Children who experience LKS have average development, yet lose their language skills; apparently for no reason.
Defining Landau-Kleffner Syndrome (LKS)
Landau-Kleffner syndrome (LKS) is a form of rare, childhood neurological disorder characterized by gradual or sudden inability to express language or understand it, also referred to as, 'aphasia,' as well as an abnormal electroencephalogram (EEG). LKS affects the portions of the child's brain that controls their speech and comprehension. The disorder commonly occurs in children who are between the ages of five and seven.
Children who experience LKS have average development, yet lose their language skills; apparently for no reason. Many of the children affected by LKS also experience seizure activity, although some do not. LKS is a difficult disorder to diagnose and can be misdiagnosed as pervasive developmental disorder, autism, learning disability, hearing impairment, attention deficit disorder, auditory/verbal processing disorder, childhood schizophrenia, mental retardation, or emotional/behavioral problems.
The presence of additional forms of aphasia complicates achievement of a diagnosis of LKS. Acquired childhood aphasia involves a child's loss of ability to understand words or use language due to a brain injury. Acquired means the aphasia has happened after the child has already begun to develop language skills. Landau-Kleffner syndrome is an acquired epileptiform aphasia. Aphasia may also occur in children who have experienced an infection such as encephalitis, a brain tumor, head trauma, cerebrovascular accidents, or additional brain disorders. Researchers have not discovered a connection between aphasia and a child's gender, age, or race.
Causes of Landau-Kleffner Syndrome
The human brain and some basic information about can help where understanding the causes of aphasia is concerned. The cerebrum is the main portion of the brain and is divided into halves referred to as the right and left hemispheres. The cerebrum is further divided into lobes, which are referred to as the parietal, frontal, temporal and occipital lobes. In the majority of people, language control is located in the left hemisphere of the brain, called the, 'language-dominant,' area of the brain. In this portion of the brain, people store information about the meaning of words, how and when to use words properly, as well as how to formulate both written and spoken language.
Damage to the left hemisphere of a person's brain many times results in the symptoms of aphasia. Research; however, has demonstrated that a number of left-handed people have language areas in both the left and right hemispheres of their brains. These people may develop aphasia damage to either side of their brain. The types of language skills the person loses, or experiences impairment in relation to, is dependent upon the area of their brain that is injured.
Symptoms of Landau-Kleffner Syndrome
The symptoms of Landau-Kleffner syndrome, as well as other forms of aphasia, can include impaired language functions such as naming, repetition, or speech; all of which are referred to as expressive language. Injuries that cause aphasia may also affect a child's motor speech abilities, affecting abilities such as the ability to make sounds, or swallowing. Aphasia symptoms in children can start with speech delays, progressing to loss of communication skills in all other areas.
The symptoms of aphasia can be temporary or permanent depending upon the amount of damage to the person's brain. Landau-Kleffner syndrome affects the portions of the brain that control their ability to speak and comprehend. Children with LKS experience average development, yet lose the ability to both speak and understand what other people are saying to them with no apparent cause. Children with LKS may also experience seizures.
Diagnosing Landau-Kleffner Syndrome
When a child experiences a brain injury or a stroke, they are assessed for any affects to their language skills. A doctor might refer a child to a speech-language pathologist, or perhaps a neuropsychologist, who will perform a comprehensive speech and language skills assessment. A pathologist will use materials that are age-appropriate to test the child's receptive and expressive language. The results will be used to determine the area of the child's brain that has been injured, as well as to develop a speech therapy program that is best suited for them. Computed tomography scans (CT) or magnetic resonance imaging (MRI) may be used to map the area of the child's brain that has been affected, or to diagnose a brain tumor.
Landau-Kleffner syndrome may be difficult to diagnose because the symptoms are similar to other forms of neurological disorders such as attention deficit disorder, autism, and mental retardation. LKS is diagnosed when a gradual or sudden development of aphasia in a child is accompanied by abnormal electroencephalogram results.
Treatment of Landau-Kleffner Syndrome
The main form of treatment for most people with aphasia in general is speech therapy, something which is started as soon as possible. There are no medications currently available for the treatment of aphasia, and there is no cure. A speech therapist works with a child affected by aphasia in order to strengthen their remaining language skills and to discover ways to compensate for the skills they have lost. There are various techniques the therapist may use, such as:
Repetition of words
Learning sign language
Exercising the child's facial muscles
Completing reading and writing exercises in workbooks
Flash cards to improve the child's memory of object names
Use of picture of objects and activities to communicate with others
Using computer programs to aid in speech, hearing, and reading comprehension, as well as recall
Surgery is something used only in relation to the cause of aphasia; for example, to reduce pressure from a brain tumor, or to reduce swelling from head trauma. Recovery from aphasia is dependent upon the severity of the brain injury the person has sustained. Aphasia from head injury results in the best prognosis, while aphasia from damage to both sides of a person's brain presents the worst prognosis in general. Research has demonstrated that the undamaged right hemisphere of the brain may assist in recovery when only the left hemisphere is damaged.
Treatment for Landau-Kleffner syndrome involves anticonvulsant medications if seizures are a symptom. Corticosteroids may be administered, as well as speech therapy. One controversial form of treatment that has been used for LKS is referred to as, 'subpial transaction.' The treatment involves the severing of pathways in the person's brain where abnormal electrical activity exists.
The prognosis for children with LKS varies. Some children who experience Landau-Kleffner syndrome might have a permanent language disorder; others may regain much of their language abilities, although it could take months or even years to do so. Some children with LKS who have regained language abilities may experience remission or relapse. The prognosis of children with LKS is improved when the onset of the disorder is after the age of six and speech therapy is started as early as possible. Seizure activity associated with LKS commonly disappears by the time the child reaches adulthood.
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