What Is the Difference Between LKS and Autism?

Landau-Kleffner syndrome (LKS) is not the same as autism; however, their symptoms are similar, so distinguishing between them can be challenging. An electroencephalogram (EEG) recorded when a child is awake and asleep can often differentiate between the two conditions.

LKS may be mistaken for autism, but it appears later in childhood and does not involve social difficulties.

• Autism spectrum disorder(ASD) is a group of developmental disorders characterized by various signs, symptoms, and disabilities.
• LKS, also known as acquired epileptic aphasia, is a pediatric disorder characterized by epileptiform EEG abnormalities and acquired aphasia (impaired understanding and expression of language).

Autism symptoms may manifest early in LKS, leading to misdiagnosis and further neurocognitive deterioration and seizures. Children with LKS are often misdiagnosed with ASD, particularly infantile autism or regressive forms.

Autistic regression refers to children who lose language and social communication skills, developing a repetitive and constrictive pattern of interests and behaviors consistent with ASD. However, there is no precise definition of autistic regression, and there is ongoing debate about its prevalence.

ASD vs. LKS differences chart

Children with LKS typically maintain eye contact and appear engaged with their surroundings, but they may struggle to comprehend their environment. In contrast, children with ASD generally do not engage with others.

Differences between children with autistic regression and those with an epileptic encephalopathy like LKS are summarized below:

In awake mode: Normal or posterotemporal interictal epileptiform discharges (IEDs) can be observed

Electrical status epilepticus in sleep is rare.

Multifocal, independent, infrequent EEG abnormalities are observed

Studies have found that individuals with ASD who have no history of seizures are more likely to have epileptic disorders than those who do. However, there is considerable debate about how these findings specifically apply to the ASD phenotype and whether or not regression occurs.

Additionally, some studies suggest that antiepileptic drugs can improve behavior, but there is currently no evidence to suggest that treating epileptic disorders improves social, language, cognitive, or behavioral outcomes.

QUESTION

What are the common signs and symptoms of LKS?

Landau-Kleffner syndrome (LKS) is characterized by the sudden or gradual onset of aphasia (inability to understand or express language), seizures, and behavioral issues.

Common signs and symptoms of LKS

• Aphasia: The loss of auditory receptive language is typically the first indication of a language problem (auditory verbal agnosia). It is characterized by:

• Loss of ability to understand spoken language
• Failure to respond to verbal communication
• Inability to recognize familiar noises
• Inability to lateralize or localize sound
• Receptive language impairment
• Profound expressive language impairment
• Reduced use of complex sentences, jargon speech, word-finding deficits, dyssomnia, paraphasias, and mutism
• Many affected individuals experience clinical seizures
• Some have only electrographic seizures, including electrographic status epilepticus of sleep. About 70 percent of children with LKS experience seizures, typically during sleep.
• Hyperactivity
• Uncontrolled temper or anger
• Withdrawal from social interaction
• Poor attention span
• Negativity, nightmares, tantrums, depression, and withdrawal
• Children with LKS often have low IQ
• Impaired short-term memory is a recorded feature in long-standing cases

LKS is a rare neurological disorder in which the parts of the brain responsible for speech and comprehension are affected. As a result, aphasia and abnormal electroencephalogram results are typically present in LKS patients.

In addition, some patients lose the ability to identify environmental sounds and may have auditory agnosia. The symptoms of LKS may resemble those of other conditions, so it is always advisable to consult a doctor about any signs and symptoms a child may exhibit.

What are the causes and risk factors for LKS?

The causes and risk factors for Landau-Kleffner syndrome (LKS) are still debated and researched. Some experts believe this disorder has multiple causes.

Potential causes and risk factors for LKS include:

• Epileptic activities in the brain: Children with LKS appear normal until they have their first seizure or develop language problems. It is believed that LKS is caused by epileptic activity in the brain region responsible for speech and language comprehension.
• The causes of these epileptic activities (or the secondary causes of LKS) include:

• Head trauma, primarily closed head injuries
• Underlying autoimmune disease
• Neurocysticercosis
• Demyelinating diseases
• Some parents report that their children had a viral infection before the onset of LKS, but diseases have not been proven to cause the condition. Some children improve with anti-inflammatory treatment, suggesting that inflammation may play a role.

Although the cause of LKS is still unknown in 85 percent of cases, researchers have made significant progress in understanding the disease.

What are the treatment options for children with LKS?

Treatment for LKS depends on the child’s symptoms, age, overall health, and the severity of their condition. While there is currently no cure for LKS, supportive care is available.

Treatment options for LKS may include medical, surgical, and pharmacological management.

Here are a few treatment options for LKS:

• Steroids: Often used to treat inflammation and seizure disorders like LKS. Steroids can help with EEG electrical activity, language problems, and behavioral issues.
• Epilepsy medications: These drugs may help control seizures and brainwave activity and can improve language problems.
• Intravenous immunoglobulins (IVIG): Antibodies extracted from donated blood that can be beneficial in some cases of LKS.
• Antiepileptic drugs: These drugs are often administered during EEG studies when seizures are suspected. They have been reported to modulate and reduce disruptions in brainwaves in children with LKS.
• Behavioral therapy: Often recommended to help with behavioral issues such as anxiety.
• Brain surgery: A type of surgery that severs nerve fibers within specific brain regions responsible for generating epileptic activity. This treatment is reserved for those who have not responded to multiple medical therapies but have shown significant improvement in language skills and behavior in some cases.
• Speech therapy: Required for those with special educational needs. Speech therapy, including sign language and other interventions, can help.
• Complementary and alternative therapies: May be tried alongside epilepsy medications and surgery. Results may vary depending on the individual’s condition.

What is the outcome of children with LKS?

The progression and severity of LKS vary between cases. Some children permanently lose their ability to speak, while others are only briefly affected. In some cases, it may take years for a child’s language skills to recover.

Symptoms may come and go in certain cases. Seizures and abnormal brainwave activity typically resolve by the time the child reaches adulthood. Children who develop symptoms after the age of six have the best chance of recovery.

Early intervention with speech therapy has been shown to yield better outcomes.

There is no international consensus on how to treat LKS, but early initiation of drug therapy has been shown to improve prognosis. Communication is crucial for a child’s interactions with family, peers, and others, as well as for their behavioral and psychosocial functioning. This requires the early development of alternative and augmentative communication strategies.

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