Children affected with lks have developed normally until signs and symptoms begin between the ages of 2 and 8. It is named after william landau and frank kleffner, who characterized it in 1957 with a diagnosis of six children. Acquired epileptic aphasia landaukleffner syndrome pediatric. Typical lks 2,3 is part of the epileptic encephalopathy of late childhood defined by 1. Magnetoencephalography in children with landaukleffner. Landau kleffner syndrome is characterized by subacute onset of acquired aphasia in a child with normal previous development and cognition. 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. 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. Landaukleffner syndrome lks is an epileptic encephalopathy marked by sudden and relatively rapid onset of aphasia in a child with normal or nearnormal language development. Landau kleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years.
Landau kleffner syndrome lksacquired epileptic aphasia aea typically develops in healthy children who acutely or progressively lose receptive and. Identification and treatment of landaukleffner syndrome. 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. He initially developed generalized convulsions which later changed to partial seizures during the course of illness. He was started on sodium valproate and continued with the drug 30 mgkg. 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 landau kleffner syndrome. Landau kleffner syndrome lks or acquired epileptic aphasia is a pediatric disorder characterized by the association of epileptiform electroencephalographic eeg abnormalities and acquired aphasia. Core of the syndrome which relates to an epileptic receptive aphasia. Landau kleffner syndrome causes, symptoms, diagnosis. 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 nord national organization for.
Download pdf page lks is a rare form of epilepsy with a typical onset of between 2 and 8 years of age, affecting males and females equally. Landaukleffner syndrome lks is a rare form of epilepsy diagnosed as acquired aphasia alternatively as acquired verbal agnosia cooccurring with epileptic seizures. A child with lks has a progressive loss of language abilities understanding and speaking after previously developing normally for their age. It is named after william landau and frank kleffner, who. Landau kleffner syndrome lks is a rare and underdiagnosed epilepsy syndrome. The landau kleffner syndrome or acquired aphasia with convulsive disorder. Landau kleffner syndrome lks is an agerelated epileptic encephalopathy where developmental regression occurs mainly in the language domain and the. Families of landau kleffner syndrome home facebook.
Landau kleffner 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. A strong relationship was found, which is opposite to that for childhood aphasia after structural lesions of the left hemisphere. This paper introduces into acquired childhood aphasia with focus on a syndrome called landau kleffner syndrome, in which aphasia and epilepsy are combined. Landaukleffner syndrome lks is an acquired epileptic aphasia occurring in childhood and associated with a generally poor prognosis for recovery of speech. Children with lks typically develop normally until signs and symptoms of the syndrome begin to develop between age 2 and 8 years. 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. A major feature of lks is the gradual or sudden loss of the ability to understand and use spoken language. Her expertise and services span over forty years in schools, universities, hospital clinics, private practice, workshopinservice speaking and consulting. It is characterised by a sudden or gradual loss of ability to understand of express language aphasia and usually occurs in children between the ages of 3 and 7 years. The peak age at time of onset is between 3 and 8 years.
Sudden or gradual loss of the ability to understand language and to speak is a feature of lks. Landau kleffner syndrome lks is a rare form of epilepsy diagnosed as acquired aphasia alternatively as acquired verbal agnosia cooccurring with epileptic seizures. 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. 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. Landaukleffner syndrome lks, or acquired epileptiform aphasia, is an. Landau kleffner syndrome responsive to levetiracetam. 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. The acquired aphasia classically begins with verbal auditory agnosia word deafness and often progresses to expressive language impairment.
Landaukleffner syndrome lks is a rare form of epilepsy diagnosed as. Characteristics of lks include language deterioration, seizure disorders, and severe electroencephalogram abnormalities. Differential diagnoses include deafness, an acute behavioural or psychiatric dis. Landau kleffner syndrome what is landaukleffner syndrome.
In additional to language regression, the diagnosis requires the presence of severely epileptiform activity on eeg, particularly during nonrem sleep. 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. The aim of this single case study is to describe a child with landau kleffner syndrome who misdiagnosed as a child with autism spectrum 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. Despite careful search, no pathologic evidence of encephalitis was found. It usually occurs in children between the ages of 3 and 9 and affects the area of the brain that controls speech and comprehension. Therefore, we recommend a sleep electroencephalogram in all children with landau kleffner syndrome. Landau kleffner syndrome and misdiagnosis of autism. Landaukleffner syndrome lks or acquired epileptic aphasia is a childhood disorder in which acquired aphasia and epileptiform electroencephalographic. Multiple subpial transection for landaukleffner syndrome. This syndrome is also characterized by an abnormal electroencephalogram eeg, especially during sleep. Landau kleffner syndrome lks is an acquired epileptic aphasia occurring in childhood and associated with a generally poor prognosis for recovery of speech.
It is thought to be the result of an epileptogenic lesion arising in speech cortex during a critical period of. Oct 19, 2017 the landau kleffner syndrome or acquired aphasia with convulsive disorder. Landaukleffner syndrome definition of landaukleffner. This has since come to be termed the landaukleffner syndrome lks. Landaukleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory. This article provides an overview of some relevant case studies of landau kleffner.
Landau kleffner syndrome causes loss of ability to understand and use language, and it can cause seizures. This has since come to be termed the landau kleffner syndrome lks. Affected siblings and discordant monozygotic twins have been reported rarely. Moreover, it will be compared to other forms of acquired childhood aphasia. Typically, a care team for children with lks consists of a neurologist, a neuropsychologist, and a speech pathologist or audiologist. Comments on key trends and developments related to the diagnosis and treatment of the nervous system disorder, the landau kleffner syndrome.
The condition is characterized by the gradual or sudden loss of the ability to understand and use spoken language, and the onset of seizures, which usually occur at night. Kleffner syndrome, the older the child at onset the better the prognosis for language. Secondary symptoms include psychomotor or behavioral disturbances and epilepsy with a favorable outcome for seizure control. Acquired epileptic aphasia 1 is an epileptic syndrome described in the international classification of epilepsies by the eponym landau kleffner syndrome lks. This syndrome is also characterized by an abnormal electroencephalogram eeg, especially during. The landau kleffner syndrome lks, formerly known as acquired epileptic aphasia, is a rare syndrome that typically presents in early childhood with language regression and seizures. The landaukleffner syndrome or acquired aphasia with convulsive disorder. Many children with lks also have seizures, episodes of uncontrolled body movement. Pat van slyke is an engaging, highly qualified and well respected speaker, retired professor, writer and provider of clinical services. Landaukleffner syndrome lks is an acquired epileptic aphasia in which children 38years old, who already have developed normal speech, experience language regression with verbal auditory agnosia, abnormal epileptiform activity, and behavioral disturbances. Landaukleffner syndrome epilepsy and seizures jama.
Landaukleffner syndrome lks is an acquired aphasic syndrome occurring in a previously normal child and probably is a variant of csws. The syndrome of acquired verbal auditory agnosia in childhood with mutism and epileptic discharges has been described in over 100 cases. Landau kleffner syndrome great ormond street hospital. Children with lks lose the ability to talk and understand speech. Landau kleffner syndrome lks is an acquired epileptic aphasia caused by a lesion in the speech centers of the cerebral cortex during a critical period of childhood development. Systemic examination, including neurological evaluation, was normal. Hirsch, e marescaux, c maquet, p metzlutz, mn kiesmann, m salmon, e et al. It also blindsides them with autisticlike symptoms and severe aggression.
Landaukleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in. Rolandic epilepsy is the most common epilepsy syndrome in childhood with involvement of the oropharyngeal manifestations. Landau kleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with. We report a case of lks in an 7yearold boy who presented with aggressive behavior, difficulty in maintaining posture, and language regression. Sudhansu chokroverty, pasquale montagna, in sleep disorders medicine third edition, 2009. Landaukleffner syndrome lksalso called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorderis a rare childhood neurological syndrome. 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. Eeg in landaukleffner syndrome pediatric neurology briefs. An encephalitic etiology has often been postulated but never proved. This article provides an overview of some relevant case studies of landau kleffner patients and also some neuromeasurement studies of the neurophysiology of the disease.
Kleffner syndrome lks is an acquired epileptic aphasia caused by a lesion in the speech centers of the cerebral cortex during a critical period of childhood development. Landaukleffner syndrome an overview sciencedirect topics. Finally, our findings did not demonstrate the beneficial effect of treatment with anticonvulsants on the aphasia, but recent studies have shown that treatment with corticosteroids, whether combined with anticonvulsants, is effective. Landaukleffner syndrome lks lennoxgastaut syndrome lgs reflex epilepsies rasmussens syndrome. Landau kleffner syndrome also known as acquired epileptic aphasia, progressive epileptic aphasia or aphasia with convulsive disorder, is a rare childhood neurological syndrome characterized by the sudden or gradual development of aphasia the inability to understand or express language and recurrent seizures epilepsy. It can also be seen as early as 18 months of age or as late as 14 years. Landaukleffner syndrome, is a rare disorder with no known cause. Landau kleffner syndrome lksalso called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorderis a rare childhood neurological syndrome. The condition usually appears in children between 3 and 7 years of age. Child with landau kleffner syndrome misdiagnosed as. Evidence on the relationship between the severity of language outcome and length of electrical status epilepticus in sleep eses. Lks usually occurs in children between ages 3 and 7 and can affect the childs ability to speak, understand language or both.
Key points about landau kleffner syndrome in a child. Schools for the deaf were in the majority oralist in their approach, and the use of sl was forbidden lane, 1993. Landau kleffner syndrome is a rare disorder that affects twice as many males as females. You wont find landau kleffner variant in the medical diagnostic manuals though. Landau kleffner syndrome lks is characterized by the sudden or gradual development of aphasia the inability to understand or express language. Landau kleffner syndrome what is landau kleffner syndrome. The early stages of the lks may be manifested by the symptoms of the autism leading to misdiagnosis. Landau kleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions. Kleffner syndrome lks, or acquired epileptiform aphasia, is an epilepsy syndrome involving progressive neuropsychological impairment related to the appearance of paroxysmal electroencephal. Unlimited viewing of the articlechapter pdf and any associated supplements and figures.
Landaukleffner syndrome lks or acquired epileptic aphasia is a childhood disorder in which. It is important to be aware that if language regression is observed in a child, subclinical seizure activity could be the cause. Acquired childhood aphasia with focus on landaukleffner. The landaukleffner syndrome or acquired aphasia with. Landau kleffner syndrome is a rare childhood neurological disorder of unknown cause. This syndrome is also known as progressive epileptic aphasia and aphasia with convulsive disorder.
The organization, which currently consists of approximately 100 members, is dedicated to providing information, support, and resources to families of. Acquired epileptic aphasia landaukleffner syndrome. This is a page of resource and comfort for families and friends affect by landau kleffner syndrome. Landau kleffner syndrome will be discussed in regard to symptoms, aetiology, therapy and prognosis. Landau kleffner syndrome boston childrens hospital. Child with landau kleffner syndrome misdiagnosed as autism. We report two patients with this syndrome who were treated surgically. 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. Rolandic epilepsy is the most common epilepsy syndrome in childhood with involvement of the oropharyngeal manifestations, hypersalivation and arrest of. Landaukleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. Landau kleffner syndrome lks is a rare childhood neurological syndrome. Thelandaukeffnersyndrome 387 diagnosis the diagnosis of landau kleffner syndrome depends largely on being aware that the condition exists, and its usual pattern of presentation.
Landaukleffner syndrome lks is a condition that shows up during childhood, causing difficulties with speech and seizures. Landaukleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most of the patients associated with. 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. Course and correlates with outcome robinson ro, baird g, robinson g, simonoff e developmental medicine and child neurology, 2001. Landaukleffner syndrome lks is a rare neurological disorder that causes a loss of language skills aphasia. Pdf landaukleffner syndrome is a rare childhoodonset epileptic encephalopathy. Kleffner, coauthor of their initial paper and former director of the central institute for the deaf in saintlouis, missouri, u. Landau kleffner syndrome an overview sciencedirect topics. It may be associated with convulsive seizures and additional difficulties with behaviour, social interaction, motor skills and learning. Lks is a rare form of epilepsy with a typical onset of between 2 and 8 years of age, affecting males and females equally. With lks overnight seizures in the temporal lobes rob children of their speech.
Article information, pdf download for the landaukleffner syndrome, open epub for. Sep 21, 2007 landau kleffner syndrome lks is a childhood disorder. Landaukleffner syndrome symptoms and treatment of landau. These children will have developed speech normally and then, suddenly or gradually lose language skills for no apparent reason. It is thought to be the result of an epileptogenic lesion arising in speech cortex during a critical period of development. Landaukleffner syndromea rare experience journalagent.