Schools for the deaf were in the majority oralist in their approach, and the use of sl was forbidden lane, 1993. However, a number of candidate genes have been implicated, especially one involving glutamate metabolism known as grin2a. This disorder impairs the ability of the child to understand instructions and be able to communicate to others. Landaukleffner syndrome lks is a rare entity characterized by epilepsy and aphasia. There are many similarities between csws and lks, and the type of. Pdf landaukleffner syndrome lks is a childhood disorder. The epileptiform activity in these children is thought to result in a functional ablation of eloquent speech areas. Landaukleffner syndrome lks, or acquired epileptiform aphasia, is an epilepsy. The speech and language skills may improve over time. Many children with lks also have seizures, episodes of uncontrolled body movement. Landaukleffner syndrome lks is a childhood disorder. There are many similarities between csws and lks, and the type of neuropsychological. Specifically, the eeg typcally shows an increase to nearly continuous abnormal brain activity firing spikes. Landau kleffner syndrome lks is a rare and underdiagnosed epilepsy syndrome.
Landaukleffner syndrome definition of landaukleffner. Landaukleffner syndrome lks is a rare epileptic syndrome characterized by acquired aphasia with epilepsy. Auditory remediation for a patient with landaukleffner syndrome. The landaukleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957. Lks usually occurs in children between ages 3 and 7 and can affect the childs ability to speak, understand language or both. Secondary symptoms include psychomotor or behavioral disturbances and epilepsy with a favorable outcome for seizure control. Landaukleffner syndrome symptoms and causes boston. Education and landau kleffner syndrome great ormond street. Landaukleffner 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. Children with lks typically develop normally until signs and symptoms of the syndrome begin to develop between age 2 and 8 years. Feb 18, 2011 landau kleffner syndrome is an acquired aphasia secondary to an epileptic disturbance affecting a cortical area involved in verbal processing. It occurs in previously normal children, usually between three and seven years of age. To report a case with landaukleffner syndrome lks presenting with.
We describe 11 patients affected by landaukleffner syndrome lks with a mean. Landaukleffner syndrome symptoms and treatment of landau. Epileptic encephalopathy with continuous spikeandwave during sleep although both syndromes may have progressive cognitive, behavioral and psychiatric impairments, if acquired aphasia is the predominant feature then a diagnosis of landau kleffner syndrome is appropriate the presence of slow spikeandwave on eeg is not mandatory for diagnosis. The first lks cases were described by landau and kleffner 1957. A child with lks has a progressive loss of language abilities understanding and speaking after previously developing normally for their age. In rare cases, children with lks may also have behavioral problems, including outbursts of temper, hyperactivity or acting withdrawn. Landaukleffner 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. It is important for the prognosis to utilize antiepileptic treatment and possibly.
Landau kleffner syndrome lks is a very rare epilepsy syndrome of childhood. Experts do not know the cause of landau kleffner syndrome. Key points about landaukleffner syndrome in a child. The organization, which currently consists of approximately 100 members, is dedicated to providing information, support, and resources to families of. Affected children who have developed ageappropriate speech then experience language regression with verbal auditory agnosia, abnormal epileptiform activity, behavioural disturbances, and sometimes overt. Incredibles wannabe sidekick turned bad guy, from the incredibles 2004. The disorder is characterised by gradual or rapid loss of language in a previously normal child. 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.
With lks overnight seizures in the temporal lobes rob children of their speech. The aim of the study was to retrospectively analyze the electroclinical features, etiology, treatment, and prognosis of 29 patients with landau kleffner syndrome lks with a longterm followup. This syndrome is also characterized by an abnormal electroencephalogram eeg, especially during sleep. Characteristics the main feature of lks is the gradual or sudden loss of the ability to understand and use spoken language. Differential diagnoses include deafness, an acute behavioural or psychiatric dis. The first symptoms seem to appear before the age of 6, and it typically affects twice as many boys as girls. Affected siblings and discordant monozygotic twins have been reported rarely. 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. Treatment with anticonvulsant monotherapy is generally effective for seizure. In this article, acquired epileptic aphasia is used as a synonym for landaukleffner syndrome lks.
Epileptic encephalopathy with continuous spikeandwave during sleep although both syndromes may have progressive cognitive, behavioral and psychiatric impairments, if acquired aphasia is the predominant feature then a. Lks affects the portions of the childs brain that controls their speech and comprehension. Landaukleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. Landau kleffner syndrome nord national organization for rare. Landau kleffner syndrome an overview sciencedirect topics.
Landau kleffner 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. Landau and kleffner 1957 described 6 children with acquired aphasia and convulsive disorder. 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. Know the causes, symptoms, treatment, prognosis and recovery of landau kleffner syndrome. Oct 19, 2017 the landau kleffner syndrome or acquired aphasia with convulsive disorder. Seizures typically occur in the temporal lobe, or rolandic brain region, which affects speech and language, and electroencephalogram eeg characteristically shows centrotemporal spikewave discharges. Families of landau kleffner syndrome home facebook. Course and correlates with outcome robinson ro, baird g, robinson g, simonoff e developmental medicine and child neurology, 2001. A disorder with seizures starting in childhood in which the patient loses skills, such as speech, and develops behavior characteristic of autism a major feature of the landau kleffner syndrome lks is the gradual or sudden loss of the ability to understand and use spoken language. These children will have developed speech normally and then, suddenly or gradually lose language skills for no apparent reason. Syndrome pop culture when innocent heroworship goes unrequited, the consequences can be dire for both the admirer and the admired. Landau kleffner syndrome shares a common pathological mechanism with continuous spikewave of sleep csws, acquired epileptic opercular syndrome aeos and benign childhood epilepsy with centrotemporal spikes bects.
This leads to more expensive treatment than would otherwise be required. Friends of landau kleffner syndrome is a voluntary organization in the united kingdom uk that was established in 1989 by parents of children with landau kleffner syndrome lks. Hurley, phd university of south florida, tampa, florida landau kleffner syndrome lks is a rare, childhood neurological disorder characterized by a sudden or gradual. Children and young people with lks will benefit from a school placement that can support their individual pattern of abilities and needs. Landau kleffner syndrome boston childrens hospital. Aug 11, 2017 landau kleffner syndrome lks is a rare neurological syndrome characterized by the sudden or gradual development of aphasia the inability to understand or express language and recurrent seizures epilepsy. This has since come to be termed the landaukleffner syndrome lks. Landau kleffner syndrome lks is a rare entity characterized by epilepsy and aphasia. In additional to language regression, the diagnosis requires the presence of severely epileptiform activity on eeg, particularly during nonrem sleep. Lks usually starts between 3 and 6 years and affects twice as many boys as girls. 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. Magnetoencephalography in children with landaukleffner.
Despite its relatively low incidence and prevalence, the landaukleffner 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. Markedly improved in the walking posture after treatment with just left slightly. Landaukleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions. Landaukleffner syndrome is a rare disorder that affects twice as many males as females.
A disorder with seizures starting in childhood in which the patient loses skills, such as speech, and develops behavior characteristic of autism a major feature of the landaukleffner syndrome lks is the gradual or sudden loss of the ability to understand and use spoken language. Landaukleffner syndrome lks is a rare neurological disorder that causes a loss of language skills aphasia. The outlook for recovering language is poor when lks starts early in life or lasts for a long time without successful treatment. Auditory remediation for a patient with landaukleffner. In 1957, landau and kleffner initially described acquired epileptic aphasia and subsequently reluctantly agreed to the attachment of their names to the syndrome.
A case study annette hurley, phd louisiana state university health sciences center, new orleans, louisiana raymond m. Children affected with lks have developed normally until signs and symptoms begin between the ages of 2 and 8. Know the causes, symptoms, treatment, prognosis and recovery of landaukleffner syndrome. 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 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. You wont find landau kleffner variant in the medical diagnostic manuals though. Landaukleffner syndrome shares a common pathological mechanism with continuous spikewave of sleep csws, acquired epileptic opercular syndrome aeos and benign childhood epilepsy with centrotemporal spikes bects. A third approach involves combining the two above approaches. Landau kleffner syndrome lks is epileptiform aphasia acquired during childhood and occurring in children with previously normal language development. Thelandaukeffnersyndrome 387 diagnosis the diagnosis of landau kleffner syndrome depends largely on being aware that the condition exists, and its usual pattern of presentation. 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 article about landaukleffner. The peak age at time of onset is between 3 and 8 years. The landaukleffner syndrome or acquired aphasia with convulsive disorder.
There may be focal temporalparietal or diffuse slowing. Landau kleffner syndrome lks may be associated with complex language, and additional learning and behaviour difficulties. Many children with the disorder experience seizures which generally disappear by adulthood, the syndrome usually occurs in children between the ages of 5 and 7 years who develop normally. 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. It occurs in children usually between the ages of three and nine years and is characterised by loss of language skills and silent electrical seizures during sleep. Landaukleffner syndrome lksalso called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorderis a rare childhood neurological syndrome. Landau kleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions. In this article, acquired epileptic aphasia is used as a synonym for landau kleffner syndrome lks. Lennox gastaut syndrome the presence of generalized tonic seizures suggests lennox gastaut syndrome, focal motor seizures are rare in lennox gastaut syndrome. Characteristically, landaukleffner syndrome lks affects previously normal children who undergo a regression of receptive andor expressive language abilities. 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.
Landaukleffner syndrome lks is characterized by the sudden or gradual development of aphasia the inability to understand or express language. Landau kleffner syndrome nord national organization for. Case report a 5 year old boy developed episodes of sadness and crying for about one hour, immediately after the departure of his father for higher study. 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. Kleffner, coauthor of their initial paper and former director of the central institute for the deaf in saintlouis, missouri, u. It can also be seen as early as 18 months of age or as late as 14 years. 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. It also blindsides them with autisticlike symptoms and severe aggression. Key points about landau kleffner syndrome in a child. It is named after william landau and frank kleffner, who characterized it in 1957 with a diagnosis of six children. A major feature of lks is the gradual or sudden loss of the ability to understand and use spoken language.
Focal epilepsy with speech disorder is a childhoodonset seizure disorder with a highly variable phenotype. Landaukleffner syndrome lks is a rare childhood disorder and. The purpose of this study was to investigate the usefulness of magnetoencephalography meg for. It has been diagnosed primarily in young children, usually beginning at. The most common form of reflex epilepsy is photosensitive epilepsy, in which flashing lights trigger seizures. The international league against epilepsy ilae diagnostic manuals goal is to assist clinicians who look after people with epilepsy to diagnose the epilepsy syndrome and if possible the etiology of the epilepsy. What is landaukleffner syndromecausessymptomstreatment. This is a page of resource and comfort for families and friends affect by landau kleffner syndrome. Arriving at the correct epilepsy syndrome andor etiology allows better decisionmaking about treatment and improves patient care. The most commonly used treatments were clobazam, ethosuximide, sulthiame. They reported five children with a gradual regression of language abilities showing paroxysmal unilateral or bilateral spikeandwave discharges, which were characteristically localized. Landau kleffner syndrome what is landau kleffner syndrome. Common symptoms of landaukleffner syndrome lks include. Landaukleffner syndrome and aphasia facts and information.
The surgical treatment of lks was first discussed by frank morrell. Landaukleffner syndrome lks is a rare childhood epilepsy accompanied by acquired aphasia. Landaukleffner syndrome is an extremely rare form of seizures or epilepsy occurring in children normally between the ages of 3 and 9 years of age. Landaukleffner syndrome lks is a condition that shows up during childhood, causing difficulties with speech and seizures. Landaukleffner 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. It may be associated with convulsive seizures and additional difficulties with behaviour, social interaction, motor skills and learning. Landau kleffner syndrome epilepsy action australia. All children have abnormal eeg compatible with the diagnosis of epilepsy, however, only 70% have clinical seizures.
Landaukleffner syndrome causes loss of ability to understand and use language, and it can cause seizures. Auditory remediation for a patient with landau kleffner syndrome. Landau kleffner syndrome lks is characterized by the sudden or gradual development of aphasia the inability to understand or express language. The landaukleffner syndrome has been defined as an acquired epileptic aphasia or auditory agnosia.
Pdf changing perspectives on landaukleffner syndrome. Sep 21, 2007 landau kleffner syndrome lks is a childhood disorder. The aim of the study was to retrospectively analyze the electroclinical features, etiology, treatment, and prognosis of 29 patients with landaukleffner syndrome lks with a longterm followup. Landau kleffner syndrome causes loss of ability to understand and use language, and it can cause seizures. Reflex epilepsies are a group of epilepsy syndromes in which a certain trigger or stimulus brings on seizures. Landau kleffner syndrome lks is a rare childhood disorder characterized by the. The language dysfunction may have an acute or insidiously progressive onset and may. Landau kleffner syndrome is an extremely rare form of seizures or epilepsy occurring in children normally between the ages of 3 and 9 years of age. Landau kleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. Children with lks lose the ability to talk and understand speech. Hurley, phd university of south florida, tampa, florida landaukleffner syndrome lks is a rare, childhood neurological disorder characterized by a sudden or gradual. High amplitude epileptiform activity is seen in the temporalparietal regions spikes, sharp waves, spikeandwave or sharpslow wave, these may be unilateral or bilateral synchronous or asynchronous.
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