Congenital bilateral perisylvian syndrome associated with congenital constriction band syndrome.
We report a 7-year-old boy with congenital bilateral perisylvian syndrome and congenital constriction band syndrome. The former is a congenital neurologic syndrome characterized by pseudobulbar palsy, mental retardation, epilepsy, and bilateral perisylvian polymicrogyria The latter is a malformative disorder with digital ring constrictions and amputations, probably caused by early amnion rupture resulting in entanglement of fetal parts by amniotic strands. We believe that the combination of these two malfornative disorders was not coincidental; instead, fetal circulatory disturbance related to chronic abruptio placentae could account for this combination.
Yamanouchi
Hideo
H
Department of Pediatrics, Dokkyo University School of Medicine, Tochigi, Japan. yhideo@dokkyomed.ac.jp
Ota
Takako
T
Imataka
George
G
Hagiwara
Yuri
Y
Nakagawa
Eiji
E
Eguchi
Mitsuoki
M
D000652:Amniotic Band Syndrome / Q000150:complications* / Q000473:pathology; D001927:Brain Diseases / Q000150:complications / Q000151:congenital / Q000175:diagnosis; D002535:Cerebral Aqueduct / Q000473:pathology*; D002648:Child; D005532:Foot Deformities, Congenital / Q000209:etiology*; D006228:Hand Deformities, Congenital / Q000209:etiology*; D006801:Humans; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging; D008297:Male; D013577:Syndrome