Know the causes, symptoms, treatment, prognosis and recovery of landaukleffner syndrome. What is landaukleffner syndromecausessymptomstreatment. The condition should be suspected if a child with normal development shows a fairly abrupt loss of established language skills. 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. Managing landau kleffner syndrome landaukleffner syndrome lks is an acquired epileptic aphasia of childhood and is a rare, childhood neurological syndrome.
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. Initially, these individuals have a healthy, problemfree development with normal speech and vocabulary. 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. In rare cases, children with lks may also have behavioral problems, including outbursts of temper, hyperactivity or acting withdrawn. Landau kleffner syndrome lks is a rare and underdiagnosed epilepsy syndrome. Arriving at the correct epilepsy syndrome andor etiology allows better decisionmaking about treatment and improves patient care.
All children have abnormal eeg compatible with the diagnosis of epilepsy, however, only 70% have clinical seizures. The landaukleffner syndrome is a rare syndrome of unknown etiology. Most patients appeared normal before the onset of the 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. There are many similarities between csws and lks, and the type of.
Landaukleffner syndrome what is landaukleffner syndrome. Landaukleffner syndrome, or acquired epileptic aphasia, was first reported in 1957 and is now recognized as an epileptic encephalopathy. Lks by all technical definitions she is not considered deaf or hard of hearing. It is named after william landau and frank kleffner, who characterized it in 1957 with a diagnosis of six children. This disorder impairs the ability of the child to understand instructions and be able to communicate to others. 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 a condition that shows up during childhood, causing difficulties with speech and seizures. Landaukleffner syndrome is a rare nervous system disorder. It causes loss of ability to understand and use language, and it can cause seizures.
Lks usually occurs in children between ages 3 and 7 and can affect the childs ability to speak, understand language or both. It also blindsides them with autisticlike symptoms and severe aggression. My daughter developed normally and hit all of her milestones on time or early. A major feature is the loss of ability to understand and use spoken language. Landaukleffner syndrome lks usually has an onset between three and seven years old. Landaukleffner syndrome lks is a rare epileptic syndrome characterized by acquired aphasia with epilepsy. Landaukleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions.
Laundaukleffner can often cooccur with autism or autistic symptoms, and the most common mutation that underlies the syndrome, grin2a, is a glutamate receptor that is firmly housed in the accepted core set of autism risk genes, as are other closely related glutamaterelated genes. Jenny mccarthy setting the record straightwhen it suits. Landaukleffner syndrome is manifested as a form of aphasia, loss of language, which usually develops between 3 and 7 years. Landau kleffner syndrome an overview sciencedirect topics. These children will have developed speech normally and then, suddenly or gradually lose language skills for no apparent reason. 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. The reasons that sl was successful and brought some children out of isolation while it could not be implemented in others are discussed. A syndrome is a group of signs and symptoms, which if they occur together, can suggest a particular condition. This article provides an overview of some relevant case studies of landaukleffner patients and also some neuromeasurement studies of the neurophysiology of the disease. Landaukleffner syndrome lks is an acquired aphasic syndrome occurring in a previously normal child and probably is a variant of csws. A syndrome characterized by the onset of isolated language dysfunction in otherwise normal children age of onset 47 years and epileptiform discharges on electroencephalographyseizures, including atypical absence epilepsy, absence, complex partial epilepsy, complex partial, and other types may occur. Families of landau kleffner syndrome home facebook. You wont find landau kleffner variant in the medical diagnostic manuals though.
The main epileptic activity happens during sleep and is usually not obvious to others. In a prospective study, children diagnosed between 2002 and 2006 with either lks or csws syndrome were treated soon after diagnosis with intravenous courses of. This study reports results of therapy with immunoglobulin in children with landau. A rare, childhood neurological disorder that is characterized by.
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. Kleffner, coauthor of their initial paper and former director of the central institute for the deaf in saintlouis, missouri, u. 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. Case report a 5 year old boy developed episodes of sadness and crying for about one hour, immediately after the departure of his father for higher study. Specifically, the eeg typcally shows an increase to nearly continuous abnormal brain activity firing spikes. Onset occurs between 18 months and years of age, with threefourths of the cases appearing before the age of 7 years. Landau kleffner syndrome is an uncommon and poorly understood form of childhood epilepsy. Landaukleffner syndrome lks is a rare childhood epilepsy accompanied by acquired aphasia. Children with lks lose the ability to talk and understand speech. Schools for the deaf were in the majority oralist in their approach, and the use of sl was forbidden lane, 1993.
The disorder is characterised by gradual or rapid loss of language in a previously normal child. Social skills training for a young adult with landau. She has lost the ability to speak verbally, does not process spoken language or environmental sounds, and appears most of. Disease bioinformatics research of landaukleffner syndrome has been linked to epilepsy, aphasia, status epilepticus, epilepsies, partial, language disorders. 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 lks is an acquired epileptic aphasia disorder in which children, usually 38 years of age who have developed ageappropriate speech, experience language regression. Typically, a care team for children with lks consists of a neurologist, a neuropsychologist, and a speech pathologist or audiologist. Landaukleffner syndrome symptoms and causes boston. Many authors state that there is a spectrum, including this syndrome and the syndrome of continuous spikesandwaves during slow sleep, and data supporting this concept are presented. Common symptoms of landaukleffner syndrome lks include. Landau kleffner syndrome boston childrens hospital. The study of landaukleffner syndrome has been mentioned in research publications which can be found using our bioinformatics tool below. Landau kleffner syndrome lks also called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder is a rare childhood neurological syndrome. This syndrome is typically characterized by an abrupt or.
Landaukleffner syndrome lks is a childhood disorder. Brain biopsy and serological tests give mixed results and do not confirm the presence of specific encephalopathy. The landaukleffner syndrome or acquired aphasia with convulsive disorder. Clinical data suggest the possibility of an encephalitic process. Landaukleffner syndrome article about landaukleffner. Landau kleffner syndrome lks is a rare epileptic disorder that affects children under 10 years of age. My daughter has a rare brain disorder, called landaukleffner syndrome. Many children with lks also have seizures, episodes of uncontrolled body movement. The disorder results in children losing their ability to talk and understand daily conversation. It is accompanied by abnormal electroencephalogram eeg and behaviour symptoms of autism. Incredibles wannabe sidekick turned bad guy, from the incredibles 2004.
The most commonly used treatments were clobazam, ethosuximide, sulthiame. 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. 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 landaukleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957. The first symptoms seem to appear before the age of 6, and it typically affects twice as many boys as girls. Landaukleffner syndrome lks is characterized by the sudden or gradual development of aphasia the inability to understand or express language.
It can be seen on brain wave recordings eeg, electroencephalography. They reported five children with a gradual regression of language abilities showing paroxysmal unilateral or bilateral spikeandwave discharges, which were characteristically localized. Temporoparietal resection in a patient with landau. Such children should be referred to a regional department. How the causes of the landaukleffner syndrome develop also are unknown. Children with lks typically develop normally until signs and symptoms of the syndrome begin to develop between age 2 and 8 years. Syndrome pop culture when innocent heroworship goes unrequited, the consequences can be dire for both the admirer and the admired. It can happen in a child that has been developing normally. Many children and young people will have a set of related symptoms that fit a particular pattern and this will determine which syndrome they have.
A major feature of lks is the gradual or sudden loss of the ability to understand and use spoken language. T1 temporoparietal resection in a patient with 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 a rare neurological disorder that causes a loss of language skills aphasia. It may develop slowly over many months or very suddenly. It usually occurs in children between the ages of 3 and 9 and affects the area of the brain that controls speech and comprehension. Landau kleffner syndrome nord national organization for. 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. Acquired epileptic aphasia in children anne marie tharpe barbara j. Landau kleffner syndrome what is landaukleffner syndrome. Landaukleffner syndrome lks is a rare form of epilepsy diagnosed as acquired aphasia alternatively as acquired verbal agnosia cooccurring with epileptic seizures. This syndrome is also characterized by an abnormal electroencephalogram eeg, especially during sleep. Landau kleffner syndrome lks landau kleffner syndrome lks is a rare form of epilepsy that only affects children, and causes them to lose their understanding of language. 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. Landau kleffner syndrome responsive to levetiracetam.