Landaukleffner syndrome lks, first described in 1957 by neurologist william landau and speech pathologist frank kleffner, is a rare childhood disorder in. The abnormal eeg and the impairment of higher cognitive function developed and regressed together. Landaukleffner syndrome with continuous spikes and waves. Landau kleffner syndrome an overview sciencedirect topics. The eeg abnormality is markedly enhanced by sleep deprivation and in sleep. The speech and language skills may improve over time. See more ideas about epilepsy, epilepsy awareness and epilepsy seizure. Landaukleffner syndrome is a rare disorder that affects twice as many males as females. Landaukleffner syndrome and aphasia facts and information.
Spectral and historical topographic mapping of eeg was done on 2 siblings with landaukleffner syndrome. Eeg in landaukleffner syndrome pediatric neurology briefs. A relative decrease in perfusion was found in the left temporal cortex of all three patients and also in the left frontoparietal cortex of one patient with hyperkinetic. Oct 19, 2017 solomon ge, carson d, pavlakis s, et al. Lks affects the parts of the brain that control comprehension and speech brocas area and wernickes area. Tc99mhmpao spect was performed in three right handed children with lks. The landau kleffner syndrome is a rare disorder characterized by an acquired receptive and expressive aphasia and epileptic seizures. Magnetoencephalography in children with landaukleffner. Landau kleffner syndrome lks is a form of rare, childhood neurological disorder characterized by gradual or sudden inability to express language or understand it, also referred to as, aphasia, as well as an abnormal electroencephalogram eeg. Intracranial eeg monitoring in landau kleffner syndrome associated with left temporal lobe astrocytoma. Landaukleffner syndrome presents with infrequent seizures and with the severe. Even though an abnormal eeg reading is common in lks patients.
Landau kleffner syndrome lks is a rare childhood disorder characterized by. In these patients, the cognitive symptoms focus mainly on the language domain. It usually occurs in children between the ages of 3 and 9 and affects the area of the brain that controls speech and comprehension. Landau kleffner syndrome causes loss of ability to understand and use language, and it can cause seizures. Eeg examination during wakefulness and sleep is fundamental for diagnosis. Dec 11, 2018 a major feature of the landau kleffner syndrome lks is the gradual or sudden loss of the ability to understand and use spoken language. The clinical significance of this association is unclear. Secondary symptoms include psychomotor or behavioral disturbances and epilepsy with a favorable outcome for seizure control. In 1957, landau and kleffner reported the cases of six children with developmentally normal language function who then developed aphasia in association with a convulsive disorder. During sleep, discharges increased and bilateral spikewaves occurred more than 85% of the sleep period. Landaukleffner syndrome lks is a rare agerelated epileptic encephalopathy, characterized by a developmental regression in the area of language, and electroencephalogram eeg anomalies located mainly around the temporoparietal areas. Acquired epileptic aphasia the landau kleffner syndrome due to neurocysticercosis.
Landaukleffner syndrome definition of landaukleffner. Until a definite name for this pattern is decided on, here we will use the abbreviation of cswseses to define the typical eeg features. What is landaukleffner syndromecausessymptomstreatment. Landaukleffner syndrome lks also called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder is a rare childhood neurological syndrome. The language dysfunction may have an acute or insidiously progressive onset and may. Anticonvulsant medications readily control clinical seizures, which. Landau kleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. Landaukleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with continuous or almost continuous spike. A striking eeg abnormality often occurs during sleep, also called continuous spikes and waves during slow sleep or electrical status epilepticus during slowwave sleep. During sleep, the eeg pattern is dominated by spike and wave discharges.
School and boston childrens hospital, the program for quality and safety at. Landau kleffner syndrome lks is a rare childhood disorder characterized by the loss of language comprehension auditory verbal agnosia and verbal expression aphasia in association with severely abnormal electroencephalographic eeg findings during sleep and clinical seizures in most patients. Landaukleffner syndrome a neurologic condition primarily affecting young age 37 children, which is characterised by acquired aphasia and an abnormal eegspike and wave discharges and multifocal spikeswith lesions in brocas and wernickes areas, which control comprehension and speech. Landaukleffner syndrome lks, first described in 1957 by neurologist. Key points about landau kleffner syndrome in a child. Treatment of electrical status epilepticus in sleep. Landaukleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. Landaukleffner syndrome lks is a rare neurological disorder that causes a loss of language skills aphasia. Childrens hospital boston and its augmentative communication program. It can also be seen as early as 18 months of age or as late as 14 years. Despite its relatively low incidence and prevalence, the landau kleffner syndrome lks is a neurological disorder that, in the last decade, has generated a great degree of interest, coupled with significant controversy with respect to its diagnostic criteria and treatment. Sleep eeg shows increased activity with bilateral and. Children with landaukleffner syndrome have a characteristic eeg pattern.
These previously normal children had no evidence of expressive aphasia during their symptomatic periods. All children have abnormal eeg compatible with the diagnosis of epilepsy, however, only 70% have clinical seizures. Landau kleffner syndrome lks is a rare childhood disorder characterized by acquired aphasia with seizures and electroencephalogram eeg abnormalities. Landau kleffner syndrome is characterized by subacute onset of progressive aphasia in a child with previous ageappropriate language development. All children with lks have abnormal electrical brain waves that can be documented by an electroencephalogram eeg, a recording of the electric activity of the brain. Meg studies suggest that in more than 80% of lks patients, the bilateral epileptic discharges are generated in the auditory. Landaukleffner syndrome lks is a rare agerelated epileptic encephalopathy, characterized by a. The landaukleffner syndrome is sometimes associated with continuous spikewaves during slowwave sleep. The peak age at time of onset is between 3 and 8 years. Its main features are a loss of speech and language skills, with seizures and learning and behaviour problems. The initial presentation may be with progressive aphasia 40%, seizures or both.
Landaukleffner syndrome lks is a rare childhood disorder and is most often diagnosed in normally developing children who are between 3 and 7 years of age. Episodic epileptic verbal auditory agnosia in landau. This disorder impairs the ability of the child to understand instructions and be able to communicate to others. To report a case with landaukleffner syndrome lks presenting with unusual. A child with lks has a progressive loss of language abilities understanding and speaking after previously developing normally for their age. In contrast to other childhood epileptic encephalopathies, such as lennoxgastaut syndrome, landaukleffner syndrome is the result of agedependent epileptic activity that occurs primarily in deep sleep. Landau kleffner syndrome is characterized by a regression of speech skills after a period of normal speech development against the background of epileptic changes in the eeg and epileptic seizures. Know the causes, symptoms, treatment, prognosis and recovery of landau kleffner syndrome.
In additional to language regression, the diagnosis requires the presence of severely epileptiform activity on eeg, particularly during nonrem sleep. The most effective way of confirming lks is by obtaining overnight sleep eegs. Lks affects the portions of the childs brain that controls their speech and comprehension. In order to investigate differences in glucose metabolic patterns between awake and sleep states in two children with landaukleffner syndrome and continuous spikewaves during slowwave sleep, fluorodeoxyglucose positronemission. Landaukleffner syndrome lksalso called infantile acquired aphasia, acquired epileptic. Lks usually occurs in children between ages 3 and 7 and can affect the childs ability to speak, understand language or both. Identification and treatment of landaukleffner syndrome the.
The eeg in children with this epilepsy syndrome is abnormal, especially as they enter sleep. Landau kleffner syndrome lks is a condition that shows up during childhood, causing difficulties with speech and seizures. The condition belongs to a spectrum of related dis. Landau kleffner syndrome boston childrens hospital. Many children with lks also have seizures, episodes of uncontrolled body movement. Landau kleffner syndrome lks is a very rare epilepsy syndrome of childhood. Pdf landau kleffner syndrome lksacquired epileptic aphasia aea typically develops in.
Sep 21, 2007 landau kleffner syndrome lks is a childhood disorder. Aug 11, 2017 landau kleffner syndrome lks is diagnosed based on clinical features and the results of an electroencephalogram eeg. These clinical syndromes are referred to as epileptic encephalopathy with eses, eses syndrome, or continuous spikes and waves during sleep csws syndrome. Landau kleffner syndrome nord national organization for rare. The landau kleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957. Landau kleffner syndrome lks is a rare, childhood neurological disorder characterized by the sudden or gradual development of aphasia the inability to understand or express language and an abnormal electroencephalogram eeg. In this article, acquired epileptic aphasia is used as a synonym for landau kleffner syndrome lks. This pattern of continuous spikes and waves during slow sleep csws or electrical status epilepticus in sleep eses in patients with landau kleffner syndrome tends to be unilateral or clearly lateralized. Landau kleffner syndrome statpearls ncbi bookshelf. It is typical to see continuous bilateral spikeandwave in slow sleep at some time in the course of the disease, but this is not a prerequisite for the diagnosis. A major feature of lks is the gradual or sudden loss of the ability to understand and use spoken language.
An eeg is a recording of the electrical activity of the brain, and this can be completed when a child is asleep or awake. The conditions in the epilepsyaphasia spectrum, which all begin in childhood, include landaukleffner syndrome lks, epileptic encephalopathy with continuous spikeandwave during sleep syndrome ecsws, autosomal dominant rolandic epilepsy with speech dyspraxia adresd, intermediate epilepsyaphasia disorder iead, atypical childhood. Pdf landaukleffner syndromea rare experience researchgate. Continuous spikes and waves during sleep comprise the severest epileptic. Social skills training for a young adult with landaukleffner. On the basis of the presentation, eeg, and the normal mri, landau kleffner syndrome was diagnosed and the child was started on sodium valproate, prednisolone, and speech therapy. Landau kleffner syndrome what is landaukleffner syndrome. These children will have developed speech normally and then, suddenly or gradually lose language skills for no apparent reason. Affected siblings and discordant monozygotic twins have been reported rarely. Mar 27, 2019 landau kleffner syndrome lks is a rare, childhood neurological disorder characterized by the sudden or gradual development of aphasia the inability to understand or express language and an abnormal electroencephalogram eeg. Landau kleffner syndrome lks is an uncommon condition in which a child between the ages of 3 to 9 years loses the ability to speak and understand speech. In many children, the degree of language dysfunction correlates with the severity of the eeg abnormality, as in the original report by landau and kleffner 1.
Hirsch, e marescaux, c maquet, p metzlutz, mn kiesmann, m salmon, e et al. The syndrome has onset between the ages of 2 and 8 years peak 5 to 7 years, or rarely later. Landaukleffner syndrome lks is an uncommon condition in which a child between the ages of 3 to 9 years loses the ability to speak and understand speech. The literature contains several small series of patients and many case reports. Landaukleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions. Oct 19, 2017 in 1957, landau and kleffner initially described acquired epileptic aphasia and subsequently reluctantly agreed to the attachment of their names to the syndrome. Both eeg and meg are necessary for comprehensive spatial and temporal description of perisylvian epileptic networks in the landau. Kleffner syndrome lks, also known as acquired epileptic aphasia. Landaukleffner syndrome is characterized by acquired aphasia and paroxysmal, sleep activated eeg paroxysms predominating over the temporal or parietooccipital regions. Landaukleffner syndrome symptoms and treatment of landau. Landaukleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with continuous or almost continuous spikeandwave discharges during slow wave sleep. Children with lks lose the ability to talk and understand speech. Guidelines for eeg in encephalopathy related to esescsws.
Landau kleffner syndrome lks is a rare and underdiagnosed epilepsy syndrome. This has since come to be termed the landau kleffner syndrome lks. We report 2 pediatric patients who presented initially with seizures followed by subacute language regression characterized by a verbal auditory agnosia. Lks usually starts between 3 and 6 years and affects twice as many boys as girls. The clinical features of the elder sister were acquired aphasia developed at the age of 5, followed by convulsions a year later, and those of the younger brother were progressive ataxia, hemiparesis, urinary incontinence and convulsions at the age of 4 years and 10 months, followed by. It was difficult to differentiate the landaukleffner syndrome and epilepsy with continuous spikewaves during slow wave sleep. The landaukleffner syndrome is characterized by the sudden or gradual development of aphasia the inability to understand or express language and an abnormal electroencephalogram eeg.
The disorder is characterised by gradual or rapid loss of language in a previously normal child. Social skills training for a young adult with landau. When present, the seizures consist of absence seizures or tonicclonic episodes and occur more frequently during sleep. Continuous spike and waves during slowwave sleep syndrome. Kleffner syndrome is closely related to eses and is characterized by an acquired aphasia. Children must have an eeg during sleep for lks to be diagnosed. This has since come to be termed the landaukleffner syndrome lks.
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