Clinical and neuroimaging findings in children with gray matter heterotopias: A single institution experience of 36 patients.
OBJECTIVE
To describe the clinical spectrum and neuroimaging features of childhood gray matter heterotopias in a single tertiary hospital in Taiwan.


METHODS
We retrospectively reviewed the medical records and magnetic resonance images (MRI) of 36 patients with gray matter heterotopias, 19 females and 17 males, between July 1999 and June 2014. The MRI morphologic findings of gray matter heterotopias were recorded along with the presence of associated cerebral malformations. The clinical, electrophysiological and associated systemic malformation data were also recorded.


RESULTS
A total of 36 patients were included in the study. Their ages ranged from 1 month to 18 years with a mean age of 3 years 6 months. According to the location of gray matter heterotopias, patients were classified into two groups: periventricular (26) and band (10). The phenotypic spectrum in our population differed from that described previously. In the periventricular group, additional cerebral malformations were found in 18/26 (69%) and systemic malformations in 14/26 (54%). In the band group, additional cerebral malformations were found in 5/10 (50%) and systemic malformations in 2/10 (20%). The majority of patients had developmental delay and intellectual deficit. Twenty-two patients suffered from epileptic seizures with 12 developing refractory epilepsy.


CONCLUSIONS
In periventricular heterotopias, the most common associated cerebral malformation was ventriculomegaly, followed by agenesis of corpus callosum. Congenital heart disease was the most common additional systemic malformation. However, the most common associated cerebral malformation was pachygyria in band form. The majority of patients had developmental delay, intellectual deficit, especially in band heterotopias.
Division of Pediatric Neurology, Chang Gung Children Hospital, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, 5, Fu-Hsing St., Kwei-Shan, Taoyuan 333, Taiwan, ROC. Electronic address: h2918@cgmh.org.tw.
Po-Cheng
PC
Hung
Division of Pediatric Neurology, Chang Gung Children Hospital, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, 5, Fu-Hsing St., Kwei-Shan, Taoyuan 333, Taiwan, ROC.
Huei-Shyong
HS
Wang
Division of Pediatric Neurology, Chang Gung Children Hospital, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, 5, Fu-Hsing St., Kwei-Shan, Taoyuan 333, Taiwan, ROC.
Ming-Liang
ML
Chou
Division of Pediatric Neurology, Chang Gung Children Hospital, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, 5, Fu-Hsing St., Kwei-Shan, Taoyuan 333, Taiwan, ROC.
Kuang-Lin
KL
Lin
Division of Pediatric Neurology, Chang Gung Children Hospital, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, 5, Fu-Hsing St., Kwei-Shan, Taoyuan 333, Taiwan, ROC.
Meng-Ying
MY
Hsieh
Division of Pediatric Neuroradiology, Chang Gung Children Hospital, Chang Gung Memorial Hospital, College of Medicine, Chang Gung University, 5, Fu-Hsing St., Kwei-Shan, Taoyuan 333, Taiwan, ROC.
Alex M-C
AM
Wong
D000015:Abnormalities, Multiple / Q000473:pathology; D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002828:Choristoma; D002658:Developmental Disabilities / Q000209:etiology; D005260:Female; D005315:Fetal Diseases; D066128:Gray Matter; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D059906:Neuroimaging; D054091:Periventricular Nodular Heterotopia / Q000150:complications / Q000473:pathology*; D012189:Retrospective Studies; D013624:Taiwan
Band; Cerebral; Childhood; Heterotopias; Periventricular