Landau kleffner syndrome boston childrens hospital. The landau kleffner syndrome is a rare syndrome of unknown etiology. It also blindsides them with autisticlike symptoms and severe aggression. Pipers journey with lks all started one afternoon while picking her up at preschool. Sebects, landau kleffner syndrome lks, and epileptic encephal. 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. Initially, these individuals have a healthy, problemfree development with normal speech and vocabulary. The disorder is characterised by gradual or rapid loss of language in a previously normal child. Landau kleffner syndrome is not an eponymic badge of ignorance. This disorder impairs the ability of the child to understand instructions and be able to communicate to others. Landau kleffner syndrome nord national organization for. As a syndrome, epilepsy with csws is defined as a combination of this.
Most patients appeared normal before the onset of the syndrome. Oct 19, 2017 is cerebral arteritis the cause of the landaukleffner syndrome. It is named after william landau and frank kleffner, who. Little research exists about its causes, effective intervention strategies, and transition planning.
Differential diagnoses include deafness, an acute behavioural or psychiatric dis. Affected siblings and discordant monozygotic twins have been reported rarely. Child with landau kleffner syndrome misdiagnosed as. Almost as if to confirm this passing thought, piper burst out in a string of complete jibberish and became. Landaukleffner syndrome will be discussed in regard to symptoms, aetiology, therapy and prognosis.
Landaukleffner syndrome is characterized by difficulty in receptive or expressive language, abnormal electroencephalograms, and seizures. Landau kleffner syndrome lks is a rare childhood disorder characterized by the loss of. He has landaukleffner syndrome, hypotonia of his fingers and hands, and dyspraxia. Slow wave sleep and landaukleffner syndrome protocol. Landaukleffner syndrome lks is a rare epileptic syndrome, reported by landau and kleffner in 1957, 1 characterized by the association of receptive aphasia, epileptic seizures in most cases, behavioral disorders and electroencephalographic changes with discharges at the temporal lobe unilaterally or bilaterally.
Raising awareness and pursuing research into landau kleffner syndrome. Pdf epileptic encephalopathies in infants and children. 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. Landau kleffner syndrome lks is a rare form of childhood epilepsy which results in a severe language disorder, a form of aphasia, loss of language, and usually develops between 3 and 7 years. Epileptic seizures are infrequent and not a prerequisite for landaukleffner syndrome. It occurs in previously normal children, usually between three and seven years of age. Magnetoencephalography in children with landau kleffner syndrome and acquired epileptic aphasia. Landau kleffner syndrome is characterized by difficulty in receptive or expressive language, abnormal electroencephalograms, and seizures. 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.
He has landau kleffner syndrome, hypotonia of his fingers and hands, and dyspraxia. 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 spike. The landaukleffner syndrome has been defined as an acquired epileptic aphasia or auditory agnosia. 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. With lks overnight seizures in the temporal lobes rob children of their speech. Child with landau kleffner syndrome misdiagnosed as autism. Landaukleffner syndrome, epilepsy with continuous spikeandwaves. 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. It is accompanied by abnormal electroencephalogram eeg and behaviour symptoms of autism. The landaukleffner syndrome is a rare syndrome of unknown etiology. Landaukleffner syndrome lks or acquired epileptic aphasia is a childhood. What is landaukleffner syndromecausessymptomstreatment. They reported language improvement concordant with eeg improvement and suggested a functional ablation of language areas by persistent.
A child with lks has a progressive loss of language abilities understanding and speaking after previously developing normally for their age. 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. Schools for the deaf were in the majority oralist in their approach, and the use of sl was forbidden lane, 1993. Pdf benign epilepsy with centrotemporal spikes bects is the most common type of focal epilepsy in children. Kleffner, coauthor of their initial paper and former director of the central institute for the deaf in saintlouis, missouri, u. It is named after william landau and frank kleffner, who characterized it in 1957 with a. Landaukleffner syndrome lks, also called infantile acquired aphasia, acquired epileptic aphasia 1 or aphasia with convulsive disorder, is a rare, childhood neurological syndrome. The landaukleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957. It is named after william landau and frank kleffner, who characterized it in 1957 with a diagnosis of six children. Landau kleffner syndrome nord national organization for rare. It usually occurs in children between the ages of 3 and 9 and affects the area of the brain that controls speech and comprehension. Know the causes, symptoms, treatment, prognosis and recovery of landau kleffner syndrome.
Know the causes, symptoms, treatment, prognosis and recovery of landaukleffner 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. On this particular day, i happened to notice that many of the other kids pipers age had a much more expansive vocabulary at 2 12 than i had been observing from piper. Thelandaukeffnersyndrome 387 diagnosis the diagnosis of landaukleffner syndrome depends largely on being aware that the condition exists, and its usual pattern of presentation. Objectives define landaukleffner syndrome lks understand course of lks osymptoms otreatment oprognosis neurodiagnostic studies 3. Landaukleffner syndrome lks is a rare entity characterized by epilepsy and aphasia.
Landau kleffner syndrome lks usually has an onset between three and seven years old. Landau kleffner syndrome is characterized by acquired aphasia and paroxysmal, sleepactivated eeg paroxysms predominating over the temporal or parietooccipital regions. These children will have developed speech normally and then, suddenly or gradually lose language skills for no apparent reason. Hoeppnerepileptic encephalopathy of late childhood. Getting insurance to cover various therapies a landau kleffner syndrome lks is a rare disorder also known as acquired epileptiform aphasia. The correlation between paroxysmal eeg discharges and language deterioration was first suggested by landau and kleffner 1957, who reported five children with acquired aphasia associated with a convulsive disorder 1. Landaukleffner syndrome is manifested as a form of aphasia, loss of language, which usually develops between 3 and 7 years. However, there is a high risk of significant residual language impairment. Landaukleffner syndrome lks, or acquired epileptiform aphasia, is an epilepsy. 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. Secondary symptoms include psychomotor or behavioral disturbances and epilepsy with a favorable outcome for seizure control. This classical syndrome is well recognized in pediatric neurology but the diagnosis is less well known to pediatric otolaryngology, speech language pathology and audiology services. Landau kleffner syndrome is characterized by subacute onset of acquired aphasia in a child with normal previous development and cognition.
Landaukleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. In 1957, landau and kleffner initially described acquired epileptic aphasia and subsequently reluctantly agreed. Landaukleffner syndrome, electroencephalogram, prednisone, language, children, aphasia. Landau kleffner syndrome what is landaukleffner syndrome. 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. Landaukleffner syndrome and the syndrome of continuous spikes and waves during slowwave sleep j. Landau kleffner syndrome lks is a rare entity characterized by epilepsy and aphasia. Specifically, the eeg typcally shows an increase to nearly continuous abnormal brain activity firing spikes. The landau kleffner syndrome or the syndrome of acquired epileptic aphasia was first described in 1957.
It has been diagnosed primarily in young children, usually beginning at age 3 years or older. Icd10cmpcs codes version 201620172018, icd10 data search engine. 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. Lks usually occurs in children between ages 3 and 7 and can affect the childs ability to speak, understand language or both. Seizures may not occur in all cases, and when present are infrequent and selflimiting. Landaukleffner syndrome lks is a rare neurological disorder that causes a loss of language skills aphasia. Signssymptoms kids are healthy and problem free until the. Acquired epileptic aphasia in children anne marie tharpe barbara j. All children have abnormal eeg compatible with the diagnosis of epilepsy, however, only 70% have clinical seizures. You wont find landau kleffner variant in the medical diagnostic manuals though. My daughter developed normally and hit all of her milestones on time or early.
Feb 18, 2011 landau kleffner syndrome, online mendelian inheritance in man omim hirsch e, valenti mp, rudolf g, et al. Landaukleffner syndrome is a rare disorder that affects twice as many males as females. The cause of landaukleffner syndrome is unknown although a spectrum of epileptic conditions. What every educator should know about landaukleffner syndrome. This article describes the primary and secondary characteristics of children with this syndrome and offers educators a framework for intervention techniques. To describe the clinical presentation and treatment of 3 children with an auditory processing disorder with an identifiable neurological cause. Landaukleffner syndrome lks or acquired epileptic aphasia is a childhood disorder in which acquired aphasia and epileptiform electroencephalographic eeg abnormalities are associated. 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. Acquired childhood aphasia with focus on landaukleffner. Sebects, landaukleffner syndrome lks, and epileptic encephal.
Landau kleffner syndrome lks is a rare and underdiagnosed epilepsy syndrome. Landaukleffner syndrome lks usually has an onset between three and seven years old. Landaukleffner syndrome acquired aphasia with epilepsy by genevieve v. Onset occurs between 18 months and years of age, with threefourths of the cases appearin g before the age of 7 years. A theory is that it is caused by a dysfunctional immune system, exposure to a virus, and brain trauma. Magnetoencephalography in children with landaukleffner syndrome and acquired epileptic aphasia. Getting insurance to cover various therapies a landaukleffner syndrome lks is a rare disorder also known as acquired epileptiform aphasia. Objectives define landau kleffner syndrome lks understand course of lks osymptoms otreatment oprognosis neurodiagnostic studies 3. Landau kleffner syndrome lks begins between 2 and 8 years of age average 5 to 7 years. There has never been any family history of lks so it is most likely not inherited. Children with lks typically develop normally until signs and symptoms of the syndrome begin to develop between age 2 and 8 years. These include using predictable language, creating the need to communicate, and. It has been diagnosed primarily in young children, usually beginning at.
Olson f abstract acquired epileptic aphasia, or landaukleffner syndrome lks, once thought to be a rare syndrome, may occur more frequently in the pediatric population than once thought. 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. In additional to language regression, the diagnosis requires the presence of severely epileptiform activity on eeg, particularly during nonrem sleep. Selective epileptic gait disorder journal of neurology. Landau kleffner syndrome is an example of a rare severe and challenging disability. 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. Nonconvulsive aspects of epilepsy in children maastricht university. Managing landau kleffner syndrome landaukleffner syndrome lks is an acquired epileptic aphasia of childhood and is a rare, childhood neurological syndrome. Landau kleffner syndrome is manifested as a form of aphasia, loss of language, which usually develops between 3 and 7 years people with landau kleffner syndrome have abnormal eeg patterns involves examining the persons eeg patterns during sleep often diagnosed in conjunction with autism, is the failure to respond to sounds. The landau kleffner syndrome has been defined as an acquired epileptic aphasia or auditory agnosia. It can also be seen as early as 18 months of age or as late as 14 years. Landau kleffner syndrome is manifested as a form of aphasia, loss of language, which usually develops between 3 and 7 years.
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. What every educator should know about landaukleffner. Landaukleffner syndrome lksalso called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorderis a rare childhood neurological syndrome. Landau kleffner syndrome acquired aphasia with epilepsy by genevieve v.