Article (Scientific journals)
Acquired auditory agnosia in childhood and normal sleep electroencephalography subsequently diagnosed as Landau-Kleffner syndrome: report of three cases.
VAN Bogaert, Patrick; King, Mary D.; Paquier, Philippe et al.
2013In Developmental Medicine and Child Neurology, 55 (6), p. 575-9
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Abstract :
[en] Aim We report three cases of Landau-Kleffner syndrome (LKS) in children (two females, one male) in whom diagnosis was delayed because the sleep electroencephalography (EEG) was initially normal. Method Case histories including EEG, positron emission tomography findings, and long-term outcome were reviewed. Results Auditory agnosia occurred between the age of 2 years and 3 years 6 months, after a period of normal language development. Initial awake and sleep EEG, recorded weeks to months after the onset of language regression, during a nap period in two cases and during a full night of sleep in the third case, was normal. Repeat EEG between 2 months and 2 years later showed epileptiform discharges during wakefulness and strongly activated by sleep, with a pattern of continuous spike-waves during slow-wave sleep in two patients. Patients were diagnosed with LKS and treated with various antiepileptic regimens, including corticosteroids. One patient in whom EEG became normal on hydrocortisone is making significant recovery. The other two patients did not exhibit a sustained response to treatment and remained severely impaired. Interpretation Sleep EEG may be normal in the early phase of acquired auditory agnosia. EEG should be repeated frequently in individuals in whom a firm clinical diagnosis is made to facilitate early treatment.
Disciplines :
Neurology
Pediatrics
Author, co-author :
VAN Bogaert, Patrick
King, Mary D.
Paquier, Philippe
Wetzburger, Catherine
Labasse, Catherine
DUBRU, Jean-Marie ;  Centre Hospitalier Universitaire de Liège - CHU > Pédiatrie
Deonna, Thierry
Language :
English
Title :
Acquired auditory agnosia in childhood and normal sleep electroencephalography subsequently diagnosed as Landau-Kleffner syndrome: report of three cases.
Publication date :
June 2013
Journal title :
Developmental Medicine and Child Neurology
ISSN :
0012-1622
eISSN :
1469-8749
Publisher :
Mac Keith Press, London, United Kingdom
Volume :
55
Issue :
6
Pages :
575-9
Peer reviewed :
Peer Reviewed verified by ORBi
Commentary :
(c) The Authors. Developmental Medicine & Child Neurology (c) 2012 Mac Keith Press. Epub 2012 Nov 4
Available on ORBi :
since 22 January 2013

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