Department of Neurology, Massachusetts General Hospital, Boston, MA 02114, USA.
Department of Neurology, Harvard Medical School, Boston, MA 02115, USA.
Brain. 2024 Sep 3;147(9):3216-3233. doi: 10.1093/brain/awae161.
Intraventricular haemorrhage is a common complication of premature birth. Survivors are often left with cerebral palsy, intellectual disability and/or hydrocephalus. Animal models suggest that brain tissue shrinkage, with subsequent vascular stretch and tear, is an important step in the pathophysiology, but the cause of this shrinkage is unknown. Clinical risk factors for intraventricular haemorrhage are biomarkers of hypoxic-ischaemic stress, which causes mature neurons to swell. However, immature neuronal volume might shift in the opposite direction in these conditions. This is because immature neurons express the chloride, salt and water transporter NKCC1, which subserves regulatory volume increases in non-neural cells, whereas mature neurons express KCC2, which subserves regulatory volume decreases. When hypoxic-ischaemic conditions reduce active ion transport and increase the cytoplasmic membrane permeability, the effects of these transporters are diminished. Consequentially, mature neurons swell (cytotoxic oedema), whereas immature neurons might shrink. After hypoxic-ischaemic stress, in vivo and in vitro multi-photon imaging of perinatal transgenic mice demonstrated shrinkage of viable immature neurons, bulk tissue shrinkage and blood vessel displacement. Neuronal shrinkage was correlated with age-dependent membrane salt and water transporter expression using immunohistochemistry. Shrinkage of immature neurons was prevented by prior genetic or pharmacological inhibition of NKCC1 transport. These findings open new avenues of investigation for the detection of acute brain injury by neuroimaging, in addition to prevention of neuronal shrinkage and the ensuing intraventricular haemorrhage, in premature infants.
脑室出血是早产儿的常见并发症。幸存者常伴有脑瘫、智力障碍和/或脑积水。动物模型表明,脑组织收缩,随后血管拉伸和撕裂,是病理生理学中的一个重要步骤,但这种收缩的原因尚不清楚。脑室出血的临床危险因素是缺氧缺血应激的生物标志物,这会导致成熟神经元肿胀。然而,在这些情况下,未成熟神经元的体积可能会向相反的方向移动。这是因为未成熟神经元表达氯离子、盐和水转运蛋白 NKCC1,它在非神经细胞中发挥调节体积增加的作用,而成熟神经元表达 KCC2,它在调节体积减少中发挥作用。当缺氧缺血条件下减少活性离子转运并增加细胞质膜通透性时,这些转运体的作用会减弱。结果,成熟神经元肿胀(细胞毒性水肿),而未成熟神经元可能会收缩。缺氧缺血应激后,对围产期转基因小鼠的体内和体外多光子成像显示,存活的未成熟神经元、组织整体收缩和血管移位。免疫组织化学显示,神经元收缩与年龄依赖性膜盐和水转运体表达相关。通过 NKCC1 转运的遗传或药物抑制可以预防未成熟神经元的收缩。这些发现为通过神经影像学检测急性脑损伤开辟了新的研究途径,除了预防神经元收缩和随之发生的脑室出血,这对早产儿有益。