time of initial neuropsycholog-
ical assessment ranged from 5 to 11 years (mean 8.0
years). Three of the four children had mild speech/
language delays before the occurrence of profound lan-
guage regression and the diagnosis of Landau-Kleffner
syndrome. Time between probable onset of Landau-Kleff
ner syndrome and the diagnosis and treatment ranged from
⬍1 year to ⱖ7 years (Patient). All assessments were
conducted in hospital except for Patient (#8), who was
assessed pretreatment in the community. Each assessment
included the Child Behavior Checklist, the age-appropriate
Wechsler Intelligence Scale (Wechsler Preschool and
Primary Scale of Intelligence–Revised; Wechsler Intelli-
gence Scale for Children–Third Edition, Revised), the
Grooved Pegboard Test, and Design Memory from the
Wide Range Assessment of Memory and Learning. The
Child Behavior Checklist was completed by mothers and
is a well-established measure of behavior problems that
includes global scores (Total Problems, Internalizing
Problems, Externalizing Problems) and domain scores.
The Grooved Pegboard Test is a measure of fine-motor
coordination. Design memory requires a child to repro-
duce four different configurations (designs) of simple
figures such as dots, lines, and rectangles immediately
after viewing each design for 10 seconds. The academic
skills of the three older children were measured with the
Wide Range Achievement Test (Revised/3). Standard
T-scores (mean 50, S.D. 10) from these tests were grouped
into six domains of performance for statistical analysis:
Attention (Child Behavior Checklist), Behavior (Child

302 PEDIATRIC NEUROLOGY Vol. 32 No. 5

Table 1. Patient information

SPECT Treatment Outcome Side Effects Follow-up

abn. lt. temp. lobe Carbamazepine Significant improvement wt. gain 10 yr

language
abn. lt. temp. lobe Ethosuximide, valproic acid, Significant improvement 0 7 yr
carbamazepine language and behavior
Normal Carbamazepine Significant improvement 0 5 yr
language and behavior
abn. lt. frontal Valproic acid Significant improvement wt. gain, edema 3 yr
language
Normal Ethosuximide, carbamazepine, Some improvement 0 3 yr
valproic acid language and behavior
abn. lt. temp. Carbamazepine, valproic acid Significant improvement wt. gain, mood 2 yr
behavior and language change
Normal Carbamazepine, valproic acid, Significant improvement 0 2 yr
frisium language and behavior
n/a Carbamazepine Significant improvement 0 3 yr
language
n/a Valproic acid Significant improvement in wt. gain, increased 1 yr
cognition and behavior blood pressure
n/a Valproic acid Significant improvement 1 yr
cognition and behavior

Behavior Checklist Total), Verbal Ability, Nonverbal
Ability, Manual Coordination, and Figural Memory. Al-
though other tests were administered to each child, these
varied according to age and clinical need and are not
reported. Pre-steroid and post-steroid domain scores for
each child are listed in Table 2.
Mean Wechsler Performance Intelligence Quotient be-
fore treatment was 105.8 (S.D. 19.6), a score at the 66th
percentile. Nonparametric statistical analysis of pretreat-
ment vs post-treatment test performance was done using
Friedman two-way analysis of variance by ranks because
of the few children included. This analysis indicated
significant post-treatment changes (chi-square 12.31, P ⫽
0.0009) that consisted of improvement and normalization
of attention, manual coordination, and total behavior.
Nonverbal ability and figural memory, which were gener-
ally normal before treatment, were unchanged. As a group,
there was no measurable change in verbal ability; how-
ever, longer-term follow-up of two patients demonstrated
prominent language improvement in one and persistent
global language impairment in the other (Table 3). These
patients differed for age of onset of Landau-Kleffner
syndrome (8 years vs ⬍6 years) and length of time
between onset and treatment (⬍1 year vs ⬎6 years). The
patient with marked language gains was administered a
dichotic listening test and manifested atypical speech
dominance. Despite improvement in some aspects of
language and in verbal memory, both patients manifested
persistent deficits of sentence repetition and picture nam-
ing.

Table 2. T scores (mean 50, S.D. 10) for domains of functioning for four children tested before and after treatment with prednisone

Assessment Verbal Spatial Social Preferred Nonpreferred

Patient Time Ability Ability Behavior Attention Problems Dexterity Dexterity Memory Read Math

#1 pre 19 66 65 67 81 63 45 47 14 33
#1 post 17 53 58 51 70 76 67 53 17 30
#9 pre 31 57 70 67 64 55 51 50 — —
#9 post 33 48 47 57 56 60 61 50 33 37
#7 pre 16 40 64 66 70 48 32 37 39 37
#7 post 37 53 47 51 62 51 50 53 41 47
#8 pre 22 57 47 73 60 30 31 40 — —
#8 post 45 57 40 51 56 57 59 43 — —

Sinclair and Snyder: Treatment of Landau-Kleffner Syndrome 303

Table 3. Long-term language and verbal memory outcome (>4 years) represented as T-scores* for two patients (#1 and #7) after
treatment with prednisone
Receptive
Patient Time PPVT Phonological Processing
#1 Pre 10 — 20
Post 20 — 30
#7 Pre 25 26
Post 38 40
*Normal T-score ⱖ40 relative to same-age females.
Abbreviations:
BNT ⫽ Boston Naming Test
PPVT ⫽ Peabody Picture Vocabulary Test
WJPC ⫽ Woodcock Johnson Passage Comprehension
WRAT ⫽ Wide Range Achievement Test
Discussion
Landau and Kleffner in 1957 described six children who
developed an acquired receptive aphasia in conjunction
with an epileptic disorder. The electroencephalograms
demonstrated severe paroxysmal changes, which appeared
to parallel the course of language impairment [1]. This
syndrome of acquired aphasia, seizures, and an epileptic
electroencephalogram has become known as the Landau-
Kleffner syndrome. The mechanism is thought to be a
verbal auditory agnosia or “word deafness” resulting from
a functional interference with the posterior temporal lan-
guage areas secondary to persistent epileptic discharges
[2]. The term acquired aphasia with epilepsy is also used
for this condition, which appears to be a progressive
dysfunction of auditory association cortex [3,4]. The
condition is often associated with changes in behavior.
Since that time there have been several reports with
clinical and electroencephalographic features well de-
scribed [5-9].
Seizures are observed in a majority of patients (70%)
and are often nocturnal. Seizures include simple partial
seizures, generalized tonic-clonic seizures, and atypical
absence seizures [10]. Treatment with standard antiepilep-
tic drugs, although successful in controlling clinical sei-
zures, does little to alter the progressive nature of this
devastating disease [3]. The patients in the present study
fit with this reported clinical picture. Seizures were ob-
served in most patients (8/10). One patient with Landau-
Kleffner syndrome (Patient 3) and one patient with con-
tinuous spike-wave discharge during sleep (Patient 10) did
not have clinical seizures. Seizure type included complex
partial seizures in one patient, absence seizures in four
patients, generalized tonic-clonic seizures in 10, and both
absence and generalized tonic-clonic seizures in one pa-
tient. All patients were tried on antiepileptic medication
with usually good seizure control but progression of their
language or cognitive problems.
The electroencephalogram in Landau-Kleffner syn-
drome is abnormal. A number of electroencephalo-
graphic patterns have been described, including bilat-
304 PEDIATRIC NEUROLOGY Vol. 32 No. 5
Language
Repetition Naming Fluency
Sentence BNT Categorical Phonological
⫺39 20 ⫺2
⫺23 46 47
20 — 48 25
23 19 49 36
eral temporal or temporal/parietal spikes, or generalized
spike/wave patterns, particularly in sleep. The striking
epileptic activity and continuous spike-wave pattern in
sleep draws parallel to continuous spike-wave discharge
during sleep or electrical status epilepticus during sleep,
a similar, often overlapping condition. The majority of
Landau-Kleffner syndrome patients go on to develop
continuous epileptic discharge during sleep [10], further
blurring the boundaries between these conditions. Both
focal (temporal or parietal) spikes and generalized
epileptic discharge were evident at the onset of the
syndrome. Focal discharge was observed in two, con-
tinuous spike-wave discharge during sleep was docu-
mented in the majority eight (Fig 1), and one patient
manifested both features. Of interest, in all patients with
a successful response to steroids there was normaliza-
tion of the electroencephalogram (Fig 2) usually by 3-6
months time. Clinical relapse was then associated with
a return of an epileptic electroencephalogram.
Neuroimaging is usually normal, both computed
tomography and magnetic resonance imaging [10-12],
suggesting the problem is not in brain anatomy. In all of
our patients, the magnetic resonance imaging was
normal. Single-photon emission computed tomography
scan, an indicator of focal brain dysfunction, was
abnormal (4/8). This result included focal interictal
abnormalities in the temporal lobe, left in four, right in
one, although this patient was right hemisphere domi-
nant. Because of the often infrequent and nocturnal
nature of the seizures in this study, ictal single-photon
emission computed tomography scan was not available.
Our findings are similar to those in the literature with
focal post-temporal abnormalities on interictal single-
photon emission computed tomography scans [13].
Several standard first-line antiepileptic drugs have
been used in Landau-Kleffner syndrome, including
ethosuximide (Zarontin), valproic acid (Depakene,
Epival), carbamazepine (Tegretol), and clobazam (Fri-
sium). Seizures were easily controlled in most of our
patients, although all patients continued to manifest
Table 3. Long-term language and verbal memory outcome (>4 years) represented as T-scores* for two patients (#1 and #7) after
treatment with prednisone

Verbal Learning/Recall Reading Spelling

Recall of 15-Word List Stories
Learning Recall Delay Recall Delay WRAT WJPC WRAT

⫺14 24 17 26 33 14 — —
41 53 51 35 41 18 34 —
19 13 7 20 20 39 37 46
51 63 57 40 45 50 44 45

language or cognitive/behavioral regression. This pat-
tern has been reported in other series [14,15]. Vigaba-
trin (Sabril) has also been reported to be effective [16],
but we have no personal experience of its use in
Landau-Kleffner syndrome.
There are reports of corticosteroids improving both clinical
features of the syndrome as well as normalization of electro-
encephalograms [17-19]. Studies have used both oral pred-
nisone [17] and high-dose intravenous corticosteroid pulsing
[18]. We share the experience of Lermen et al. [17] that early
treatment may be more successful. In the present series,
significant documented improvements were observed in at-
tention, behavior, and some aspects of language in all but one
patient. The results of neuropsychological assessments of
four children with “pure” Landau-Kleffner syndrome re-
vealed no deleterious treatment effects. Although long-term
follow-up of two of these children indicated that post-
treatment improvement persisted, the improvement in lan-
guage was neither global nor uniform. Individual differences
in age of onset, promptness of treatment, and premorbid
language functioning are likely associated with the variable
outcome for individual children, while the consistent findings
of unilateral extinction for dichotic listening indicates a
permanent change in temporal auditory cortex attributable to
the epileptic encephalopathy of Landau-Kleffner syndrome.
The experience reported by some authors [18] of transient
improvement on steroids followed by relapse during tapering
was not our experience. Our experience suggests that early
one-time corticosteroid pulse treatment may arrest the epi-
leptic encephalopathy underlying Landau-Kleffner syndrome

Figure 2. Repeat electroencephalogram after corticosteroid therapy. The patient is in remission and the epileptic discharges have completely
disappeared (Patient 8).

Sinclair and Snyder: Treatment of Landau-Kleffner Syndrome 305

and continuous spike-wave discharge during sleep. This
finding is similar to our experience in Lennox-Gastaut
syndrome and may be due to permanent changes in the
␥-aminobutyric acid receptor in the developing brain. In that
study, early, aggressive steroid treatment appeared to arrest
the condition, although this was not the case in older children
[20].
Other treatment options that have been explored more
recently have included the use of intravenous immunoglob-
ulin and surgery. In a few reports, intravenous immunoglob-
ulin has been successful in arresting Landau-Kleffner syn-
drome [21,22]. Lastly, epilepsy surgery, multiple subpial
resections, have been reported to be successful in this group
of patients. Morrell et al. [23] first reported a series of
Landau-Kleffner syndrome patients undergoing multiple sub-
pial transection as their seizures orginated from eloquent
cortex and not resectable by lesionectomy and other forms of
epilepsy surgery. The outcome was generally favorable,
although there has been only sporadic use of this treatment
option, numbers are small, and long-term outcome remains
uncertain at this time [24,25]. In addition, as the natural
history of this group of patients is not known, assessment of
treatment outcomes is difficult. Improvements over time
observed in some patients may reflect improvement or
spontaneous remission with maturation rather than effects
resulting from medical intervention, because the natural
history of these epileptic encephalopathies remains unclear.
Long-term outcome studies for patients with Landau-Klef-
fner syndrome and continuous spike-wave discharge during
sleep may help us understand the natural history of these
conditions and whether corticosteroid therapy remains a
preferred treatment.
Conclusion
Corticosteroids are a safe and effective treatment for
patients with Landau-Kleffner syndrome and continu-
ous spike-wave discharge during sleep. Many patients
manifest improvement in language, cognition, and be-
havior after treatment. Side effects are few and revers-
ible, and the benefits long lasting. Corticosteroids
should be considered as a treatment option in children
with Landau-Kleffner syndrome and continuous spike-
wave discharge during sleep.
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