Landau-Kleffner syndrome (also known as “infantile acquired aphasia,” “acquired epileptic aphasia (AEA),” and “aphasia with convulsive disorder”) is a rare childhood neurological disorder. It is characterized by the gradual or sudden deterioration or regression of the ability to use or comprehend spoken language and an abnormal electroencephalogram (EEG) pattern. The disorder results in an impairment of receptive language, with an impairment of expressive language thought to be secondary to the receptive disorder. The onset of the language problem typically occurs between the ages of 2 and 8. Usually, the children develop normally and then, for no apparent reason, they lose the ability to understand others, referred to as auditory agnosia or “word deafness,” and then the ability to speak. Some children with classic Landau-Kleffner syndrome retain normal social play and reciprocal interaction. Approximately 80% of the children with Landau-Kleffner syndrome (LKS) have a history of one or more epileptic seizures that usually occur at night.
The EEG abnormalities in LKS are thought to underlie a disturbance involving cerebral integration or the loss of the ability to process complex auditory signals such as speech, but the exact cause of LKS is unknown and even with successful treatment of the seizures associated with LKS, the aphasia does not typically resolve. Some suggested causes are demyelinating or encephalopathic processes, infectious or inflammatory illnesses, unilateral brain lesions, and autoimmune diseases.
Landau-Kleffner syndrome is commonly diagnosed using an electroencephalogram (EEG), a scan that shows the brain’s electrical waves, as well as other diagnostic tests. Many clinicians consider the presence of continuous spoke-wave during slow-wave sleep during the period of language regression a requirement for the diagnosis of LKS. A normal EEG, particularly during wakefulness, need not necessarily rule out the diagnosis. Other tests that look at brain function in different ways, including Single Photon Emission Computed Tomography (SPECT), Positron Emission Tomography (PET), or magnetoencephalography may be needed to reveal the abnormalities associated with LKS.
The importance of a careful and thorough history cannot be overemphasized in differentiating LKS from other developmental disorders. LKS is difficult to diagnose and may be misdiagnosed as autism, continuous spike-wave discharges during slow-wave sleep (CSWS), pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, mental retardation, childhood schizophrenia, or emotional/behavioral problems. In the case of autism, however, autistic children typically show other distinctive symptoms that should differentiate them from children with language impairments only. These include impairments in nonverbal communication as well as verbal communication, stereotyped and repetitive behaviors, unusually focused interests, and impairments in social skills. Children with LKS remain socially interactive, although some develop behavior problems, and their language loss usually occurs after the development of phrases, as opposed to children with autistic disorder that leave earlier language regression. Unlike people with developmental disorders and hearing disorders who often do not develop speech normally from the start, a child with LKS will begin to develop normally and then deteriorate and will often have a history of seizures.
All children with LKS have an abnormal EEG compatible with a diagnosis of epilepsy even for those children without clinical seizures. Epileptic attacks in children with LKS are usually brief, easily controlled, and generally do not progress to status epilepticus. The seizures usually stop by the time the child becomes a teenager. There is often functional asymmetry with a change in hand dominance and general clumsiness and poor coordination when the disorder is at its worst.
Hearing and intelligence are usually confirmed to be normal in children with LKS. Nonetheless, the communication problems associated with LKS may lead to or be accompanied by behavioral or psychological problems including hyperactivity, aggressiveness, depression, decreased attention, and social withdrawal, and the interference with normal brain functioning may jeopardize academic achievement or social adjustment. Behavioral disturbances may be in the forefront and mask the language problem, mainly in very young children. Some children can show psychotic behavior and also can have a more global cognitive regression.
LKS may be associated with head trauma, stroke, neurocysticercosis, etc. The presence of other neurological deficits such as hemiparesis or signs of increased intracranial pressure may be a clue for an underlying structural lesion. Imaging, especially MRI, will clarify the etiology if caused by structural changes. Also, people with neurodegenerative diseases may occasionally present with problems in language comprehension.
A multidisciplinary approach to identification and rehabilitation is encouraged in order to effectively reestablish communication skills for children with LKS. Ideally, this should include audiology, otology, psychology, neurology, and speech-language pathology. The team will need to regularly assess speech/language ability/progress, hearing, mental health, behavior/development, cognitive-behavioral therapy, support services of caregivers, and client and/or caregiver education, and consider psychopharmacological or surgical interventions. Psychotherapy and psychiatric consultation may be indicated in selected people with LKS in whom the secondary behavioral problems need pharmacological intervention. Patient should be considered for referral as a candidate for supplementary security income (SSI).
Anticonvulsants used to control the seizure usually have very little effect on language recovery. Carbamazepine may exacerbate clinical seizures, but valproic acid can be helpful. Corticosteroid therapy (ACTH or prednisone) has improved the language ability of some children. IVIG has been suggested as a possible treatment.
Surgical therapyhas been associated with improvement in language and seizure control. A controversial treatment option involves a surgical technique called multiple subpial transection (MST) in which the pathways of abnormal electrical brain activity are severed. This treatment has not been proven superior to other therapeutic options.
Speech/language therapy is important and should be started as soon as possible. The speech and language therapist has an essential role in the management of children with LKS. Learning sign language can diminish anxiety and improve socialization, and it does not appear to delay the potential recovery of speech. Some children quickly develop a natural sign language, which, if encouraged and used with the child, can prevent or decrease the secondary behavioral difficulties.
Visuo/spatial strengths should be utilized in therapies that will allow associations and categorizations necessary for learning and visual memory storage. An aphasic multimodal sensory approach—including Signing Exact English, pictures, printed word, computers, a communication board, and vocabulary therapy– is to be utilized for maximum progress. Directions should be explained with a multiplicity of visual cues.
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The Tarjan Center for Developmental Disabilities at UCLA,
Robin L. Hansen, M.D.
Theodore A. Kastner, M.D., M.S.
Felice Weber Parisi, M.D., M.P.H.
Jaime Mejlszenkier, M.D., F.A.A.N.
Mary B. Tierney, M.D.
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