This paper introduces into acquired childhood aphasia with focus on a syndrome called landau kleffner syndrome, in which aphasia and epilepsy are combined. It usually occurs in children between the ages of 3 and 9 and affects the area of the brain that controls speech and comprehension. A major feature of lks is the gradual or sudden loss of the ability to understand and use spoken language. Typically, a care team for children with lks consists of a neurologist, a neuropsychologist, and a speech pathologist or audiologist. Landau kleffner syndrome causes loss of ability to understand and use language, and it can cause seizures. The first symptoms seem to appear before the age of 6, and it typically 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. Landau kleffner syndrome what is landaukleffner syndrome. Landaukleffner syndrome will be discussed in regard to symptoms, aetiology, therapy and prognosis.
Children with lks typically develop normally until signs and symptoms of the syndrome begin to develop between age 2 and 8 years. Landaukleffner syndrome epilepsy and seizures jama. All children have abnormal eeg compatible with the diagnosis of epilepsy, however, only 70% have clinical seizures. Landaukleffner syndrome symptoms and causes boston. The landaukleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957. Landaukleffner syndrome lks is characterized by the sudden or gradual development of aphasia the inability to understand or express language. Landaukleffner syndrome lks is a childhood disorder. Landaukleffner syndrome lks or acquired epileptic aphasia is a childhood disorder in which acquired aphasia and epileptiform electroencephalographic. 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. Our series showed that the clinical picture can vary at onset, as well as during the course of the illness, and that the longterm outcome of the aphasia is quite unpredictable. Children affected with lks have developed normally until signs and symptoms begin between the ages of 2 and 8. This is a page of resource and comfort for families and friends affect by landau kleffner syndrome. Common symptoms of landaukleffner syndrome lks include. Landaukleffner syndrome definition of landaukleffner.
The mean duration of cswseses was 8 months range 216 months. Landaukleffner syndrome synonyms, landaukleffner syndrome pronunciation, landaukleffner syndrome translation, english dictionary definition of landaukleffner syndrome. The early stages of the lks may be manifested by the symptoms of the autism leading to misdiagnosis. Moreover, it will be compared to other forms of acquired childhood aphasia.
In rare cases, children with lks may also have behavioral problems, including outbursts of temper, hyperactivity or acting withdrawn. Landau kleffner information and support group has 679 members. Landau kleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions. Key points about landau kleffner syndrome in a child. Landaukleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions.
The peak age at time of onset is between 3 and 8 years. 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. Landau kleffner information and support group public. There may be focal temporalparietal or diffuse slowing. A child with lks has a progressive loss of language abilities understanding and speaking after previously developing normally for their age.
Landaukleffner syndrome lks is a condition that shows up during childhood, causing difficulties with speech and seizures. Landau kleffner syndrome lks or acquired epileptic aphasia is a pediatric disorder characterized by the association of epileptiform electroencephalographic eeg abnormalities and acquired aphasia. Landaukleffner syndrome and aphasia facts and information. 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. 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. It is named after william landau and frank kleffner, who characterized it in 1957 with a diagnosis of six children. In additional to language regression, the diagnosis requires the presence of severely epileptiform activity on eeg, particularly during nonrem sleep. When innocent heroworship goes unrequited, the consequences can be dire for both the admirer and the admired.
Landau kleffner syndrome responsive to levetiracetam. There are many similarities between csws and lks, and the type of. He was started on sodium valproate and continued with the drug 30 mgkg. This disorder impairs the ability of the child to understand instructions and be able to communicate to others. Landau kleffner syndrome lksacquired epileptic aphasia. A group of symptoms that collectively indicate or characterize a disease, disorder, or other condition considered abnormal. Landaukleffner syndrome lksalso called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorderis a rare childhood neurological syndrome.
Key points about landaukleffner syndrome in a child. 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. Pdf landaukleffner syndrome is a rare childhoodonset epileptic encephalopathy. Typical lks 2,3 is part of the epileptic encephalopathy of late childhood defined by 1. Acquired epileptic aphasia 1 is an epileptic syndrome described in the international classification of epilepsies by the eponym landaukleffner syndrome lks. It may be associated with convulsive seizures and additional difficulties with behaviour, social interaction, motor skills and learning. Landau kleffner syndrome synonyms, landau kleffner syndrome pronunciation, landau kleffner syndrome translation, english dictionary definition of landau kleffner syndrome.
What every educator should know about landaukleffner. What is landaukleffner syndromecausessymptomstreatment. Landau kleffner syndrome lks is an agerelated epileptic encephalopathy where developmental regression occurs mainly in the language domain and the. He was started on sodium valproate and continued with the drug 30. Landaukleffner syndrome is a rare disorder that is characterized by the gradual or sudden loss of language in children between the ages of five and seven. Department of neurology, max superspecialty hospital, saket, new delhi 110017, india. 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. Landaukleffner syndrome causes loss of ability to understand and use language, and it can cause seizures. He initially developed generalized convulsions which later changed to partial seizures during the course of illness. Landau kleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. 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. Erfolgreiche dexamethasontherapie bei landaukleffnersyndrom.
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. The landaukleffner syndrome or acquired aphasia with convulsive disorder. 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. Landau kleffner syndrome lks is a rare and underdiagnosed epilepsy syndrome. Acquired epileptic aphasia landaukleffner syndrome. Lks usually starts between 3 and 6 years and affects twice as many boys as girls. However, a number of candidate genes have been implicated, especially one involving glutamate metabolism known as grin2a. This group was started in february of 2009 in hopes of finding another family to compare. Acquired aphasia in children download ebook pdf, epub. Landau kleffner syndrome will be discussed in regard to symptoms, aetiology, therapy and prognosis. Landaukleffner syndrome responsive to levetiracetam. Landaukleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with. 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. Autism research institute boston childrens hospital epilepsy foundation john hopkins medicine medicine net medscape national organization for rare disorders ninds webmd.
These include using predictable language, creating the need to communicate, and using alternative. Landau kleffner syndrome and misdiagnosis of autism. Landau kleffner syndrome lks is a condition that shows up during childhood, causing difficulties with speech and seizures. Landau kleffner syndrome is characterized by subacute onset of acquired aphasia in a child with normal previous development and cognition. Hirsch, e marescaux, c maquet, p metzlutz, mn kiesmann, m salmon, e et al. Landau kleffner syndrome an overview sciencedirect topics. Landau w, kleffner f 1957 syndrome of acquired aphasia with convulsive disorder in children. Lks affects the portions of the childs brain that controls their speech and comprehension. In 1957, landau and kleffner initially described acquired epileptic aphasia and subsequently reluctantly agreed to the attachment of their names to the syndrome. 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. Know the causes, symptoms, treatment, prognosis and recovery of landaukleffner syndrome. In this article, acquired epileptic aphasia is used as a synonym for landau kleffner syndrome lks. 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. Landaukleffner syndromea rare experience journalagent.
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. 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. The disorder is characterised by gradual or rapid loss of language in a previously normal child. Secondary symptoms include psychomotor or behavioral disturbances and epilepsy with a favorable outcome for seizure control. Landaukleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. Landau kleffner information and support group public group.
An eightyearold male child presenting with history of generalized convulsions, gradual loss of speech and generalized eeg electroencephalography abnormalities was diagnosed as landau kleffner syndrome. Oct 19, 2017 robinson ro, baird g, robinson g, simonoff e. Families of landau kleffner syndrome home facebook. The language dysfunction may have an acute or insidiously progressive onset and may. 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. 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. 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. It can also be seen as early as 18 months of age or as late as 14 years.
Landau kleffner syndrome boston childrens hospital. Landaukleffner syndrome lks is an agerelated epileptic encephalopathy where developmental regression occurs mainly in the language domain and the. 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. The organization, which currently consists of approximately 100 members, is dedicated to providing information, support, and resources to families of.
This has since come to be termed the landau kleffner syndrome lks. Landaukleffner syndrome lks is an acquired aphasic syndrome occurring in a previously normal child and probably is a variant of csws. 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. Its main features are a loss of speech and language skills, with seizures and learning and behaviour problems. Landaukleffner syndrome lks is a rare form of epilepsy diagnosed as.
This paper introduces into acquired childhood aphasia with focus on a syndrome called landaukleffner syndrome, in which aphasia and epilepsy are combined. Landau kleffner syndrome is characterized by subacute onset of acquired aphasia in a child. Many children with lks also have seizures, episodes of uncontrolled body movement. Landaukleffner syndrome is characterized by difficulty in receptive or expressive language, abnormal electroencephalograms, and seizures. The most commonly used treatments were clobazam, ethosuximide, sulthiame. This syndrome is also characterized by an abnormal electroencephalogram eeg, especially during sleep. This has since come to be termed the landaukleffner syndrome lks. Landau kleffner syndrome great ormond street hospital. Syndrome pop culture when innocent heroworship goes unrequited, the consequences can be dire for both the admirer and the admired. Eeg in landaukleffner syndrome pediatric neurology briefs. Landaukleffner syndrome lks is a rare neurological disorder that causes a loss of language skills aphasia. Lks usually occurs in children between ages 3 and 7 and can affect the childs ability to speak, understand language or both.
The speech and language skills may improve over time. Such children should be referred to a regional department of paediatric medicine or. Characteristics the main feature of lks is the gradual or sudden loss of the ability to understand and use spoken language. Landaukleffner syndrome article about landaukleffner. This article describes the primary and secondary characteristics of children with this syndrome and offers educators a framework for intervention techniques. Erworbene aphasie mit epilepsie, benannt nach william. Landau kleffner syndrome lks is a very rare epilepsy syndrome of childhood. In this article, acquired epileptic aphasia is used as a synonym for landaukleffner syndrome lks. The landaukleffner syndrome or acquired aphasia with.
The landau kleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957. Children with lks lose the ability to talk and understand speech. Incredibles wannabe sidekick turned bad guy, from the incredibles 2004. Landau kleffner syndrome nord national organization for. Sep 21, 2007 landau kleffner syndrome lks is a childhood disorder. These children will have developed speech normally and then, suddenly or gradually lose language skills for no apparent reason. Pixars computeranimated film the incredibles 2004 presents a case in point in one buddy pine, a decidedly nonsuperpowered adolescent with a talent for invention and a stalkerlike obsession with the ultrastrong superhero known as mr. Arriving at the correct epilepsy syndrome andor etiology allows better decisionmaking about treatment and improves patient care. Electrophysiology of csws in landau kleffner syndrome. Landaukleffner syndrome special needs resource and.
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