Acquired Epileptiform Aphasia in Children (Landau-Kleffner Syndrome)

Clinical characteristics of language regression in children

“…This contrasts with the examiner's practice, which is heavily skewed toward young children with language disorders and autism. Nonetheless, the findings in the two studies suggest that there is a real difference between language regression in younger children, in whom regression tends to be more global and associated with the emergence of autism, and that in older children, in whom it is somewhat more likely to be restricted to language and associated with epilepsy in the form of classic Landau-Kleffner syndrome (Kurita 1985, Deonna 1991, Kurita et al 1992, Mantovani 2000.…”

“…The third of parents of children with autism report language regression between 18 and 24 months of age, and 95% before age 3 years, whereas only 13% of children with Landau-Kleffner syndrome regress before that age (Deonna 1991, Kurita et al 1992, Rapin 1997, Tuchman and Rapin 1997, Mantovani 2000. Autistic regression is global by definition, whereas in Landau-Kleffner syndrome behavioral problems develop as a reaction to the child's emotional frustration at being unable to communicate adequately, and to the experience of social rejection, isolation, and low selfesteem.…”

“…Language regression is called disintegrative disorder when loss of language is in association with the appearance of autistic behaviors and severe cognitive deficit in a somewhat older child who is speaking in sentences and whose development has given no cause for concern, and who generally remains permanently severely impaired (American Psychiatric Association 1994). When the same scenario occurs in association with epileptic seizures or an epileptiform EEG and is not associated with severe behavioral deterioration or autism, the condition is currently referred to as acquired epileptic aphasia or Landau-Kleffner syndrome (Landau and Kleffner 1957, Rapin et al 1977, Deonna 1991, Tassinari et al 2000.…”

Landau kleffner syndrome, electrical status epilepticus in sleep and autistic regression: an overview of literature / Landau kleffner sendromu, uykuda elektriksel status epileptikus ve otistik regresyon: Literatüre bir bakış

“…The EEG of the children with LKS shows frequent sleep activated epileptiform activity maximally over the posterior temporal areas, unilaterally or bilaterally, with a dominance in the perisylvian or intrasylvian cortex (6,(12)(13)(14). Centro-temporal spikes in the intraperisylvian region which have a characteristic vertical dipole are also reported in these patients (12,15).…”

“…Although the presence of seizures is not needed for the diagnosis, 70-80% of LKS patients have clinical seizures (10,13,17). Patients tend to have partial onset seizures with eye deviation, eye blinking and head drops or complex partial seizures with secondarily generalization may be seen (7).…”