Abstracts

Landau-Kleffner syndrome (epileptic aphasia) is a rare disease and there are few cases with long follow-up. Most of these are the ones with worse evolution that are more linked to reference centers. Normal outcome is described in some reports but there is lack of quantitative data. We describe three children that had typical Landau-Kleffner syndrome and their speech evolution after several yrs. of treatment. All of them were submitted to Boston Diagnostic Aphasia Examination (Goodglass [italic]et al[/italic]., 2001) 7 to 13 yrs. after acute phase of the disease by a speech pathologist specialist. Three children, two girls and one boy, with normal IQ, presented acquired aphasia, with onset at 4 to 7yrs. of age and epileptic seizures. Investigation revealed normal brain MRI, focal epileptiform activity on perisylvian area, two of them showing bilateral and continuous discharges, and all interictal unilateral temporal hypoperfusion. All patients received AED and two, corticosteroids. During follow-up all three patients had normal EEG and adequate scholastic performance. Language evaluation (Boston Diagnostic Aphasia Examination) obtained 7 to 13 yrs. after the onset of aphasia showed normal performance regarding the following items: auditory comprehension (word discrimination, commands, complex ideational material), oral expression (repetition, responsive naming, visual confrontation naming), written comprehension and production. Although several overlap of presentation may be seen in childhood epileptic aphasia, there are some typical pure Landau-Kleffner syndrome cases that may constitute the end of a spectrum pointing to a benign entity with good outcome. The presence of continuous epileptiform activity and temporal hypoperfusion present in some patients may urge to prompt acute treatment, but even in those cases normal language outcome can be present.