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. It can also be seen as early as 18 months of age or as late as 14 years. Landau kleffner syndrome what is landaukleffner syndrome. Sep 21, 2007 landau kleffner syndrome lks is a childhood disorder. This syndrome is also characterized by an abnormal electroencephalogram eeg, especially during sleep. Landau kleffner syndrome is a rare disorder that affects twice as many males as females. Boys are thought to be affected with lks twice as often as girls. Landaukleffner syndrome lks is a rare neurological disorder that causes a loss of language skills aphasia. What is landaukleffner syndromecausessymptomstreatment. The landaukleffner syndrome or acquired aphasia with. Landau kleffner syndrome synonyms, landau kleffner syndrome pronunciation, landau kleffner syndrome translation, english dictionary definition of landau kleffner syndrome.
Landau kleffner syndrome lks is epileptiform aphasia acquired during childhood and occurring in children with previously normal language development. Oct 19, 2017 the landau kleffner syndrome or acquired aphasia with convulsive 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. Landaukleffner syndrome lks is a rare childhood disorder and is. The syndrome of acquired verbal auditory agnosia in childhood with mutism and epileptic discharges has been described in over 100 cases. 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 rare disorder of communication. Landau kleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years.
Many children with lks also have seizures, episodes of uncontrolled body movement. Children with lks typically develop normally until signs and symptoms of the syndrome begin to develop between age 2 and 8 years. Key points about landau kleffner syndrome in a child. Med any combination of signs and symptoms that are indicative of a particular disease or disorder syndrome, mr. The speech and language skills may improve over time. An encephalitic etiology has often been postulated but never proved. Landaukleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parieto. Methods a prospective pilot study of ivig therapy for landau kleffner syndrome was undertaken. Landau kleffner syndrome lks or acquired epileptic aphasia was first described.
Landau kleffner syndrome lksacquired epileptic aphasia aea typically develops in healthy children who acutely or progressively lose receptive and. Landau kleffner syndrome causes loss of ability to understand and use language, and it can cause seizures. The purpose of this study was to investigate the usefulness of magnetoencephalography meg for. Lks usually starts between 3 and 6 years and affects twice as many boys as girls. Children diagnosed with landau kleffner syndrome based on the international league against epilepsy criteria 7 at the american university of beirut medical center were recruited into the study. Effect of early corticosteroid therapy for landaukleffner syndrome. Landaukleffner syndrome lks is a childhood disorder characterized by an acquired aphasia that emerges in association with epileptiform electroencephalographic abnormalities. Polkey introduction landau kleffner syndrome lks and the syndrome of continuous spikesandwaves during slow sleep csws were described independently and have been considered separate disorders. Differential diagnoses include deafness, an acute behavioural or psychiatric dis. Landaukleffner syndrome lks is characterized by the sudden or gradual development of aphasia the inability to understand or express language. Aug 11, 2017 landau kleffner syndrome lks is characterized by the sudden or gradual development of aphasia the inability to understand or express language. We report two patients with this syndrome who were treated surgically. The description of landau kleffner syndrome lks has profoundly influenced not only clinical practice but also cognitive neuroscience.
Landau kleffner syndrome nord national organization for rare. These children will have developed speech normally and then, suddenly or gradually lose language skills for no apparent reason. 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. Schools for the deaf were in the majority oralist in their approach, and the use of sl was forbidden lane, 1993. 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. Despite its relatively low incidence and prevalence, the landau kleffner 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. Acquired desmielinizahte aphasia landau kleffner syndrome due to neurocysticercosis. Jul 29, 2009 so far, the therapy of choice for landau.
It is concluded that the existence of an acquired aphasia and the discharges observed in the electroencephalogram are very important for the diagnosis of this syndrome. 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. Landaukleffner syndrome lksalso called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorderis a rare childhood neurological syndrome. Landaukleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions. Typically, a care team for children with lks consists of a neurologist, a neuropsychologist, and a speech pathologist or audiologist. Its main features are a loss of speech and language skills, with seizures and learning and behaviour problems. 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. At the time of assessment, frank was no longer suffering from primary symptoms of lks. Bilateral volume reduction of the superior temporal areas in landau kleffner syndrome. Lks usually occurs in children between ages 3 and 7 and can affect the childs ability to speak, understand language or both.
Kleffner syndrome lks or the syndrome of continuous spikes and waves during sleep csws syndrome has not been clearly established. Children affected with lks have developed normally until signs and symptoms begin between the ages of 2 and 8. Jan 04, 2019 landaukleffner lwndau is characterized by acquired aphasia and electroencephalographic abnormalities during wakefulness and sleep. Landau kleffner syndrome lks is a rare and underdiagnosed epilepsy syndrome. Systemic examination, including neurological evaluation, was normal. In additional to language regression, the diagnosis requires the presence of severely epileptiform activity on eeg, particularly during nonrem sleep. 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. Secondary symptoms include psychomotor or behavioral disturbances and epilepsy with a favorable outcome for seizure control. Pdf on oct 18, 2019, maria rosaria muzio and others published landau kleffner syndrome find, read and cite all the research you need on researchgate. Social skills training for a young adult with landaukleffner. Kleffner, coauthor of their initial paper and former director of the central institute for the deaf in saintlouis, missouri, u. Landaukleffner syndrome was first described in a series of cases described in 1957 by william landau, a neurologist at washington university, and frank kleffner, a speech pathologist at st louis central institute for the deaf. In this case report, we describe the anesthetic management of a child with a diagnosis of landaukleffner syndrome.
Landau kleffner syndrome boston childrens hospital. When seen, seizures are typically infrequent may only occur once, nocturnal and selflimiting remission is common by 10 years of age. 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. A landau kleffner syndrome lks is a rare disorder also known as acquired epileptiform aphasia. Landau kleffner syndrome nord national organization for. A major feature of lks is the gradual or sudden loss of the ability to understand and use spoken language. Therefore, we recommend a sleep electroencephalogram in all children with 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. In a study of 77 cases of landaukleffner syndrome, 6 were found to have this type of aphasia. This disorder impairs the ability of the child to understand instructions and be able to communicate to others. Landau kleffner syndrome responsive to levetiracetam. Affected siblings and discordant monozygotic twins have been reported rarely. We present the differential diagnosis and treatment of frank, a 26yearold man with landaukleffner syndrome lks.
Landau kleffner syndrome epilepsy action australia. Children with lks lose the ability to talk and understand speech. This syndrome is also known as progressive epileptic aphasia and aphasia with convulsive disorder. 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. Find out information about landau kleffner syndrome. We report a case of lks in an 7yearold boy who presented with aggressive behavior, difficulty in maintaining posture, and language regression. Landaukleffner syndrome lks or acquired epileptic aphasia is a childhood disorder in which. At the last control, 26 patients had remained seizure free. Despite careful search, no pathologic evidence of encephalitis was found. Acquired epileptic aphasia, verbal auditive agnosia, enfermeda encephalopathy, landau kleffner s syndrome. General anesthesia for a child with landaukleffner. It usually occurs in children between the ages of 3 and 9 and affects the area of the brain that controls speech and comprehension. 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 definition of landaukleffner. Know the causes, symptoms, treatment, prognosis and recovery of landau kleffner syndrome. 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. Landaukleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. The epileptiform activity in these children is thought to result in a functional ablation of eloquent speech areas. Various antiepileptic drugs aeds have been tried, some of them with moderate success sodium valproate, benzodiazepines, ethosuximide, acetazolamide, levetiracetam. Landaukleffner syndrome article about landaukleffner. Insights into possible genetic factors gabrielle rudolf. Landau kleffner syndrome lks is a very rare epilepsy syndrome of childhood. It is named after william landau and frank kleffner, who characterized it in 1957 with a diagnosis of six children. Landaukleffner syndrome symptoms and treatment of landau. Thelandaukeffnersyndrome 387 diagnosis the diagnosis of landau kleffner syndrome depends largely on being aware that the condition exists, and its usual pattern of presentation. Efficacy of intravenous immunoglobulin in landaukleffner. The description of landaukleffner syndrome lks has profoundly influenced not only clinical practice but also cognitive neuroscience.
General discussion 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. Landaukleffner syndrome lks or acquired epileptic aphasia is a childhood. 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. Magnetoencephalography in children with landaukleffner. Landau kleffner syndrome lks is a rare childhood disorder characterized by. Landaukleffner syndrome epilepsy and seizures jama. Landau kleffner syndrome lks is a condition that shows up during childhood, causing difficulties with speech and seizures.
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