Recent developments in the genetics of childhood epileptic. Landaukleffner 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. Landau kleffner syndrome great ormond street hospital. Landau kleffner syndrome lks or acquired epileptic aphasia is a childhood disorder in which acquired aphasia and epileptiform electroencephalographic. It can also be seen as early as 18 months of age or as late as 14 years. Landau kleffner syndrome, autistic regression, behavior disorder, epilepsy, eeg. As lks is usually readily responsive to an adequate pharmacological management, an early diagnosis of children at. Isoelectric csf focusing showed oligoclonal igg bands. Landau kleffner syndrome lks or acquired epileptic aphasia, first described by landau and kleffler in 1957 1, is a rare childhood neurological disorder, being characterized by an acquired aphasia that emerges in relation to epileptiform electroencephalographic eeg abnormalities 1,2.
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. Lks is a severe disease, affecting language abilities, attention and behavior, and evolving to acquired global aphasia. Pdf changing perspectives on landaukleffner syndrome. In a study of 77 cases of landau kleffner syndrome, 6 were found to have this type of aphasia.
Systemic examination, including neurological evaluation, was normal. It usually occurs in children between the ages of 3 and 9 and affects the area of the brain that controls speech and comprehension. Landaukleffner syndrome lks, which was first described in 1957, is a childhood disorder manifesting two main symptoms. At the time of their interdisciplinary collaboration, landau and kleffner figure 1 and figure 2 could not have known the significance of their description of this. Social skills training for a young adult with landaukleffner. Article information, pdf download for the landaukleffner syndrome, open epub for. If you have problems viewing pdf files, download the latest version of adobe reader. We report a case of lks in an 7yearold boy who presented with aggressive behavior, difficulty in maintaining posture, and language regression. Aphasia and hyperkinesia were isolated in three patients and associated with global regression of higher cortical functions in one pat.
Article information, pdf download for the landau kleffner syndrome, open epub for. Landau kleffner syndrome lks or acquired epileptic aphasia is a childhood. Feb 01, 2014 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. Children with lks typically develop normally until signs and symptoms of the syndrome begin to develop between age 2 and 8 years. 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 landau kleffner syndrome lks. Educational therapeutic approaches used with child presenting. Introductionlandau kleffner syndrome lks, also known as acquired epileptic aphasia, was described for the first time in 1957 by landau and kleffner in six children with a history of normal language acquisition, but who later developed acquired aphasia with convulsive disorder. This has since come to be termed the landaukleffner syndrome lks. The landaukleffner syndrome is a rare disorder charac terised by an acquired receptive and expressive aphasia and epileptic seizuresl4. Recognizing their unique pattern, landau took the lead in submitting to the journal neurology the 1957 paper that led to the designation of landau. The evidence that these discharges in the landaukleffner syndrome can have a focal. We present the differential diagnosis and treatment of frank, a 26yearold man with landau kleffner syndrome lks.
Few of the 150 publications or more dealing with landau kleffner syndrome have properly addressed the issues concerning speech therapy. Speech therapy in landau and kleffner syndrome springerlink. 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 lksalso called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorderis a rare childhood neurological syndrome.
Children affected with lks have developed normally until signs and symptoms begin between the ages of 2 and 8. In the april 1992 issue of the archives, the landaukleffner syndrome was considered in an editorial by landau 1 and in an article by paquier et al. Aug 11, 2017 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. Aphasia and hyperkinesia were isolated in three patients and associated with global regression of higher cortical functions in one patient. If you would like to participate, please visit the project page, where you can join the discussion and see a list of open tasks. Landau kleffner syndrome lks is a rare childhood neurological syndrome.
The most commonly used treatments were clobazam, ethosuximide, sulthiame. Olgu sunumu muharrem burak baytunca1, ipek percinel2, hande gazeteci tekin3, gul serdaroglu3, sarenur gokben3 1ege university faculty of medicine, department of child psychiatry, izmir, turkey 2osmaniye state hospital. Secondary symptoms include psychomotor or behavioral disturbances and epilepsy with a favorable outcome for seizure control. Landaukleffner syndrome lks is an acquired aphasic syndrome occurring in a previously normal child and probably is a variant of csws. The landau kleffner syndrome is a rare disorder charac terised by an acquired receptive and expressive aphasia and epileptic seizuresl4. Acquired epileptic aphasia landaukleffner syndrome. Landau kleffner syndrome lks is a rare childhood epileptic encephalopathy, along with a deterioration of cognitive, sensory, andor motor functions results from epileptic activity. Landau kleffner syndrome and misdiagnosis of autism. 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.
Sudhansu chokroverty, pasquale montagna, in sleep disorders medicine third edition, 2009. Pdf use of flunarizine in a case of landaukleffner. Landau kleffner syndrome lks is an acquired epileptic aphasia occurring in childhood and associated with a generally poor prognosis for recovery of speech. The landaukleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957. Efficacy of intravenous immunoglobulin in landaukleffner. 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, or acquired epileptiform aphasia, is an. Landaukleffner syndrome lks, or acquired epileptiform aphasia, is an epilepsy. Kleffner, coauthor of their initial paper and former director of the central institute for the deaf in saintlouis, missouri, u. Patients tend to relapse during tapering of drugs, especially steroids. Magnetoencephalography in children with landaukleffner. The peak age at time of onset is between 3 and 8 years. Landaukleffner syndrome lks is a rare agerelated epileptic encephalopathy, characterized by a developmental. A rare, childhood neurological disorder that is characterized by. We compared the 1month baseline to that following ivig using a. Landau kleffner syndrome is within the scope of wikiproject epilepsy, a collaborative effort to improve the coverage of all aspects of epilepsy and epileptic seizures on wikipedia. Landau kleffner syndrome should be considered in the differential diagnosis in a child with epilepsy and aphasia. Landaukleffner syndrome lks or acquired epileptic aphasia is a childhood.
Download fulltext pdf download fulltext pdf read fulltext. 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 kleffner syndrome is an epileptic encephalopathy characterized by acquired. The place taken by speech therapy has in fact varied according to the explanatory model to report on the syndrome s particular evolutionary pattern. Landaukleffner syndrome epilepsy and seizures jama. Landaukleffner syndromea rare experience journalagent. Pdf landaukleffner syndrome is a rare childhoodonset epileptic encephalopathy. 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 causes, symptoms, diagnosis. One japanese study has reported that the incidence of children with lks aged 5a.
Landau kleffner nonlinguistic syndrome treatment verbal auditory aphasia a b s t r a c t purpose. C this article has been rated as cclass on the projects quality scale. The landau kleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957. Rolandic epilepsy is the most common epilepsy syndrome in childhood with involvement of the oropharyngeal manifestations, hypersalivation and arrest of. Landau kleffner syndrome and misdiagnosis of autism spectrum. These children will have developed speech normally and then, suddenly or gradually lose language skills for no apparent reason.
Download medical conditions landau kleffner syndrome 145. 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. Landaukleffner syndrome lks is characterized by the sudden or gradual development of aphasia the inability to understand or express language. The description of landau kleffner syndrome lks has profoundly influenced not only clinical practice but also cognitive neuroscience. Effect of early corticosteroid therapy for landau kleffner syndrome. Landaukleffner syndrome an overview sciencedirect topics. Landau kleffner syndrome lks is a rare agerelated epileptic encephalopathy, characterized by a developmental. Followup is essential, as response to treatment varies.
Pdf landau kleffner syndrome is a rare childhoodonset epileptic encephalopathy. Pdf landaukleffner syndrome and swearing michal korenar. Landaukleffner syndrome lks and benign epilepsy with centrotemporal spikes bects are two forms of nonlesional agerelated focal epilepsies. Her expertise and services span over forty years in schools, universities, hospital clinics, private practice, workshopinservice speaking and consulting.
We compared the 1month baseline to that following ivig using a severity score assessing speech, comprehension, behavior, seizures, and electroencephalography. Jul 29, 2009 as a result, we were both surprised, landau because he had never seen such children, and kleffner because he had no idea that such cases would be unknown to landau. Landau kleffner syndrome lks or acquired epileptic aphasia is a pediatric disorder characterized by the association of epileptiform electroencephalographic eeg abnormalities and acquired aphasia. Introductionlandau kleffner syndrome lks is characterized by normal speech acquisition followed by epileptic seizures, receptive and expressive language deterioration coupled with agnosia for nonverbal sounds, having variable longterm evolution. 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.
Landau kleffner syndrome lks is a rare and underdiagnosed epilepsy syndrome. This article provides an overview of some relevant case studies of landau kleffner. Pat van slyke is an engaging, highly qualified and well respected speaker, retired professor, writer and provider of clinical services. At the time of assessment, frank was no longer suffering from primary symptoms of lks. Jan 04, 2019 landau kleffner lwndau is characterized by acquired aphasia and electroencephalographic abnormalities during wakefulness and sleep. The distribution of age of onset of aphasia in landau kleffner syndrome based on 266 cases from the literature.
Landau kleffner syndrome lksacquired epileptic aphasia aea typically develops in healthy children who acutely or progressively lose receptive and. Landaukleffner syndrome is an epileptic encephalopathy characterized by acquired. Department of neurology, childrens national medical center, and george washington university school of medicine, washington, d. The landaukleffner syndrome of acquired epileptic aphasia. 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. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Landau kleffner syndrome lks or syndrome of acquired aphasia with convulsive disorder in children is an epileptic encephalopathy that occurs in previously normal children with normally developed ageappropriated language9. It is named after william landau and frank kleffner, who characterized it in 1957 with a diagnosis of six children. Differential diagnoses include deafness, an acute behavioural or psychiatric dis.
Landaukleffner syndrome is an epileptic encephalopathy characterized by acquired verbal auditory aphasia and seizures in most. Apr 01, 2002 we administered 2 gmkg of intravenous gamma globulin ivig to each of five consecutive patients with landau kleffner syndrome, over 4 days. Join researchgate to discover and stay uptodate with the latest research from leading experts in landau kleffner syndrome and many. Social skills training for a young adult with landau. It is characterized by seizures and acquired aphasia, typically verbal auditory agnosia, with onset between 2 and 8 years. Landaukleffner syndrome lks, or acquired epileptiform aphasia, is an. Mar 15, 2018 autism spectrum disorders asd is the name for a group of developmental disorders including a wide range of signs, symptoms and disability. Kleffner syndrome lks, or acquired epileptiform aphasia, is an epilepsy syndrome involving progressive neuropsychological impairment related to the appearance of paroxysmal electroencephal. In five children with normal initial psychomotor development, a landau. Landau kleffner syndrome what is landau kleffner syndrome. The evidence that these discharges in the landau kleffner syndrome can have a focal.
Landaukleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions. Choueiri, md we administered 2 gmkg of intravenous gamma globulin ivig to each of five consecutive patients with landau kleffner syndrome, over 4 days. Landaukleffner syndrome lks is a rare form of epilepsy diagnosed as acquired aphasia alternatively as acquired verbal agnosia cooccurring with epileptic seizures. Thelandaukeffnersyndrome 387 diagnosis the diagnosis of landaukleffner syndrome depends largely on being aware that the condition exists, and its usual pattern of presentation.
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