BRAIN SINGLE-PHOTON EMISSION COMPUTED-TOMOGRAPHY IMAGING IN LANDAU-KLEFFNER SYNDROME

Five right-handed children with Landau-Kleffner syndrome (LKS) who had disease onset between the ages of 3 and 9 years were studied with EEG and single-photon emission computed tomography (SPECT) before and, in four cases, after 6 months of corticosteroid treatment. EEG findings included both focal and generalized spikes as well as spike-wave disch arges with bilateral temporal predominance. These increased markedly d uring sleep in 1 child, and continuous spike-and-wave complexes appear ed during slow-wave sleep in another patient. Neuropsychological testi ng demonstrated verbal auditory agnosia. Magnetic resonance imaging (M RI) was performed in 4 children and was normal. Brain SPECT imaging de monstrated abnormal perfusion in the left temporal lobe in all patient s. The response to corticosteroid therapy was mixed. Our findings rein force the concept that LKS is a functional disease affecting the langu age-dominant brain areas. We conclude that SPECT imaging may be of dia gnostic assistance in the evaluation of this syndrome of unknown etiol ogy.