Billiards Saraid S, Haynes Robin L, Folkerth Rebecca D, Borenstein Natalia S, Trachtenberg Felicia L, Rowitch David H, Ligon Keith L, Volpe Joseph J, Kinney Hannah C
Department of Pathology, Children's Hospital Boston, Harvard Medical School, Boston, MA 02115, USA.
Brain Pathol. 2008 Apr;18(2):153-63. doi: 10.1111/j.1750-3639.2007.00107.x. Epub 2008 Jan 3.
The cellular basis of myelin deficits detected by neuroimaging in long-term survivors of periventricular leukomalacia (PVL) is poorly understood. We tested the hypothesis that oligodendrocyte lineage (OL) cell density is reduced in PVL, thereby contributing to subsequent myelin deficits. Using computer-based methods, we determined OL cell density in sections from 18 PVL and 18 age-adjusted control cases, immunostained with the OL-lineage marker Olig2. Myelination was assessed with myelin basic protein (MBP) immunostaining. We found no significant difference between PVL and control cases in Olig2 cell density in the periventricular or intragyral white matter. We did find, however, a significant increase in Olig2 cell density at the necrotic foci, compared with distant areas. Although no significant difference was found in the degree of MBP immunostaining, we observed qualitative abnormalities of MBP immunostaining in both the diffuse and necrotic components of PVL. Abnormal MBP immunostaining in PVL despite preserved Olig2 cell density may be secondary to arrested OL maturation, damage to OL processes, and/or impaired axonal-OL signaling. OL migration toward the "core" of injury may occur to replenish OL cell number. This study provides new insight into the cellular basis of the myelin deficits observed in survivors of PVL.
脑室周围白质软化症(PVL)长期存活者经神经影像学检测到的髓鞘缺损的细胞基础尚不清楚。我们检验了这样一个假设,即PVL中少突胶质细胞谱系(OL)细胞密度降低,从而导致随后的髓鞘缺损。我们使用基于计算机的方法,测定了18例PVL病例和18例年龄匹配的对照病例的切片中的OL细胞密度,并用OL谱系标记物Olig2进行免疫染色。用髓鞘碱性蛋白(MBP)免疫染色评估髓鞘形成。我们发现PVL病例和对照病例在脑室周围或脑回内白质的Olig2细胞密度上没有显著差异。然而,我们确实发现,与远处区域相比,坏死灶处的Olig2细胞密度显著增加。尽管在MBP免疫染色程度上未发现显著差异,但我们在PVL的弥漫性和坏死性成分中均观察到MBP免疫染色的定性异常。尽管Olig2细胞密度保持不变,但PVL中MBP免疫染色异常可能继发于OL成熟停滞、OL突起损伤和/或轴突-OL信号受损。OL可能会向损伤“核心”迁移以补充OL细胞数量。这项研究为PVL存活者中观察到的髓鞘缺损的细胞基础提供了新的见解。