Delayed maturation and differentiation of neurons in focal cortical dysplasia with the transmantle sign:  analysis of layer-specific marker expression.
Transmantle dysplasia is a rare type of focal cortical dysplasia (FCD) characterized by expansion of the cortex from the deep white matter to the surface and in which there is a FCD IIA or IIB pathologic pattern. To characterize possible mechanisms underlying this regional disorder of radial migrating cells, we studied the expression patterns of neocortical layer-specific markers using immunohistochemistry in surgical specimens from 5 FCD IIA and 4 FCD IIB cases in children. All neuronal cells expressed the mature neuron marker MAP2/2B but not the microglia markers Iba-1 and CD68. Some layer-specific markers showed distinct expression patterns. TBR1-positive, SATB2-positive, and FOXP1-positive cells were diffusely distributed in the cortex and/or the white matter. TBR1-positive and FOXP1-positive cells were generally more numerous in FCD IIB than in FCD IIA and were mostly in the cortical molecular and upper layers. FOXP1-, FOXP2-, and CUTL1-positive cells also expressed the immature neuron marker, Nestin/PROX1, whereas TBR1-, CTIP2-, and SATB2-positive cells only expressed MAP2/2B. These data highlight differences between FCD IIB and FCD IIA with more cells having the immature marker in upper layer markers in the former. By analyzing layer-specific marker expression patterns, we identified apparent neuronal maturation differences between FCD IIA and FCD IIB in cases of transmantle dysplasia.
Epilepsy Center, National Center of Neurology and Psychiatry, Tokyo, Japan.
Takafumi
T
Sakakibara
Sayuri
S
Sukigara
Takashi
T
Saito
Taisuke
T
Otsuki
Akio
A
Takahashi
Yuu
Y
Kaneko
Takanobu
T
Kaido
Yuko
Y
Saito
Noriko
N
Sato
Yukio
Y
Kimura
Eiji
E
Nakagawa
Kenji
K
Sugai
Masayuki
M
Sasaki
Yu-Ichi
Y
Goto
Masayuki
M
Itoh
D002452:Cell Count; D002454:Cell Differentiation / Q000502:physiology*; D002675:Child, Preschool; D005260:Female; D005819:Genetic Markers / Q000235:genetics*; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D007231:Infant, Newborn; D007361:Intelligence Tests; D008297:Male; D054220:Malformations of Cortical Development / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D009474:Neurons / Q000502:physiology*; D019635:Neurosurgical Procedures; D012640:Seizures / Q000145:classification / Q000209:etiology
D005819:Genetic Markers