TY - JOUR
T1 - Polymicrogyria includes fusion of the molecular layer and decreased neuronal populations but normal cortical laminar organization
AU - Judkins, Alexander R.
AU - Martinez, Daniel
AU - Ferreira, Pamela
AU - Dobyns, William B.
AU - Golden, Jeffrey A.
PY - 2011/6
Y1 - 2011/6
N2 - Malformations of cortical development are frequently identified in surgical resections for intractable epilepsy. Among the more frequently identified are cortical dysplasia, pachygyria, and polymicrogyria. The pathogenesis of these common developmental anomalies remains uncertain. Polymicrogyria is particularly vexing because there are multiple described forms (2, 4, and 6 layers) that have been attributed to multiple etiologies (e.g. ischemic, genetic, infectious, and toxic). We reviewed thepathology in 19 cases and performed cortical laminar analysis in 10 ofthese cases. Our data indicate that a defining feature of polymicrogyriais fusion of the molecular layer and that most often there is a well-defined gray matter-white matter junction. Unexpectedly, the cortical laminae were normally positioned, but there were reduced neuronal populations within these laminae, particularly in the subgranular layers. On the basis of these data, we propose that the categorization of polymicrogyria according to the number of lamina is artificial and should beabandoned, and polymicrogyria should be defined according to the presence or absence of coexisting neuropathological features. Furthermore, our data indicate that polymicrogyria is not a cell migration disorder, rather it should be considered a postmigration malformation ofcortical development.
AB - Malformations of cortical development are frequently identified in surgical resections for intractable epilepsy. Among the more frequently identified are cortical dysplasia, pachygyria, and polymicrogyria. The pathogenesis of these common developmental anomalies remains uncertain. Polymicrogyria is particularly vexing because there are multiple described forms (2, 4, and 6 layers) that have been attributed to multiple etiologies (e.g. ischemic, genetic, infectious, and toxic). We reviewed thepathology in 19 cases and performed cortical laminar analysis in 10 ofthese cases. Our data indicate that a defining feature of polymicrogyriais fusion of the molecular layer and that most often there is a well-defined gray matter-white matter junction. Unexpectedly, the cortical laminae were normally positioned, but there were reduced neuronal populations within these laminae, particularly in the subgranular layers. On the basis of these data, we propose that the categorization of polymicrogyria according to the number of lamina is artificial and should beabandoned, and polymicrogyria should be defined according to the presence or absence of coexisting neuropathological features. Furthermore, our data indicate that polymicrogyria is not a cell migration disorder, rather it should be considered a postmigration malformation ofcortical development.
KW - Cell migration
KW - Cerebral cortex
KW - Cortical lamina
KW - Malformation of cortical development
KW - Polymicrogyria
KW - Seizures
UR - http://www.scopus.com/inward/record.url?scp=79957479149&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=79957479149&partnerID=8YFLogxK
U2 - 10.1097/NEN.0b013e31821ccf1c
DO - 10.1097/NEN.0b013e31821ccf1c
M3 - Article
C2 - 21572338
AN - SCOPUS:79957479149
SN - 0022-3069
VL - 70
SP - 438
EP - 443
JO - Journal of neuropathology and experimental neurology
JF - Journal of neuropathology and experimental neurology
IS - 6
ER -