Efficacy of very high dose steroid treatment in a case of Landau-Kleffner syndrome

Dev Med Child Neurol. 2006 Sep;48(9):766-9. doi: 10.1017/S0012162206001630.

Abstract

Landau-Kleffner syndrome (LKS) is an acquired childhood aphasia associated with paroxysmal bitemporal electroencephalogram (EEG) abnormalities and, sometimes, clinical seizures. We report the case of a female aged 5 years 6 months who presented clinically with apparent hearing loss, deterioration in speech, and seizure activity over 12 days. The female had previous detailed speech/language assessments at 3 to 4 years of age due to articulation delay. LKS was diagnosed on EEG with bitemporal spike and wave activity during sleep. The patient was treated with high dose prednisolone 3mg/kg/day, intensive speech/language therapy, and followed a modified educational program. We recorded a marked regression in receptive and expressive language skills, as well as her speech, language, and cognitive profiles before and during treatment with prednisolone, during an 18-month follow-up period. The patient demonstrated an excellent clinical response highlighting the importance of a multidisciplinary approach to management of LKS.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Child, Preschool
  • Cognition*
  • Dose-Response Relationship, Drug
  • Drug Administration Schedule
  • Electroencephalography / drug effects
  • Female
  • Glucocorticoids / administration & dosage*
  • Humans
  • Landau-Kleffner Syndrome / drug therapy*
  • Landau-Kleffner Syndrome / rehabilitation
  • Language Development
  • Prednisolone / administration & dosage*
  • Speech Therapy
  • Treatment Outcome

Substances

  • Glucocorticoids
  • Prednisolone