Abstracts

RATIONALE: Landau-Kleffner Syndrome is a condition in young children with epileptic activity causing verbal agnosia and receptive aphasia, regression in language skills, and occasionally autistic behaviour. We report a case of Landau-Kleffner Syndrome with language regression, receptive aphasia, left temporal spikes and electrical status epilepticus during slow wave sleep (ESES) on EEG, who was studied with MRI volumetric analysis and quantitative EEG. METHODS: MRI was acquired with 1.5 mm 3D-SPGR T1 coronal slices and analyzed with the CARDVIEWS program (Caviness et al., 1996) for various substructures of the brain and subdivisions of the neocortex. Source localization of the interictal spikes and frequency modulated auditory evoked response (FMAER) were analyzed. RESULTS: Interictal spikes during awake and drowsiness localized to the left temporal area on source localization analysis. FMAER was absent. Additional slowing suggested dysfunction in the region of Wernicke's area. MRI volume measurements revealed volume reduction greater in the left temporal lobe including Heschl's gyrus, planum temporale, and superior temporal gyrus. CONCLUSIONS: In Landau-Kleffner Syndrome, acquired epileptic activity is hypothesized to interfere with function, resulting in verbal agnosia. Absent FMAER has been reported in case series with Landau-Kleffner Syndrome. We detected subtle anatomical changes at the location of the interictal spikes in the region of Wernicke's area. These changes were very difficult to detect without volumetric analysis. The quantitative EEG and MRI volumetric analysis provide functional and anatomical information from the suspected area of pathology. Atrophy detected in the region of Wernicke's area would suggest neuronal loss, gliosis, and a guarded prognosis for language recovery. We postulate that MRI volumetric analysis in conjunction with source analysis and FMAER will be helpful in assessing prognoses for language recovery in Landau-Kleffner Syndrome.