What are the other Names for this Condition? (Also known as/Synonyms)

• Acquired Childhood Aphasia with Convulsive Disorder
• Acquired Epileptiform Aphasia
• Landau-Kleffner Acquired Epileptiform Aphasia

What is Landau Kleffner Syndrome? (Definition/Background Information)

• Landau Kleffner Syndrome (LKS) is a rare epileptic disorder that affects children under 10 years of age. The disorder results in children losing their ability to talk and understand daily conversation
• Children with LKS have normal language development until the onset of the disorder, when they begin to lose language comprehension and speech production ability. This may occur either suddenly or gradually, over a number of days
• This loss of communication ability is often accompanied by epilepsy, which is a disruption in the brain waves that manifest as seizures. Individuals with Landau Kleffner Syndrome often show abnormal brain wave patterns during sleep, and develop seizures while they are sleeping
• Although the affected children can hear sounds, they fail to process auditory information that they receive. The children are also incapable of deciphering spoken words, which is believed to be caused by abnormal brain function
• This abnormality in brain function is suggested to be caused by irregular electrical activity in the brain. However, it should be noted that this irregular electrical activity may not manifest always as seizures. Therefore, only some LKS-affected children develop seizures
• The administration of anti-epileptic medications can help alleviate the symptoms of Landau Kleffner Syndrome. As the cause of LKS is yet to be understood, there are no well-established preventive measures that can be taken to avoid development of this syndrome
• The prognosis of Landau Kleffner Syndrome is good, if it is diagnosed early; some individuals show a full recovery of speech function with the aid of speech therapy

Who gets Landau Kleffner Syndrome? (Age and Sex Distribution)

• Landau Kleffner Syndrome typically begins to develop when the child is between 3 to 8 years of age
• It is more common in boys, even though both boys and girls are affected; male-female ratio is 2:1
• No specific racial or ethnic group predilection is noted

What are the Risk Factors for Landau Kleffner Syndrome? (Predisposing Factors)

• Currently, no particular risk factor has been identified for Landau Kleffner Syndrome

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Landau Kleffner Syndrome? (Etiology)

The cause of Landau Kleffner Syndrome is yet to be fully understood.

• But, it has been postulated that it is caused by epileptic activities in the brain region that controls speech and language comprehension
• The cause for these epileptic activities may be attributed to head trauma, underlying autoimmune disease, or other unexplained or unidentified factors

What are the Signs and Symptoms of Landau Kleffner Syndrome?

The following signs and symptoms are often experienced by individuals with Landau Kleffner Syndrome:

• Inability to understand spoken words and recognize familiar sounds, like the ringing of a telephone
• Developing seizures while sleeping
• Autism-like behavior such as unresponsiveness to sounds
• Learning capability may be reduced upon onset of the disorder, resulting in lower IQ levels

How is Landau Kleffner Syndrome Diagnosed?

The following tests and exams can be performed for an accurate diagnosis of Landau Kleffner Syndrome:

• A detailed physical examination and evaluation of complete medical history
• Electroencephalography (EEG) and magnetoencephalography (MEG) examination: Both techniques are used to evaluate brain activity. As children with LKS tend to show epileptic activity in brain regions governing speech comprehension during slow sleep period, EEG and/or MEG should be performed while the child is sleeping
• A combination of clinical symptoms together with EEG and/or MEG results showing abnormal brain wave activities within regions that control linguistic development can help confirm a diagnosis of Landau Kleffner Syndrome

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Landau Kleffner Syndrome?

The chief complication associated with Landau Kleffner Syndrome is the loss of language and communication skills.

How is Landau Kleffner Syndrome Treated?

Medications, surgical procedures, and speech therapy may be employed to treat Landau Kleffner Syndrome:

• Antiepileptic drugs have showed to modulate and decrease disruption in brain waves in LKS-affected individuals
• Corticosteroids have also been shown to be effective in reducing epileptic activities within the brain
• In individuals who do not respond to antiepileptic medications, subpial transection should be considered. Subpial transection is a surgery in which the nerve fibers within specific regions of the brain that are responsible for generating epileptic activities are severed, without other neurological activities being affected

Medication or subpial transection surgery may help individuals regain some speech ability, but speech therapy will be needed to assist individuals in regaining normal speech capability.

How can Landau Kleffner Syndrome be Prevented?

• No known preventive measures have been identified for Landau Kleffner Syndrome
• But early diagnosis and intervention can help increase the chances of recovery and reduce linguistic developmental delay

What is the Prognosis of Landau Kleffner Syndrome? (Outcomes/Resolutions)

• The prognosis of Landau Kleffner Syndrome is variable; some children recover fully with no speech impairment at all, while others show mild-to-moderate speech impairment, which extends into adulthood
• The seizures usually disappear as the child gets older

Additional and Relevant Useful Information for Landau Kleffner Syndrome:

• Parents should note that Landau Kleffner Syndrome may often be misdiagnosed as autism. However, there are distinctive behavioral differences between the two disorders; children with LKS display speech and hearing impairment predominantly, while children with autism display abnormality in social interaction and repetitive behaviors
• An accurate diagnosis is crucial and allows for earlier intervention and treatment; thus, consulting a pediatrician early and obtaining a second opinion from other healthcare providers, if necessary, are important measures to be considered