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Landau-Kleffner syndrome.

Abdelkarim A Al-Qudah1

  • 1Pediatric Department, Jordan University Hospital, PO Box 13407, Amman, Jordan. Tel. +962 79566067. Fax. +962 65353388.

Neurosciences (Riyadh, Saudi Arabia)
|February 5, 2013
PubMed
Summary

Landau-Kleffner syndrome, an epilepsy-related language disorder, causes severe hearing comprehension loss. While seizures are manageable with medication, language recovery remains challenging, with autoimmune factors suspected.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Epileptology

Background:

  • Landau-Kleffner syndrome (LKS) is a rare neurological disorder characterized by acquired aphasia in children with epilepsy.
  • It is associated with epileptiform abnormalities on electroencephalogram (EEG), leading to significant receptive language deficits.
  • Patients often exhibit behavioral issues and seizures, impacting daily functioning.

Purpose of the Study:

  • To summarize the key features of Landau-Kleffner syndrome.
  • To highlight the diagnostic criteria and challenges in managing this condition.
  • To discuss the current understanding of its etiology and prognosis.

Main Methods:

  • Review of clinical case studies and relevant literature on Landau-Kleffner syndrome.

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  • Analysis of diagnostic markers including EEG findings.
  • Correlation of clinical presentation with neuroimaging results (CT, MRI).

    • Main Results:

    • Landau-Kleffner syndrome presents with severe impairment in auditory comprehension, often described as acquired verbal auditory agnosia.
    • Electroencephalogram (EEG) reveals characteristic epileptiform discharges, while neuroimaging (CT, MRI) is typically normal.
    • Seventy-five percent of patients experience seizures, and two-thirds have associated behavioral abnormalities.

    Conclusions:

    • Seizures in LKS are generally well-controlled with antiepileptic drugs.
    • Language recovery is often difficult and prognosis remains variable, suggesting a complex interplay of factors.
    • An autoimmune etiology is increasingly considered likely for Landau-Kleffner syndrome.