Regional cerebral perfusion in Landau-Kleffner syndrome and related childhood aphasias.
Assessment of cerebral perfusion may elucidate pathogenesis of Landau-Kleffner syndrome (LKS). We obtained 99mTc-HMPAO SPECT studies in five children with LKS and in three children with syndromes of verbal-auditory agnosia. In LKS, perfusion showed temporoparietal asymmetry (9.56% +/- 3.44%) (n = 4) or bilateral parietal abnormality (n = 1). SPECT in non-LKS patients was normal (n = 1), showed (n = 1) totihemispheral hypoperfusion accompanying structural abnormality or (n = 1) a pattern resembling but distinct from LKS. Seizures in LKS patients had never occurred (n = 1), were controlled satisfactorily (n = 2), or poorly (n = 2). Maximum EEG abnormality was left centrotemoral-occipital (n = 1), left frontocentral (n = 1), bitemporal/left central (n = 1), and left central/parasagittal (n = 1). Asymmetric temporoparietal perfusion appears characteristic of LKS, differing from findings in other childhood linguistic disturbances. This abnormality occurs across a spectrum of seizure expression, diverging from the distribution of EEG abnormalities. The SPECT abnormalities parallel PET-defined LKS metabolic abnormalities, and may indicate central pathogenetic features of the disorder.
O'Tuama
L A
LA
Department of Radiology, Children's Hospital, Boston, MA 02115.
Urion
D K
DK
Janicek
M J
MJ
Treves
S T
ST
Bjornson
B
B
Moriarty
J M
JM
D001037:Aphasia / Q000000981:diagnostic imaging*; D001072:Apraxias / Q000000981:diagnostic imaging; D001921:Brain / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004827:Epilepsy / Q000000981:diagnostic imaging*; D005260:Female; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008297:Male; D015609:Organotechnetium Compounds; D010091:Oximes; D013577:Syndrome; D019690:Technetium Tc 99m Exametazime; D013702:Temporal Lobe / Q000000981:diagnostic imaging; D015899:Tomography, Emission-Computed, Single-Photon
D015609:Organotechnetium Compounds; D010091:Oximes; D019690:Technetium Tc 99m Exametazime