LANDAU-KLEFFNER SYNDROME

LANDAU KLEFFNER SYNDROME

Landau Kleffner syndrome (LKS) is an unusual childhood condition distinguished by a lack of language understanding (auditory-visual agnosia) and visual speech (aphasia) in conjunction with extreme irregular electroencephalographic (EEG) results in most patients throughout sleep and clinical seizures.

This condition is also defined as progressive convulsive epileptic aphasia and aphasia. LKS usually begins in children aged between 2 and 8 years. The most common starting age is age 5 to 7 years. Age refers equally to both sexes. A slow or unexpected loss of the ability to understand words or things familiar sounds are usually heard in a child who has grown properly. Although it most often affects the child’s understanding of spoken language, it can impair both speech comprehension and speaking capacity.

TYPES

Seizures with this epilepsy syndrome occur in 3 out of 4 babies. The most common form of seizure seen in LKS is focal motor seizure. Focal seizures can become tonic-clonic seizures, affecting the whole body. Atypical absence (starter epilepsy) and atonic convulsions (a loss of tone which often leads to a fall) were recorded. Usually, seizures occur in sleep and aren’t very common.

 

SYMPTOMS

Landau-Kleffner syndrome (LKS) is distinguished by sudden or progressive development of aphasia (incapacity to comprehend or express language). Children afflicted with LKS have signs and symptoms that begin between 2 and 8 years of age. This syndrome also features an abnormal electroencephalogram (EEG), notably during sleep. Around 70 percent of LKS children have seizures. LKS-related seizures are known as complex partial, generalized clonic, and atypical seizures of absence, and are typically easy to manage with treatment.

Some kids with Landau-Kleffner syndrome can experience behavioral issues, including impulsivity, attention deficiencies, temperature outbursts, impulsiveness, and behaviors withdrew. Some kids with Landau Kleffner syndrome can also have an intellectual disability. Health problems associated with LKS can differ, often within the same family.

DIAGNOSIS

The diagnosis of LKS requires an EEG (electroencephalogram) and, in some instances, an extended EEG or video EEG to catch sleep. EEG is abnormal in children with this epilepsy syndrome, especially as they rest. Epilepticus in Sleep (ESES) electrical status is healthy. Usually, the areas where these spikes occur are high amplitude pulse and sharp waves in the temporal and parietal lobes. Those are typically speech-related areas. If only the EEG is performed when the child is alive, this diagnosis can be missed. You should order the genetic and metabolic tests. Usually, a scan with MRI (magnetic resonance imaging) commonly has an average result.

In some cases, LKS is initially misdiagnosed as autism, chronic developmental disorder, hearing impairment, learning disability, hearing / verbal processing disorder, attention deficit disorder, mental illness, childhood schizophrenia, or emotional/behavioral problems.

 

TREATMENT

Anti-seizure drugs may be used to treat convulsions in children with LKS. Suppressing the very regular seizures during sleep is very important too. These seizures are suspected of leading to regular LKS speech and mental issues. It is for this reason that many anti-seizure medicines are not sufficient. In rare cases, surgery can be performed.

The drugs often used are overnight opioids or a high dose of diazepam. Clobazam, ethosuximide, valproic acid, acetazolamide, and levetiracetam are other drugs used to treat this epilepsy condition.

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