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 discharges with bilateral temporal predominance. These increased markedly during sleep in 1 child, and continuous spike-and-wave complexes appeared during slow-wave sleep in another patient. Neuropsychological testing demonstrated verbal auditory agnosia. Magnetic resonance imaging (MRI) was performed in 4 children and was normal. Brain SPECT imaging demonstrated abnormal perfusion in the left temporal lobe in all patients. The response to corticosteroid therapy was mixed. Our findings reinforce the concept that LKS is a functional disease affecting the language-dominant brain areas. We conclude that SPECT imaging may be of diagnostic assistance in the evaluation of this syndrome of unknown etiology.
Guerreiro
M M
MM
Departamento de Neurologia, Universidade Estadual de Campinas, Campinas, Brasil.
Camargo
E E
EE
Kato
M
M
Menezes Netto
J R
JR
Silva
E A
EA
Scotoni
A E
AE
Silveira
D C
DC
Guerreiro
C A
CA
D017668:Age of Onset; D001921:Brain / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000175:diagnosis / Q000000981:diagnostic imaging* / Q000188:drug therapy; D008297:Male; D009483:Neuropsychological Tests; D015609:Organotechnetium Compounds; D010091:Oximes; D011241:Prednisone / Q000627:therapeutic use; D012890:Sleep / Q000502:physiology; D019690:Technetium Tc 99m Exametazime; D013702:Temporal Lobe / Q000502:physiology; D015899:Tomography, Emission-Computed, Single-Photon
D015609:Organotechnetium Compounds; D010091:Oximes; D019690:Technetium Tc 99m Exametazime; D011241:Prednisone