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EEG in Epilepsy: Landau-Kleffner Syndrome

  • Roya Tompkins
  • 2 days ago
  • 2 min read

Next up in our EEG in Epilepsy Series: Landau-Kleffner Syndrome - also know as LKS.


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Landau-Kleffner is a rare age-related epileptic encephalopathy. It usually first shows itself in children aged 3 - 8 years of age that previously were developing normally.


The disorder is characterized by:

  • acquired aphasia (loss of language) 

  • epileptiform EEG abnormalities. 

  • and cognitive deficits




EEG plays a crucial role in diagnosis, often revealing abnormalities particularly during sleep:

A key feature of LKS is the increase in spike and wave activity during sleep, sometimes referred to as continuous spikes and waves during slow sleep (CSWS) or electrical status epilepticus in sleep (ESES). 


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Capturing a sleep recording during the EEG is essential for this diagnosis.


This EEG activity becomes much more widespread and intense during non-REM sleep when it shows an almost continuous spike-wave pattern with a frequency of 1.5 to 2.5 spikes/second. 


However, seizures are not a prerequisite for an LKS diagnosis, and some children with LKS may not have any clinical seizures. 

Up to 30% of patient's with Landau Kleffner do not have seizures - this of course can lead many to not being diagnosed due to an EEG not being ordered. Landau-Kleffner Syndrome (LKS) can be misdiagnosed as autism, particularly in its early stages. Children with LKS may initially present with language regression and behavioral changes that resemble autism spectrum disorder (ASD). However, Landau Kleffener Syndrome is a neurological condition characterized by acquired aphasia (loss of language); often with seizures, and with an abnormal EEG during sleep. 


In Landau Kleffner syndrome, the EEG abnormalities, particularly the increased spike and wave activity during sleep, are thought to contribute to the language regression experienced by the child. 


Treatment for LKS often involves:

  • antiepileptic drugs

  • corticosteroids, 

  • and speech therapy. 


The prognosis for language recovery can vary though. Some children can have significant improvements, while others may have persistent language deficits. 


The EEG plays a vital role in the diagnosis for this syndrome - being aware of the necessity of capturing a sleep recording will best help our physicians and their patients.


For more in our EEG in Epilepsy Series, reference some of our earlier posts below:

Roya Tompkins, MS, REEG/EP T, RPSGT


References:

National Institute of Health

Epilepsy Foundation


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