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