Landau-Kleffner syndrome is not an eponymic badge of ignorance.
In a 1992 editorial article, Landau expressed the hope of collective agreement in the medical community about Landau-Kleffner syndrome (LKS) in terms of diagnosis criteria, etiology, pathophysiology and rational therapy. Since then, neurophysiological and neuroimaging studies have led to the view that LKS is an acquired aphasia, secondary to an epileptic disturbance affecting a cortical area involved in verbal processing. This fits with the hypothesis of a "functional ablation" caused by epileptic activity. Under these criteria, epileptic aphasia becomes a subgroup of the continuous spike-waves syndrome in which epileptic discharges originate from the temporal cortex. Genetic predisposition for KLS could be related to hyperexcitability and synchronization of interneurons within the perisylvian cortices, which generate the spike-waves. Activation of these waves during NREM sleep, following thalamo-cortical uncoupling, might then alter the blood brain barrier and provoke an autoimmune reaction. Interneuron hyperactivity might in turn have an antiepileptic protective effect, associated with the inhibition of a specific function, and spike-waves activity over the long term might eventuate in focal atrophy. This morphological defect might explain the poor verbal outcome in some cases of LKS. From this study we recommend a multicenter control study of good design and methodology be carried out to compare the efficacies of early versus delayed (3 months) corticosteroid treatment in patients with typical LKS that is being treated by clobazam (or diazepam) monotherapy.
Hirsch
Edouard
E
Department of Neurology HUS, Strasbourg, France. Edouard.Hirsch@chu-strasbourg.fr
Valenti
Maria Paola
MP
Rudolf
Gabrielle
G
Seegmuller
Caroline
C
de Saint Martin
Anne
A
Maquet
Pierre
P
Wioland
Norma
N
Metz-Lutz
Marie-Noëlle
MN
Marescaux
Christian
C
Arzimanoglou
Alexis
A
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D004569:Electroencephalography; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy; D008570:Memory, Short-Term / Q000502:physiology; D011379:Prognosis; D013702:Temporal Lobe / Q000473:pathology*
D000305:Adrenal Cortex Hormones