Table of ContentsView AllTable of ContentsSymptomsCausesDiagnosisTreatmentPrognosis
Table of ContentsView All
View All
Table of Contents
Symptoms
Causes
Diagnosis
Treatment
Prognosis
Landau-Kleffner syndrome (LKS) is a rare brain disorder that affects young children typically between the ages of 2 and 8.LKS causes children to gradually lose their ability to speak and understand language, with many experiencingseizuresand behavioral problems. Symptoms of LKS are frequently mistaken for conditions likeautismor deafness.
This article will cover the symptoms, causes, diagnosis, and treatment of Landau-Kleffner syndrome and what to expect if your child has been diagnosed with this rare disorder.
2022 Classification of LKSIn 2022, the International League Against Epilepsy (ILAE) recommended that Landau-Kleffner syndrome be considered a subtype of a form of epilepsy called DEE-SWAS (developmental/epileptic encephalopathy with spike-wave activation on sleep).The classification defines LKS as part of a spectrum of epilepsy disorders and helps consolidate the treatment approach irrespective of the variations in symptoms.
2022 Classification of LKS
In 2022, the International League Against Epilepsy (ILAE) recommended that Landau-Kleffner syndrome be considered a subtype of a form of epilepsy called DEE-SWAS (developmental/epileptic encephalopathy with spike-wave activation on sleep).The classification defines LKS as part of a spectrum of epilepsy disorders and helps consolidate the treatment approach irrespective of the variations in symptoms.
In 2022, the International League Against Epilepsy (ILAE) recommended that Landau-Kleffner syndrome be considered a subtype of a form of epilepsy called DEE-SWAS (developmental/epileptic encephalopathy with spike-wave activation on sleep).
The classification defines LKS as part of a spectrum of epilepsy disorders and helps consolidate the treatment approach irrespective of the variations in symptoms.
Nusha Ashjaee / Verywell
Landau-Kleffner Syndrome Symptoms
LKS affects children whose language skills were otherwise developing normally for their age. The symptoms tend to develop gradually over the course of weeks or months. Seizures and behavioral changes are also common.
Symptoms of LKS include:
A child with LKS who becomes angry or hyperactive may be doing so in part out of frustration at their inability to communicate clearly or have their needs understood.
What Causes Landau-Kleffner Syndrome?
Landau-Kleffner syndrome, formerly known as acquired epileptic aphasia, is a poorly understood condition that affects roughly one in a million children. Male children are twice as likely to have LKS than female children.
LKS is thought to involve changes in the temporal lobe. This is the part of the brain that helps process emotions, language, and certain aspects of visual perception.
While it is unclear why these changes occur, there is evidence that genetics play a part. Studies have found that around 20% of children with LKS have a mutation in the GRIN2A gene.
The GRIN2A gene directs the production of a chemical receptor called GluN2A which helps modify how nerve signals move through the brain. Mutations of the gene can interfere with this and cause nerve signals to misfire. This might explain why seizures occur but not necessarily the regression of language skills.
The GRIN2A mutation is thought to be a de novo mutation, meaning that a child can acquire it rather than inherit it from their parents.Some experts believe that certain severe viral infections (like therespiratory syncytial viruscommon in children) might trigger the mutation.
How Is LKS Diagnosed?
Landau-Kleffner syndrome may take weeks or months to diagnose because there are more common causes of seizures and language problems that a healthcare provider might suspect. Even when LKS is suspected, other conditions like autism, deafness, and brain tumors need to be excluded before a diagnosis can be made.
Some of the tests used to diagnose LKS include:
Criteria for DiagnosisA diagnosis of LKS will be made based on a child’s history of seizures, the regression of language and communication skills, and the presence of seizure activity on an EEG duringnon-REM sleep.
Criteria for Diagnosis
A diagnosis of LKS will be made based on a child’s history of seizures, the regression of language and communication skills, and the presence of seizure activity on an EEG duringnon-REM sleep.
How LKS Is Treated
The treatment of Landau-Kleffner syndrome is focused on controlling seizures, reducing brain inflammation, and helping to restore speech.
Anticonvulsants
Anticonvulsants, also known as anti-epileptics, are a first-line treatment for LKS. There are several effective options your healthcare provider may prescribe, including:
Corticosteroids
Corticosteroids are thought to be most effective if started as soon as possible after LKS symptoms begin and may even help reverse the loss of language, behavioral, and cognitive skills.
Some healthcare providers recommend a nightly oral dose ofprednisonecombined with abenzodiazepineto help relieve muscle spasms and seizures at night.
Speech Therapy
Although it may be difficult to regain language skills when the temporal lobe is affected,speech-language therapyis nevertheless recommended to help children with LKS optimize their ability to communicate.
Some people who have had LKS continue to benefit from speech therapy during adolescence and adulthood.
Surgery
Children who have persistent, severe seizures in spite of treatment may benefit from epilepsy surgery. This may involve a technique calledsubpial transectionduring which nerve connections at the site of the seizure activity are severed but no brain tissues are removed.
Epilepsy surgery is considered a last resort after all other treatment options have failed.
What Is the Outlook for a Child With LKS?
LKS often improves over time. Most children do not continue to have seizures after adolescence and do not need long-term treatment with anticonvulsants.
However, there is a variable degree of recovery from speech and language deficits. These can affect a person throughout their lives, particularly when LKS starts at an early age or the treatment isn’t successful.
Children treated with corticosteroids or immunoglobulin in the early stages tend to have the best long-term outcomes.
Summary
Landau-Kleffner syndrome is a rare brain disorder affecting one in a million children primarily between the ages of 2 and 8. LKS causes the loss of communication skills, behavioral problems, and seizures. In recent years, it has been cast as part of a spectrum of epilepsy disorders.
11 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Epilepsy Foundation.Landau-Kleffner syndrome.
National Institute of Neurological Disorders and Stroke.Landau-Kleffner syndrome information page.
National Organization of Rare Disorders.Landau-Kleffner syndrome.
Yang X, Qian P, Xu X, et al.GRIN2A mutations in epilepsy-aphasia spectrum disorders.Brain Dev.2018;40(3):205-210. doi:10.1016/j.braindev.2017.09.007
National Center for Advancing Translational Sciences.Landau-Kleffner syndrome.
Carvill GL, Regan BM, Yendle SC, et al.GRIN2A mutations cause epilepsy-aphasia spectrum disorders.Nat Genet.2013 Sep;45(9):1073–1076. doi:10.1038/ng.2727
Mir A, Qahtani M, Bashir S.GRIN2A-related severe epileptic encephalopathy treated with memantine: an example of precision medicine.J Pediatr Genet.2020 Dec;9(4):252–257. doi:10.1055/s-0039-3401028
Fainberg N, Harper A, Tchapyjnikov D, et al.Response to immunotherapy in a patient with Landau-Kleffner syndrome and GRIN2A mutation.Epileptic Disord.2016;18(1):97-100. doi:10.1684/epd.2016.0791
Nemours Children’s Health.Speech-language therapy.
Arts WF, Aarsen FK, Scheltens-de Boer M, et al.Landau-Kleffner syndrome and CSWS syndrome: treatment with intravenous immunoglobulins.Epilepsia. 2009;50 Suppl 7:55-8. doi:10.1111/j.1528-1167.2009.02221.x
Meet Our Medical Expert Board
Share Feedback
Was this page helpful?Thanks for your feedback!What is your feedback?OtherHelpfulReport an ErrorSubmit
Was this page helpful?
Thanks for your feedback!
What is your feedback?OtherHelpfulReport an ErrorSubmit
What is your feedback?
