Arts wf, aarsen fk, scheltensde boer m, catsmanberrevoets ce. It is accompanied by abnormal electroencephalogram eeg and behaviour symptoms of autism. Landau kleffner syndrome or continuous spike wave in slowwave sleep was identified in 12 patients during 6 years 19952000. 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. In additional to language regression, the diagnosis requires the presence of severely epileptiform activity on eeg, particularly during nonrem sleep. Pdf changing perspectives on landaukleffner syndrome. At eight months, months, she started saying dada, and by ten months, she had a burst of words that included kitty and even thank you. Total score ranges from 0 to 3,600 being 0 the worst and 3,600 the best. Landaukleffner syndrome or continuous spike wave in slowwave sleep was identified in 12 patients during 6 years 19952000. The landaukleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957. The first paper published describing landaukleffner syndrome lks was in 1957. Landau kleffner syndrome acquired aphasia with epilepsy by genevieve v. Despite careful search, no pathologic evidence of encephalitis was found.
Magnetoencephalography in children with landaukleffner. Insights into possible genetic factors gabrielle rudolf. Managing landau kleffner syndrome landaukleffner syndrome lks is an acquired epileptic aphasia of childhood and is 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. Landau kleffner syndrome boston childrens hospital. Landaukleffner syndrome lks is a childhood disorder.
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 epileptiform activity in these children is thought to result in a functional ablation of eloquent speech areas. We report two patients with this syndrome who were treated surgically. The most commonly used treatments were clobazam, ethosuximide, sulthiame. Acquired childhood aphasia with focus on landaukleffner syndrome. This syndrome is also characterized by an abnormal electroencephalogram eeg, especially during sleep. The landaukleffner syndromecase report and theoretical considerations. Affected siblings and discordant monozygotic twins have been reported rarely. Moreover, it will be compared to other forms of acquired childhood aphasia. Landau kleffner syndrome lks is an acquired epileptic aphasia disorder in which children, usually 38 years of age who have developed ageappropriate speech, experience language regression with verbal auditory agnosia, abnormal epileptiform activity, behavioral disturbances, and sometimes overt seizures. Statistics of landaukleffner syndrome 5 people with landaukleffner syndrome have taken the sf36 survey. Landau kleffner syndrome is a rare disorder that affects twice as many males as females. Feb 18, 2011 landau kleffner syndrome, online mendelian inheritance in man omim hirsch e, valenti mp, rudolf g, et al. Landaukleffner syndrome lks is a rare childhood epilepsy accompanied by acquired aphasia.
What is landaukleffner syndromecausessymptomstreatment. Landau kleffner syndrome lks, infantile acquired aphasia, acquired epileptic aphasia, or aphasia with convulsive disorder, a childhood disorder that occurs between the ages of 3 and 7, is characterized by the sudden loss of speech, epileptic seizures, depression, hyperactivity, and aggressiveness. Kleffner, coauthor of their initial paper and former director of the central institute for the deaf in saintlouis, missouri, u. Typically, a care team for children with lks consists of a neurologist, a neuropsychologist, and a speech pathologist or audiologist. 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 lksalso called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorderis a rare childhood neurological syndrome. Landau kleffner syndrome will be discussed in regard to symptoms, aetiology, therapy and prognosis. 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. Landaukleffner 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. Is cerebral arteritis the cause of the landaukleffner syndrome. Landau kleffner syndrome lks is characterized by the sudden or gradual development of aphasia the inability to understand or express language. 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. My daughter developed normally and hit all of her milestones on time or early. 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. 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.
Objectives define landaukleffner syndrome lks understand course of lks osymptoms otreatment oprognosis neurodiagnostic studies 3. The distribution of age of onset of aphasia in landaukleffner syndrome based on 266 cases from the literature. Oct 19, 2017 is cerebral arteritis the cause of the landau kleffner syndrome. 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. Landaukleffner syndrome lks or acquired epileptic aphasia is a childhood disorder in which. Landaukleffner syndrome lks, or acquired epileptiform aphasia, is an epilepsy syndrome involving. 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. The initial presentation may be with progressive aphasia 40%, seizures or both. This paper introduces into acquired childhood aphasia with focus on a syndrome called landau kleffner syndrome, in which aphasia and epilepsy are combined. Landau kleffner syndrome lks is epileptiform aphasia acquired during childhood and occurring in children with previously normal language development. Landau kleffner syndrome what is landau kleffner syndrome.
Landau kleffner syndrome what is landaukleffner syndrome. Landau kleffner syndrome an overview sciencedirect topics. It causes loss of ability to understand and use language, and it can cause seizures. With lks overnight seizures in the temporal lobes rob children of their speech. 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. The purpose of this study was to investigate the usefulness of magnetoencephalography meg. These children will have developed speech normally and then, suddenly or gradually lose language skills for no apparent reason. Landaukleffner syndrome is a rare nervous system disorder. Landaukleffner syndrome lks is a rare neurological disorder that causes a loss of language skills aphasia. Download fulltext pdf download fulltext pdf download fulltext pdf landaukleffner syndrome. Magnetoencephalography in children with landaukleffner syndrome and acquired epileptic aphasia. Mean of landaukleffner syndrome is 1900 points 53 %. Lks usually occurs in children between ages 3 and 7 and can affect the childs ability to speak, understand language or both.
This paper introduces into acquired childhood aphasia with focus on a syndrome called landaukleffner syndrome, in which aphasia and epilepsy are combined. At the last control, 26 patients had remained seizure free. 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. Know the causes, symptoms, treatment, prognosis and recovery of landaukleffner syndrome. Landau kleffner syndrome maximising cognitive potential. Landaukleffner syndrome lks or acquired epileptic aphasia is a childhood. The syndrome has onset between the ages of 2 and 8 years peak 5 to 7 years, or rarely later. Raising awareness and pursuing research into landau kleffner syndrome. Landau kleffner syndrome lks is a rare and underdiagnosed epilepsy syndrome. Landaukleffner syndrome lks is an acquired epileptic aphasia disorder in which children, usually 38 years of age who have developed ageappropriate speech, experience language regression. Children affected with lks have developed normally until signs and symptoms begin between the ages of 2 and 8. It can also be seen as early as 18 months of age or as late as 14 years. Landaukleffner syndrome lks is an acquired aphasic syndrome occurring in a previously normal child and probably is a variant of csws. Magnetoencephalography in children with landau kleffner syndrome and acquired epileptic aphasia.
All children have abnormal eeg compatible with the diagnosis of epilepsy, however, only 70% have clinical seizures. Landau kleffner syndrome is not an eponymic badge of ignorance. Landaukleffner syndrome is a rare disorder that affects twice as many males as females. Landaukleffner syndrome acquired aphasia with epilepsy by genevieve v. The organization, which currently consists of approximately 100 members, is dedicated to providing information, support, and resources to families of.
Article information, pdf download for the landaukleffner syndrome, open epub for the landaukleffner syndrome. 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. William landau neurologist and frank kleffner speech language pathologistslp first recognized the unique characteristics of patients who presented with acquired aphasia in conjunction with epilepsy. It can happen in a child that has been developing normally. Landaukleffner syndrome lks is an acquired epileptic aphasia disorder in which children, usually 38 years of age who have developed ageappropriate speech, experience language regression with verbal auditory agnosia, abnormal epileptiform activity, behavioral disturbances, and sometimes overt seizures. Children with lks typically develop normally until signs and symptoms of the syndrome begin to develop between age 2 and 8 years. The place taken by speech therapy has in fact varied according to the explanatory model to report on the syndromes particular evolutionary pattern. Sep 21, 2007 landau kleffner syndrome lks is a childhood disorder. They reported five children with a gradual regression of language abilities showing paroxysmal unilateral or bilateral spikeandwave discharges, which were characteristically localized. Few of the 150 publications or more dealing with landaukleffner syndrome have properly addressed the issues concerning speech therapy. The first lks cases were described by landau and kleffner 1957.
Speech therapy in landau and kleffner syndrome springerlink. Landaukleffner syndrome lks, infantile acquired aphasia, acquired epileptic aphasia, or aphasia with convulsive disorder, a childhood disorder that occurs between the ages of 3 and 7, is characterized by the sudden loss of speech, epileptic seizures, depression, hyperactivity, and. Landaukleffner syndrome lks is epileptiform aphasia acquired during childhood and occurring in children with previously normal language development. Know the causes, symptoms, treatment, prognosis and recovery of landau kleffner syndrome. Landau kleffner syndrome nord national organization for. The landaukleffner syndrome or acquired aphasia with convulsive disorder. It usually occurs in children between the ages of 3 and 9 and affects the area of the brain that controls speech and comprehension. The purpose of this study was to investigate the usefulness of magnetoencephalography meg for. Landaukleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years.
Named after william landau and frank kleffner, who characterized it in 1957. Landaukleffner syndrome uncountable a rare childhood neurological syndrome, characterized by the sudden or gradual development of aphasia and an abnormal electroencephalogram. Landau kleffner syndrome responsive to levetiracetam. 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.
Objectives define landau kleffner syndrome lks understand course of lks osymptoms otreatment oprognosis neurodiagnostic studies 3. An encephalitic etiology has often been postulated but never proved. 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. Landaukleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with. The distribution of age of onset of aphasia in landau kleffner syndrome based on 266 cases from the literature.
There are many similarities between csws and lks, and the type of. Pdf landaukleffner syndrome is a rare childhoodonset epileptic. Five patients had a history of earlyonset hydrocephalus. The syndrome of acquired verbal auditory agnosia in childhood with mutism and epileptic discharges has been described in over 100 cases. Case report article pdf available march 2015 with 322 reads. This disorder impairs the ability of the child to understand instructions and be able to communicate to others. Lks is a severe disease, affecting language abilities, attention and behavior, and evolving to acquired global aphasia. Landaukleffner syndrome is manifested as a form of aphasia, loss of language, which usually develops between 3 and 7 years. Landaukleffner syndrome will be discussed in regard to symptoms, aetiology, therapy and prognosis. Secondary symptoms include psychomotor or behavioral disturbances and epilepsy with a favorable outcome for seizure control. 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. Social skills training for a young adult with landaukleffner. Landau kleffner syndrome is characterized by subacute onset of progressive aphasia in a child with previous ageappropriate language development.
Landaukleffner syndrome lks is a rare agerelated epileptic. Landaukleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions. Landau kleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. You wont find landau kleffner variant in the medical diagnostic manuals though. Initially, these individuals have a healthy, problem free development with normal speech and vocabulary. A child with lks has a progressive loss of language abilities understanding and speaking after previously developing normally for their age. The disorder is characterised by gradual or rapid loss of language in a previously normal child. Landaukleffner syndrome lks usually has an onset between three and seven years old. Landaukleffner syndrome lks and benign epilepsy with centrotemporal spikes bects are two forms of nonlesional agerelated focal epilepsies. This syndrome is also known as progressive epileptic aphasia and aphasia with convulsive disorder.
Landau kleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory. Acquired childhood aphasia with focus on landaukleffner. It also blindsides them with autisticlike symptoms and severe aggression. Arriving at the correct epilepsy syndrome andor etiology allows better decisionmaking about treatment and improves patient care.
316 731 86 348 549 588 783 51 1303 128 249 625 286 646 845 225 860 173 1152 1089 1038 1538 384 642 866 1041 228 703 519 783 1008