Possible involvement of the tip of temporal lobe in Landau-Kleffner syndrome.
Landau-Kleffner syndrome (LKS) is a childhood disorder of unknown etiology characterized by an acquired aphasia and epilepsy. We have performed comprehensive neurofunctional studies on an 8-year-old girl with typical LKS, with the aim of identifying lesions that may be responsible for her condition. 18F-fluoro-D-glucose (FDG) positron emission computed tomography (PET), 11C-Flumazenil (FMZ) PET, 99mTc-hexamethylpropyleneamine oxime single photon emission computed tomography (SPECT) and magnetoencephalography were performed before and after changes to the patient's medication led to a clinical improvement. Interictal SPECT showed hypoperfusion in the left frontal, left temporal, and left occipital lobes. 18F-FDG PET demonstrated a decrease in glucose metabolism medially in both temporal lobes and superiorly in the left temporal lobe. 11C-FMZ PET revealed a deficit in benzodiazepine receptor binding at the tip of the left temporal lobe. Magnetoencephalography demonstrated equivalent current dipoles located superiorly in the left temporal lobe. Our results suggest that the tip of the left temporal lobe plays an important role in the pathogenesis of LKS in our patient.
Shiraishi
Hideaki
H
Department of Pediatrics, Hokkaido University, Graduate School of Medicine, North 15, West 7, Kita-ku, Sapporo, Hokkaido 060-8638, Japan. siraisi@med.hokudai.ac.jp
Takano
Kyoko
K
Shiga
Tohru
T
Okajima
Maki
M
Sudo
Akira
A
Asahina
Naoko
N
Kohsaka
Shinobu
S
Fukuhara
Masakazu
M
Saitoh
Shinji
S
D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D004569:Electroencephalography; D005260:Female; D006801:Humans; D018887:Landau-Kleffner Syndrome / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D015225:Magnetoencephalography; D049268:Positron-Emission Tomography; D012640:Seizures / Q000188:drug therapy / Q000209:etiology; D019690:Technetium Tc 99m Exametazime; D013702:Temporal Lobe / Q000378:metabolism* / Q000473:pathology / Q000503:physiopathology*; D015899:Tomography, Emission-Computed, Single-Photon
D000927:Anticonvulsants; D019690:Technetium Tc 99m Exametazime