A Case of Open - lip Schizencephaly.
- Author:
Woo Jin KWON
;
Sun Kim LEE
;
Seung Keun LIM
;
Jae Ho HYUN
;
Mun Young LEE
;
Jin PARK
;
Man Kyu YANG
- Publication Type:Case Report
- Keywords:
Schizencephaly;
Gray matter-lined cleft
- MeSH:
Cerebrum;
Humans;
Infant;
Lateral Ventricles;
Lip*;
Magnetic Resonance Imaging;
Male;
Malformations of Cortical Development*;
Optic Nerve;
Paresis;
Seizures;
Septum Pellucidum;
Subarachnoid Space;
Transcutaneous Electric Nerve Stimulation
- From:Journal of the Korean Society of Neonatology
1998;5(1):86-89
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
Schizencephaly, first described by Yakovlev and Wadsworth in 1946, is a rare congenital anomaly characterized by gray matter-lined clefts that extend through the entire cerebral hemisphere, from the ependymal lining of the lateral ventricle to the subarachnoid space. Clinically, patients with schizencephaly present motor dysfunction such as hemiparesis, seizures, and variable developmental delay. Absence of septum pellucidum, gray matter heterotopia, polymicrogyria, and hypoplasia of optic nerves were also frequently found to be associated with schizencephaly. We experienced a case of open-lip schizencephaly in a one-day-old male infant with wide, tense fontanel, which was confirmed by MRI.
