Efficacy of very high dose steroid treatment in a case of Landau-Kleffner syndrome.
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.
Gallagher
Siobhan
S
Division of Neurology, Department of Paediatrics, The Hospital for Sick Children, University of Toronto, Canada. sgallagher@ireland.com
Weiss
Shelley
S
Oram Cardy
Janis
J
Humphries
Tom
T
Harman
Karen E
KE
Menascu
Shay
S
D002648:Child; D002675:Child, Preschool; D003071:Cognition; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D004569:Electroencephalography / Q000187:drug effects; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000188:drug therapy* / Q000534:rehabilitation; D007804:Language Development; D011239:Prednisolone / Q000008:administration & dosage*; D013070:Speech Therapy; D016896:Treatment Outcome
D005938:Glucocorticoids; D011239:Prednisolone