The Landau-Kleffner syndrome: increased understanding

The Landau-Kleffner and the continuous spike and wave discharges during slow sleep (CSWS) syndromes are described and possible links between the two are discussed. They certainly overlap, with clinical and electroencephalographic features in common. Potential causes are discussed. There is seldom a definite reason for the seizures but it way well be that the spike and wave discharges seen in the EEG, whatever their origin, may disrupt the development of language and cognitive function at a critical stage. If neurons and axons are involved in this disorganized activity they surely cannot perform normally. The evidence that these discharges in the Landau-Kleffner syndrome can have a focal origin in areas important for language supports this hypothesis. Certain variations among studies quoted may be due to factors such as age of onset, the duration of the paroxysmal activity, its intensity and especially its localization. Also, if development has been distorted subsequent progress is likely to be disturbed after the primary condition has ceased to exist. The diagnosis can sometimes present difficulties, for example from deafness, psychiatric condition, post-ical dysphasia and from progressive degenerative neurological disorders. If the possible role of the epileptic activity is accepted, there are strong reasons for advocating anti-epileptic treatment, even if overt seizures are rare. The response to drugs is varied. Fits will usually stop, but not so often the spike and wave discharges, and it is justifiable to try various regimes. Subpial resection has been reported to be successful, possibly by preventing the cortex generating seizures, and their spread.