UCSF Weill Institute for Neurosciences, Department of Neurology, University of California at San Francisco, San Francisco, CA 94158, USA.
Department of Pediatrics, University of California at San Francisco, San Francisco, CA 94158, USA.
Brain. 2018 Jan 1;141(1):85-98. doi: 10.1093/brain/awx312.
Hypoxia can injure brain white matter tracts, comprised of axons and myelinating oligodendrocytes, leading to cerebral palsy in neonates and delayed post-hypoxic leukoencephalopathy (DPHL) in adults. In these conditions, white matter injury can be followed by myelin regeneration, but myelination often fails and is a significant contributor to fixed demyelinated lesions, with ensuing permanent neurological injury. Non-myelinating oligodendrocyte precursor cells are often found in lesions in plentiful numbers, but fail to mature, suggesting oligodendrocyte precursor cell differentiation arrest as a critical contributor to failed myelination in hypoxia. We report a case of an adult patient who developed the rare condition DPHL and made a nearly complete recovery in the setting of treatment with clemastine, a widely available antihistamine that in preclinical models promotes oligodendrocyte precursor cell differentiation. This suggested possible therapeutic benefit in the more clinically prevalent hypoxic injury of newborns, and we demonstrate in murine neonatal hypoxic injury that clemastine dramatically promotes oligodendrocyte precursor cell differentiation, myelination, and improves functional recovery. We show that its effect in hypoxia is oligodendroglial specific via an effect on the M1 muscarinic receptor on oligodendrocyte precursor cells. We propose clemastine as a potential therapy for hypoxic brain injuries associated with white matter injury and oligodendrocyte precursor cell maturation arrest.
缺氧可损伤由轴突和髓鞘形成的少突胶质细胞组成的脑白质束,导致新生儿脑性瘫痪和成人迟发性缺氧性白质脑病(DPHL)。在这些情况下,白质损伤后可发生髓鞘再生,但髓鞘形成通常失败,是固定脱髓鞘病变的重要原因,随之发生永久性神经损伤。在大量病变中经常可以发现未成熟的少突胶质前体细胞,但不能成熟,这表明少突胶质前体细胞分化阻滞是缺氧性髓鞘形成失败的关键因素。我们报告了一例成人 DPHL 病例,在使用广泛可用的抗组胺药氯苯那敏(clemastine)治疗后几乎完全康复,该药物在临床前模型中可促进少突胶质前体细胞分化。这提示在更常见的新生儿缺氧性损伤中可能具有治疗益处,我们在新生鼠缺氧性损伤中证明氯苯那敏可显著促进少突胶质前体细胞分化、髓鞘形成,并改善功能恢复。我们表明,其在缺氧中的作用是通过对少突胶质前体细胞上的 M1 毒蕈碱受体的作用而具有少突胶质细胞特异性。我们提出氯苯那敏可作为与白质损伤和少突胶质前体细胞成熟阻滞相关的缺氧性脑损伤的潜在治疗方法。