A variant case of congenital bilateral perisylvian syndrome with asymmetric findings on neuroimaging and septum pellucidum defect.
We report a 17-year-old female patient with a variant form of congenital bilateral perisylvian syndrome (CBPS). She had pseudobulbar palsy, partial epilepsy and mild pyramidal symptoms predominantly in the left hand. Magnetic resonance imaging revealed asymmetric perisylvian and perirolandic polymicrogyric cortical dysplasia and septum pellucidum defect. The clinicoradiological findings for this patient met the criteria for CBPS. Moreover, they appeared to overlap those of congenital unilateral perisylvian syndrome. The findings in this case support the hypothesis that these two syndromes are parts of a continuous spectrum of one clinico-radiological syndrome.
Department of Pediatrics, Shimane Medical University, 89-1 Enya-cho, Izumo 693-8501, Japan. h-sejima@shimane-med.ac.jp
H
H
Sejima
Y
Y
Takusa
M
M
Kimura
Y
Y
Tamaoki
K
K
Kishi
S
S
Yamaguchi
D000293:Adolescent; D001921:Brain / Q000002:abnormalities* / Q000473:pathology* / Q000503:physiopathology; D002540:Cerebral Cortex / Q000002:abnormalities / Q000473:pathology / Q000503:physiopathology; D004827:Epilepsy / Q000151:congenital / Q000473:pathology / Q000503:physiopathology; D005260:Female; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D020547:Lateral Ventricles / Q000002:abnormalities / Q000473:pathology / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D009421:Nervous System Malformations / Q000473:pathology* / Q000503:physiopathology*; D010291:Paresis / Q000151:congenital / Q000473:pathology / Q000503:physiopathology; D020828:Pseudobulbar Palsy / Q000151:congenital / Q000473:pathology / Q000503:physiopathology; D012688:Septum Pellucidum / Q000002:abnormalities / Q000473:pathology / Q000503:physiopathology