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

Published online by Cambridge University Press:  14 August 2006

Siobhan Gallagher
Affiliation:
Division of Neurology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Canada.
Shelley Weiss
Affiliation:
Division of Neurology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Canada.
Janis Oram Cardy
Affiliation:
School of Communication Sciences and Disorders, University of Western Ontario, London, Ontario, Canada.
Tom Humphries
Affiliation:
Division of Human Development and Applied Psychology, Ontario Institute for Studies in Education, University of Toronto, Canada.
Karen E Harman
Affiliation:
Division of Child Development, Department of Paediatrics, McMaster Children's Hospital, McMaster University, Hamilton, Ontario, Canada.
Shay Menascu
Affiliation:
Division of Neurology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Canada.
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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.

Type
Case Report
Copyright
2006 Mac Keith Press

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