Syntelencephaly associated with connected transhemispheric cleft of focal cortical dysplasia.
The authors report a female with syntelencephaly associated with a connected transhemispheric cleft of focal cortical dysplasia. Syntelencephaly is a rare anomaly characterized by fusion of the hemispheres in the posterior frontal and parietal regions and is considered a new variant of holoprosencephaly. Cranial magnetic resonance imaging of the patient revealed syntelencephaly associated with bilateral fused clefts of focal cortical dysplasia without the pial-ependymal seam, which was regarded as an incomplete type of schizencephaly. The underlying mechanism is discussed.
Fujimoto
S
S
Department of Pediatrics, Nagoya City University Medical School, Nagoya, Japan.
Togari
H
H
Banno
T
T
Wada
Y
Y
D002658:Developmental Disabilities / Q000209:etiology / Q000473:pathology; D005260:Female; D016142:Holoprosencephaly / Q000209:etiology / Q000473:pathology*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D016548:Prosencephalon / Q000002:abnormalities* / Q000473:pathology