Landau-Kleffner syndrome: relation of clinical, EEG and Tc-99m-HMPAO brain SPECT findings and improvement in EEG after treatment

Abstract

Landau-Kleffner syndrome (LKS) is a rare childhood disorder characterized by acquired aphasia with seizures and electroencephalogram (EEG) abnormalities. Tc-99m-HMPAO SPECT was performed in three right handed children with LKS. A relative decrease in perfusion was found in the left temporal cortex of all three patients and also in the left frontoparietal cortex of one patient with hyperkinetic behavior. Degree of regional cerebral perfusion impairment did not correlate with the severity of clinical and EEG abnormalities. Asymmetrical temporoparietal perfusion appears characteristic of LKS. SPECT findings in LKS were evaluated as useful in elucidating the pathogenic features of the disorder in the brain.