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Current thumbnail: The syndrome of Landau-Kleffner or acquired epileptic
aphasia was first reported in 1957 and is recognized now as an Epileptic
encephalopathy. Many authors state that there is a spectrum including
this syndrome and the syndrome of Continuous spikes and waves during
slow sleep, and data supporting this concept are presented. The most
important information included in this update refers to a very interesting
new pathogenetic interpretation regarding seizures and language dysfunction
in the Landau-Kleffner syndrome.
Historical note and nomenclature
In 1957 Landau and Kleffner described 6 children with a "syndrome of acquired
aphasia with convulsive disorder” (Landau and Kleffner 1957). The language
disorder was later recognized as being different from typical childhood aphasia,
which is usually expressive. Instead, the aphasia of the Landau-Kleffner syndrome
is an auditory verbal agnosia (Rapin et al 1977). The language disorder was
initially considered to be a seizure manifestation because epileptiform activity
from one or both temporal lobes is generally present on EEG recordings. Seizures
are not a constant feature, and when present, they may precede or follow the
onset of the language disorder.
Mantovani and Landau followed the 6 original cases plus 4 others and found
that the outcome was variable: 5 of the children had recovered good language
function as adults (Mantovani and Landau 1980). Their hypothesis that outcome
correlated with seizure frequency was not substantiated by subsequent reports
(Deonna et al 1977; Holmes et al 1981).
The 1989 International League Against Epilepsy placed this syndrome
under the classification of "epilepsies and syndromes undetermined as to whether
they are focal or generalized" (Anonymous 1989). In the same category,
the syndrome of continuous spikes-and-waves during slow sleep was included
as a definite entity. However, in recent years many common features between
these two syndromes have been recognized, and there are questions as to whether
they are two distinct entities or subclasses of a single syndrome (Deonna and
Roulet 1995; Hirsch et al 1995; De Negri 1997; Smith 1998; Rossi et al 1999;
Tassinari et al 2002; Smith and Hoeppner 2003). Furthermore, the mechanism
of bilateral secondary synchronies after an initial functional spike focus
seems to be the basis of language impairment and represent both for Landau-Kleffner
syndrome and continuous spikes-and-waves during slow sleep syndrome--an explanation
for the inclusion of these syndromes among the epileptic encephalopathies as
proposed by ILAE´s Task Force on Classification (Fejerman et al 2000;
Engel 2001). Clinical Manifestations
Acquired aphasia is the more prominent feature, since seizures are present
in only 70% to 80% of the patients (Beaumanoir 1985; 1992; Paquier et al
1992). Age of onset ranges from 3 to 8 years, and boys are more frequently
affected than girls (Beaumanoir 1985; Panayiotopoulos 2005). The onset of
aphasia is often insidious and progressive with spontaneous improvements
and aggravations in its course. The most common feature is verbal auditory
agnosia, which is the reason that in many cases the first diagnosis is hearing
loss (Rapin et al 1977). Agnosia may extend to familiar noises. In some cases
onset may be abrupt, and different types of aphasia may occur (Soprano et
al 1994). Variable time may elapse between the loss of the ability to understand
language and the expressive aphasia. In general, it is stated that patients
previously had been normal in both psychomotor and language development.
However, detailed history of language characteristics show that 9 of 12 patients
have had previous features of developmental dysphasia (Soprano et al 1994).
Rarely, stuttering may be the presenting feature (Tutuncuoglu et al 2002),
although it is difficult to differentiate pure stuttering from the repetitive
nature of the impaired speech in early stages of acquired aphasia.
Neuropsychological
and behavioral disturbances have been reported, and there are cases in
which the overlapping between Landau-Kleffner syndrome and the syndrome
of continuous spikes-and-waves during slow sleep is more prominent on
clinical grounds. Alternating courses with behavioral disturbances and
acquired aphasia has been described in children within the mentioned
spectrum (Fejerman et al 2000).
Nevertheless, the most frequent findings are hyperkinesia and
excitability. In fact, it is striking that children with such a severe handicap
in understanding language only present psychotic or autistic features when
aphasia appears in early ages (Deonna et al 1982; Fejerman and Medina 1986;
Deonna 1991; Klein et al 2000). In the context of a more global autistic
regression, some young children show language regression with epileptiform
EEGs, but it is controversial to state that these children are part of
an extended Landau-Kleffner spectrum (McVicar and Shinnar 2004).
Seizures are present in 70% to 80% of patients
and may appear before or after onset of aphasia (Deonna et al 1977). The
most common types of seizures are: eyelid myoclonia, eye blinking, atypical
absences, head drops and atonic fits in upper limbs, automatisms, and
occasionally, partial motor seizures with secondary generalization. Clinical Vignette
No information was provided by the author.
Etiology
Isolated cases of Landau-Kleffner syndrome were apparently associated with
overt cerebral pathology (eg, acute inflammatory disease, arteritis, cysticercosis,
tumors, and arachnoid cyst) (Cole et al 1988; Otero et al 1989; Pascual-Castroviejo
et al 1992; Nass et al 1993; Perniola et al 1993; De Volder et al 1994).
Pathological study in a surgical series of 14 patients with Landau-Kleffner
syndrome yielded a variety of abnormalities (Smith et al 1992). Perisylvian
polymicrogyria has recently been reported in one case (Huppke et al 2005).
Nevertheless, the language symptomatology is interpreted as a dysfunctional
disorder associated with the bilateral EEG discharges and deafferentation
of temporal cortex (Landau and Kleffner 1957; Gordon 1990; 1997; Beaumanoir
1992). The reported cases of benign childhood epilepsy with centrotemporal
spikes evolving into Landau-Kleffner syndrome and the prompt response to
antiepileptic drugs in some of the patients also points to a secondary bilateral
synchrony type of phenomenon (Fejerman et al 2000; Dalla Bernardina et al
2002). In a recent prospective study of 16 children with idiopathic partial
epilepsies with evolution into electrital status epilepticus during sleep
spectrum disorders, 1 of the cases developed a typical Landau-Kleffner syndrome
and the symptomatology and electrital status epilepticus during sleep in
the EEG persisted for several years (Saltik et al. 2005).
Pathogenesis and pathophysiology
Waking EEG usually shows brief bursts of temporal or temporo-occipital spike
and wave discharges, either symmetrical or asymmetrical. In fact, the most
typical EEG findings appear during slow sleep as continuous 1.5 Hz to 5 Hz
spike and wave discharges, which may be seen in approximately 85% of the
record (Beaumanoir 1992; Deonna and Roulet 1995; De Negri 1997; Smith 1998).
It has been stated that most of the cases have a unilateral primary epileptogenic
region (Morrell et al 1995).
Several authors have pointed to a relationship
between the epileptic abnormalities of Landau-Kleffner syndrome and the
benign epilepsies of childhood (Dulac et al 1983; Cole et al 1988; Deonna
and Roulet 1995). Moreover, some patients were reported with a history
of typical benign childhood epilepsy with centrotemporal spikes previous
to the onset of Landau-Kleffner syndrome (Fejerman and Medina 1986; Cole
et al 1988; Deonna et al 1993; Fejerman et al 2000; Smith and Hoeppner
2003). More recently, atypical evolutions of a few patients with initially
typical early-onset benign childhood occipital epilepsy (Panayiotopoulos
type) syndrome included language impairment secondary to the continuous
spike-and-wave discharges during slow sleep registered in these children
(Caraballo et al 2001; Ferrie et al 2002).
The correlation between the frequency and severity
of EEG abnormalities and the degree of language disturbances has been questioned,
although in active periods of aphasia, epileptiform activities in the EEG
are more prominent. Again, at present, all patients with Landau-Kleffner
syndrome show, at some time during its course, the sleep-EEG pattern
of continuous spike and wave discharges (Tassinari et al 2002). In recent
long-term follow-up of series of patients with Landau-Kleffner syndrome,
a strict correlation between length of continuous spike and wave discharges
and persistence of language impairment was found (Robinson et al 2001;
Veggiotti et al 2002).
It has been suggested
that the EEG discharges and seizures are manifestations of underlying abnormalities
of the cortex in the speech areas, rather than the cause of the aphasia (Holmes
et al 1981). A specific hypothesis has been proposed for pathophysiology
of Landau-Kleffner syndrome: a persistent paroxysmal activity during
the age-dependent period of synaptogenesis might strengthen synaptic
contacts that should have degenerated to allow neuronal aggregates mediate
normal behavior. This reinforcement of inappropriate contacts in the
developing temporo-parietal cortex produces a permanent language dysfunction
when paroxysmal activities are bilateral (Morrell et al 1995; Smith 1998).
Therefore, early treatment would be the only way to prevent persistent
aphasia. This mechanism also explains why eventual normalization of the
EEG is not necessarily paralleled with improvement of aphasia. Finally,
epileptiform activities in EEG were also seen in a percentage of patients
with developmental dysphasia, adding difficulties to the interpretation
of physiopathogenic mechanisms in Landau-Kleffner syndrome (Maccario
et al 1982; Echenne et al 1992; Picard et al 1998).
Magnetoencephalography was used to localize the source of epileptiform
activity in children with Landau-Kleffner syndrome. A thorough study of 4
right-handed Landau-Kleffner syndrome patients including video-EEG and
magnetoencephalography was performed as presurgical evaluation 3 to 6
years after the first language deterioration. Conclusion was that the
intrasylvian cortex is a likely pacemaker of epileptic discharges in
Landau-Kleffner syndrome and that magnetoencephalography provides useful
presurgical information of the cortical spike dynamics (Paetau et al
1999). Magnetoencephalography was also performed in 19 patients with
suspected diagnosis of Landau-Kleffner syndrome. Thirteen of the 19 children
had perisylvian magnetoencephalography spikes, which were bilateral in 10
and unilateral in 3 of the children. These results suggest that magnetoencephalography
might help to obviate the need for invasive video-EEG recordings when surgery
is being considered for patients with this condition (Sobel et al 2000).
Abnormal
auditory evoked potentials have been reported in several patients with Landau-Kleffner
syndrome. Some authors found normal brainstem auditory evoked potentials
and clearly abnormal long latency evoked potentials, specifically the
P300 (Fejerman and Medina 1986). Others reported abnormal brainstem auditory
evoked potentials and middle-latency evoked potentials (Isnard et al
1995). Five children having recovered from Landau-Kleffner syndrome were
compared to controls using early, middle latency, and late auditory evoked
potentials. Unilateral voltage reduction of late auditory evoked potentials
over the temporal areas previously involved by epileptic discharges was
found, suggesting a permanent dysfunction in the associative auditory
cortex (Wioland et al 2001). All studies support the hypothesis of a
deficit in the activation of the auditory cortical areas.
A new pathogenetic
interpretation was provided studying 4 children with Landau-Kleffner
syndrome compared to 4 controls. MRI volumetric analysis was performed
focusing on various neocortical regions and emphasizing the auditory
association cortex. Age of onset of the Landau-Kleffner syndrome in
the patients ranged between 3 and 4.5 years, whereas the MRI volumetry
was done at ages 5 to 6.5 years. Greater than 25% volume reduction in
both superior temporal areas was demonstrated only after comparison with
controls, and it was worse in the side with more epileptiform activity
in 2 of the children. The authors were not able to determine if volume
changes existed before language regression and, thus, could not distinguish
tissue loss from decreased growth or dysgenesis. Besides, 2 patients
received steroid treatment before the MRI study (Takeoka et al 2004).
The main question arising is whether the reduction of temporal cortical
volume is cause or effect of the Landau-Kleffner syndrome. Incidentally,
1 of the 2 authors who described originally the syndrome commented the
just mentioned findings and envisioned the following sequence of events:
- Early on temporal lobe epileptiform activity produces the positive
symptom of clinical seizures and the negative symptom of language
dysfunction;
- Excitotoxicity of unrelieved epileptic activity causes focal cortical
atrophy;
- cortical atrophy prevents language recovery despite disappearance
of epileptiform activity (Bourgeois and Landau 2004).
If the
findings of volume reduction are ratified, longitudinal volumetric
analysis might become a useful tool to evaluate evolution and treatment
effects in patients with Landau Kleffner syndrome. Epidemiology
The incidence of Landau-Kleffner syndrome is unknown. At least 198 cases were
reported by 1992 (Beaumanoir 1992). Even when Landau-Kleffner syndrome shares
many clinical and EEG features with the syndrome of continuous spikes-and-waves
during slow sleep, acquired aphasia is a more frequent finding than the psychiatric
disturbances described in association with continuous spike-wave activity
in slow sleep.
Prevention
There are no definite guidelines for prevention of idiopathic epilepsies. However,
we can do something to prevent the encephalopathic course which is seen in
a small percentage of children with benign focal epilepsies in childhood
(Fejerman et al 2000). As is emphasized in the Management section, the adequate
selection of initial antiepileptic drugs treatment may be the best way to
avoid the development of Landau-Kleffner syndrome.
Differential Diagnosis
Progressive degenerative neurologic disorders usually affect language as part
of the neuropsychological deterioration. Structural lesions in the dominant
hemisphere of children less than 5 years of age do not impair language development
because that function is served by the nondominant hemisphere. Therefore,
receptive or expressive aphasia is unusual in young children unless they
have a bitemporal lobe dysfunction. Therefore, acute or subacute aphasia
in children aged 2 to 8 years, without unilateral acquired paresis or encephalitic
symptoms, is most probably due to Landau-Kleffner syndrome.
The boundaries
between Landau-Kleffner syndrome and the syndrome of epilepsy with continuous
spikes-and-waves during slow wave sleep have been widely discussed (Hirsch
et al 1990; 1995; Tassinari 1995; Smith 1998; Tassinari et al 2002). This
type of EEG has also been found in several children with benign childhood
epilepsy with centrotemporal spikes, particularly in the reported cases with
clinical and EEG status lasting weeks (Fejerman and Di Blasi 1987; Roulet
et al 1989; Fejerman 1996; 2000). Intermediate cases between status of
benign childhood epilepsy with centrotemporal spikes and Landau-Kleffner
syndrome are also occasionally seen (Shafrir and Prensky 1995; Fejerman
1996).
In early-onset
cases of Landau-Kleffner syndrome, differential diagnosis with developmental
dysphasia associated with EEG discharges could be difficult. The same applies
to children with autistic features, regression, and epileptiform EEGs (Tuchman
and Rapin 1997). Elective mutism can be readily discarded on clinical grounds
and with EEG. Nevertheless, many cases of Landau-Kleffner syndrome are initially
diagnosed as psychosis or severe emotional disturbance on account of their
recent inability to understand spoken language, their hyperkinesia, and their
anxiety. In a significant number of cases, diagnosis is delayed because an
extensive workup for deafness is undertaken. Diagnostic Workup
Neurologic examination is normal, and special care should be given to recognition
of aphasia, mainly the onset, as auditory verbal agnosia. Neuropsychological
evaluation is fundamental to determine the nature of the language disorder
and level of intelligence (Deonna 1997). In many cases children keep their
ability to write and to communicate through nonverbal means (Gordon 1990;
Soprano et al 1994).
EEG shows a pattern of bilateral symmetrical or asymmetrical
multifocal spikes and spike-waves most frequently located in the temporal
and parieto-occipital regions. Sleep enhances the EEG paroxysms up to the
level of exhibiting spike-wave discharges in more than 85% of slow wave
sleep (Beaumanoir 1985).
Except in
occasional cases associated with cerebral structural pathologies, MRI is
normal (Gordon 1990).
Functional imaging with PET and SPECT have repeatedly
revealed unilateral or bilateral disturbances involving the temporal lobe
(Maquet et al 1990; Mouridsen et al 1993; Guerreiro et al 1996; Da Silva
et al 1997). As mentioned before, MRI volumetric analysis of superior
temporal areas may yield clues to understand pathogenesis and evaluate
evolution. Prognosis and Complications
The outcome of Landau-Kleffner syndrome varies. The seizures are usually controlled
with antiepileptic drugs, and EEG abnormalities disappear after a few years.
The
language disorder, however, may never resolve in almost half of the patients
(Mantovani and Landau 1980; Paquier et al 1992). Both improvement and aggravation
of aphasia have been reported (Deonna 1991). In many cases, correlation was
found between increase in EEG discharges and aphasia, or even between abnormalities
in the P300 wave during evoked potential studies and aphasia (Fejerman and
Medina 1986). The presence or absence of seizures, as well as their frequency,
has no correlation with the outcome of language deficiency (Gordon 1990).
A residual impairment in verbal short-term memory is frequent. Brain
activation during immediate serial recall of lists of 4 words, compared
to single word repetition, using positron emission tomography were measured
in three Landau-Kleffner syndrome patients after recovery and in 14 healthy
controls. The patients had shown abnormally increased or decreased glucose
metabolism in left or right superior temporal gyrus at different stages
during the active phase of the disease. At the time of the study, the
patients were 6 years to 10 years from the active phase of the disease.
Results showed that two patients had impaired performance in verbal short-term
memory. The data suggest that impaired verbal short-term memory at late
outcome of Landau-Kleffner syndrome might be related to a persistent
decrease of activity in the areas involved in the epileptic focus during
the active phase (Majerus et al 2003).
Out of 10 children with
electrical status epilepticus during sleep and global or specific deterioration
with long-term follow-up, 3 had Landau-Kleffner syndrome and showed that
electrital status epilepticus during sleep persisted for 1 to 5 years
and language impairment was not modified by treatment with Valproic acid
or Benzodiazepines (Scholtes et al 2005).
Outcome at adulthood has been
recently reported in seven young adults, five who had continuous spike-and-wave
during slow-sleep syndrome and two with Landau-Kleffner syndrome in childhood.
The intellectual functions of the two patients with Landau-Kleffner syndrome
were normal, but their everyday lives were disrupted by severe, disabling
language disturbances. The authors emphasized the role of location
of interictal EEG focus and age of onset as prognostic factors (Praline
et al 2003). Dementia, or more precisely, long-term deterioration of
intellectual functions, is uncommon in Landau-Kleffner syndrome (Dugas
et al 1995). However, in early onset cases, neuropsychological impairment
is more severe (Deonna et al 1982; Bishop 1985). Management
Treatment with standard antiepileptic drugs such as phenytoin, phenobarbital,
and carbamazepine may be effective against seizures, but at present, this
treatment is not recommended because these drugs may worsen the EEG discharges
and neuropsychological deficit (Marescaux et al 1990). Instead, valproate,
ethosuximide, and the benzodiazepines can be effective and deserve a trial
before attempting treatments with higher risks. However, even valproate and
most of the new antiepileptic drugs have been shown to induce continuous
spike-wave discharges and worsening of clinical features in patients with
atypical evolutions of benign focal epilepsies including Landau-Kleffner
syndrome (Prats et al 1998; Fejerman et al 2000; Prats-Vinas 2002). Sulthiame
seems to be the oldest drug of choice according to recent reports (Lerman
and Lerman-Sagie 1995; Gross-Selbeck 1995; Wakai et al 1997; Doose et al
1998; Fejerman et al 2000; Huppke et al 2005). One case was treated with
levetiracetam with control of seizures and language improvement (Kossoff
et al 2003).
Treatment with high-dose corticosteroids was reported to yield
the best results, and prolonged chronic or intermittent therapy may be
necessary (Marescaux et al 1990; Lerman et al 1991; Tsuru et al 2000).
In a recent series of 8 patients with Landau-Kleffner syndrome and 2
with the syndrome of continuous spike-and-waves during slow sleep treated
with Prednisone 1 mg/Kg/day for at least 6 months, all but 1 showed significant
improvement in language, cognition, and behavior (Sinclair and Snyder
2005). In isolated cases, the use of intravenous immunoglobulins was
successful (Fayad et al 1997; Lagae et al 1998). In a recent report,
2 of 5 children with Landau-Kleffner syndrome receiving 2 g/kg of intravenous
gamma-globulin over 4 days showed excellent response; in both children
the severe language and EEG abnormalities completely resolved (Mikati
et al 2002).
Multiple
subpial transection of the cortex to abolish epileptic discharges was used
in a series of 14 children with acquired epileptic aphasia who had been unable
to use language to communicate for at least 2 years; sustained improvement
was obtained in 11 of them. According to the authors, success depends on
selection of cases having severe EEG abnormality that can be demonstrated
to be unilateral in origin despite a bilateral manifestation (Morrell
et al 1992; 1995). In a more recent small series, 5 children with Landau-Kleffner
syndrome aged 5.5 to 10 years underwent multiple subpial transection,
and behavior and seizure frequency improved dramatically. Improvement
in language also occurred in all children, although none of them reached
an age-appropriate level of language even when their electrical status
epilepticus during sleep was eliminated by the procedure (Irwin et al
2001).
More recently, six children with Landau-Kleffner syndrome were implanted
with the vagal nerve stimulation device and three of them apparently
showed improvement in quality of life (Park 2003). Pregnancy
Not applicable.
Anesthesia
Not applicable.
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ILAE.
ILAE Copyright Notice
Abbreviations
EEG:electroencephalogram
SPECT:single photon emission computed tomography
ICD Code
784.69
Synonyms
Acquired aphasia-epilepsy syndrome
Acquired aphasia with convulsive disorder
Acquired epileptic aphasia
Acquired epileptiform aphasia
Associated Disorders
Autism
Benign epilepsy of childhood
Continuous spikes and waves during slow sleep
Developmental aphasia
Major Keyword Descriptors
aphasia
epileptiform activity
language
multifocal spikes
mutism
seizures
sleep
slow sleep
speech regression
spike-wave discharges
verbal agnosia
Minor Keyword Descriptors
alternative communication
developmental delay
epilepsy
hyperactivity
language impairment
personality change
temper tantrums
Age of Presentation
02-05 years
06-12 years
Age of Typical Presentation
02-05 years
06-12 years
Population Group(s) Preferentially Affected
none selectively affected
Occupation Group(s) Preferentially Affected
none selectively affected
Sex
male>female, >2:1
male>female, >1:1
Family history
none
Heredity
none
Permuted topic, Synonyms, Variants
Landau-Kleffner syndrome
Kleffner syndrome, Landau-
aphasia with convulsive disorder, Acquired
convulsive disorder, Acquired aphasia with
epileptic aphasia, Acquired
epileptiform aphasia, Acquired
Related Topics
Developmental language disorder
Electrical status epilepticus during slow sleep
Epilepsy
Limbic status epilepticus (psychomotor status)
Parasomnias
Sleep disorders
Sleep disorders associated with epilepsy
Differential Diagnosis
epilepsy with continuous spikes-and-waves during slow wave sleep
benign childhood epilepsy with centrotemporal spikes
developmental dysphasia associated with EEG discharges
autism
regression
elective mutism
psychosis
severe emotional disturbance
deafness
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