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Landau–Kleffner syndrome
A childhood neurological disorder with language regression and seizures.
Overview
Landau–Kleffner syndrome (LKS) is a rare childhood neurological disorder characterized by the sudden or gradual loss of language comprehension and verbal expression (aphasia), typically after a period of normal development. It is often associated with abnormal electrical activity in the brain, especially during sleep. The syndrome usually manifests between the ages of 3 and 7 and can significantly impair a child’s ability to communicate. Some children also experience seizures. Although LKS is uncommon, early recognition and intervention are crucial to improving outcomes.
Causes
The exact cause of Landau–Kleffner syndrome is not fully understood. It is classified as an epileptic encephalopathy, indicating that the brain dysfunction is linked to abnormal electrical discharges seen in epilepsy. Contributing factors may include:
Abnormal EEG activity – especially during non-REM sleep, which interferes with language processing areas of the brain.
Possible genetic factors – although no single gene mutation has been definitively linked to LKS.
Autoimmune or inflammatory mechanisms – suspected in some cases but not clearly proven.
Symptoms
Children with Landau–Kleffner syndrome typically develop normally until they begin to lose their ability to understand or use spoken language. Symptoms may include:
Auditory verbal agnosia – inability to understand spoken words, even though hearing is normal.
Loss of expressive language – difficulty speaking or forming sentences.
Behavioral issues – including hyperactivity, aggression, and social withdrawal.
Seizures – occur in about 70–85% of cases, often during sleep.
Regression of developmental milestones – particularly in language and communication skills.
Diagnosis
Diagnosing Landau–Kleffner syndrome involves a combination of clinical history, language assessments, and neurological testing. Key diagnostic tools include:
Electroencephalogram (EEG) – shows characteristic epileptiform activity, particularly during sleep.
Speech and language evaluation – to assess the severity and type of language impairment.
Neuroimaging (MRI) – usually normal but helps exclude structural brain abnormalities.
Hearing tests – to confirm that the language issues are not due to hearing loss.
Treatment
Treatment of LKS focuses on managing seizures, reducing EEG abnormalities, and improving language skills. Interventions include:
Antiepileptic drugs (AEDs) – such as valproate, levetiracetam, or benzodiazepines to control seizures and EEG abnormalities.
Corticosteroids – like prednisone, used to reduce brain inflammation and improve language function.
Speech and language therapy – critical for language rehabilitation and communication skills.
Behavioral therapy – to manage associated behavioral issues.
Surgical options – such as multiple subpial transections may be considered in severe, refractory cases.
Prognosis
The prognosis of Landau–Kleffner syndrome varies widely. Some children recover significant language function with early and aggressive treatment, while others may experience persistent communication difficulties into adulthood. The severity of EEG abnormalities, age at onset, and duration of aphasia all influence the long-term outcome. Seizures often improve or resolve by adolescence, but language recovery is less predictable. Lifelong support may be required in cases with lasting language or behavioral impairments.
Medical Disclaimer
The information provided on this page is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition.