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, Philippeet al.
2013 • In Developmental Medicine and Child Neurology, 55 (6), p. 575-9
[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
Commission on Classification Terminology of the International League Against Epilepsy. Proposal for revised classification of epilepsies and epileptic syndromes. Epilepsia 1989; 30: 389-99.
Landau WM, Kleffner FR. Syndrome of acquired aphasia with convulsive disorder in children. Neurology 1957; 7: 523-30.
Rapin I, Mattis S, Rowan AJ, Golden GG. Verbal auditory agnosia in children. Dev Med Child Neurol 1977; 19: 197-207.
Paquier PF, Van Dongen HR, Loonen CB. The Landau-Kleffner syndrome or 'acquired aphasia with convulsive disorder'. Long-term follow-up of six children and a review of the recent literature. Arch Neurol 1992; 49: 354-9.
Tassinari CA, Rubboli G, Volpi L, Billard C, Bureau M. [Status epilepticus during REM sleep (ESES or POCS) including acquired epileptic aphasia (Landau-Kleffner syndrome). In: Roger J, Bureau M, Dravet C, Genton P, Tassinari CA, Wolf P, editors. [Epileptic syndromes in children and adolescents], 3rd edn. Eastleigh: John Libbey, 2002: 265-83. (In French).
Deonna TW. Acquired epileptiform aphasia in children (Landau-Kleffner syndrome). J Clin Neurophysiol 1991; 8: 288-98.
Aicardi J. Epilepsy in Children. New York: Raven Press, 1986.
Galanopoulou AS, Bojko A, Lado F, Moshe SL. The spectrum of neuropsychiatric abnormalities associated with electrical status epilepticus in sleep. Brain Dev 2000; 22: 279-95.
Deonna T, Roulet-Perez E. Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and regressive autistic disorders with epileptic EEG abnormalities: the continuing debate. Brain Dev 2010; 32: 746-52.
Dulac O, Billard C, Arthuis M. [Electroclinical and developmental aspects of epilepsy in the aphasia-epilepsy syndrome]. Arch Fr Pediatr 1983; 40: 299-308. (In French).
Robinson RO, Baird G, Robinson G, Simonoff E. Landau-Kleffner syndrome: course and correlates with outcome. Dev Med Child Neurol 2001; 43: 243-7.
Hirsch E, Marescaux C, Maquet P, Metz-Lutz MN, et al.Landau-Kleffner syndrome: a clinical and EEG study of five cases. Epilepsia 1990; 31: 756-67.
Massa R, De Saint-Martin A, Hirsch E, et al.Landau-Kleffner syndrome: sleep EEG characteristics at onset. Neurophysiol Clin 2000; 111(Suppl 2): S87-93.
Engel J Jr. Report of the ILAE classification core group. Epilepsia 2006; 47: 1558-68.
McVicar KA, Ballaban-Gil K, Rapin I, Moshe SL, Shinnar S. Epileptiform EEG abnormalities in children with language regression. Neurology 2005; 65: 129-31.
Buzatu M, Bulteau C, Altuzarra C, Dulac O, Van Bogaert P. Corticosteroids as treatment of epileptic syndromes with continuous spike-waves during slow-wave sleep. Epilepsia 2009; 50(Suppl. 7): 68-72.
Holmes GL, McKeever M, Saunders Z. Epileptiform activity in aphasia of childhood: an epiphenomenon?Epilepsia 1981; 22: 631-9.
Da Silva EA, Chugani DC, Muzik O, Chugani HT. Landau-Kleffner syndrome: metabolic abnormalities in temporal lobe are a common feature. J Child Neurol 1997; 12: 489-95.
De Tiege X, Goldman S, Laureys S, et al. Regional cerebral glucose metabolism in epilepsies with continuous spikes and waves during sleep. Neurology 2004; 63: 853-7.
Bruehl C, Witte OW. Cellular activity underlying altered brain metabolism during focal epileptic activity. Ann Neurol 1995; 38: 414-20.
De Tiege X, Ligot N, Goldman S, Poznanski N, De Saint Martin A, Van Bogaert P. Metabolic evidence for remote inhibition in epilepsies with continuous spike-waves during sleep. Neuroimage 2008; 40: 802-10.
Christen HJ, Hanefeld F, Kruse E, Imhauser S, Ernst JP, Finkenstaedt M. Foix-Chavany-Marie (anterior operculum) syndrome in childhood: a reappraisal of Worster-Drought syndrome. Dev Med Child Neurol 2000; 42: 122-32.
Lowenthal F, Saerens J. [Utilization of logical formalisms in the examination of aphasic children]. Acta Neurol Belg 1982; 82: 215-23. (In French).
Stefanatos GA, Grover W, Geller E. Case study: corticosteroid treatment of language regression in pervasive developmental disorder. J Am Acad Child Adolesc Psychiatry 1995; 34: 1107-11.