A 7-year-old boy with a left temporal lobe tumor and Landau-Kleffner syndrome (acquired aphasia and epilepsy) who improved dramatically after surgery is reported from the New York Hospital-Cornell University Medical Center. A verbal auditory agnosia with mutism had evolved over a 3 month period after onset of refractory partial complex seizures at 5 years. EEG showed left temporal and right occipital spike and S/W discharges. Language comprehension was absent on the Peabody Picture Vocabulary test and Token test. Following removal of a grade 1 astocytoma demonstrated on MRI, seizures stopped, language returned, and after 1 year speech and comprehension were almost normal for age. [1]
COMMENT. The favorable result of surgery in this patient contrasts with the poor outcome of most patients with Landau-Kleffner syndrome treated medically. MRI and ictal SPECT should be considered in all patients with this syndrome to rule out localized structural pathology amenable to surgery.