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新生儿期起病的癫痫和偏侧巨脑畸形伴运动系统受累的髓鞘加速形成。

Accelerated myelination with motor system involvement in a neonate with immediate postnatal onset of seizures and hemimegalencephaly.

机构信息

Department of Radiology, Medical University of South Carolina, Charleston, SC, USA.

出版信息

Epilepsy Behav. 2011 Oct;22(2):391-4. doi: 10.1016/j.yebeh.2011.06.025. Epub 2011 Jul 29.

DOI:10.1016/j.yebeh.2011.06.025
PMID:21802995
Abstract

The timetable for normal myelination in the developing brain on MRI is well described. Beyond the genetically determined myelination process, environmental or other incidental factors may modulate the actual myelination, globally or regionally. Delayed myelination is well known in many pathological conditions interfering with adequate energy or myelin precursor supply. Rare but specific conditions where the normal process of myelination is accelerated are also known. We describe a case of early postnatal epilepsy demonstrating a striking degree of accelerated myelination both in the cerebral tissue adjacent to the presumed epileptic focus and along the ipsilateral pyramidal tract through the brainstem, a feature not previously described in the literature. This distal myelination highlights the peculiar system involvement pattern of function-induced myelination rather than simply regional myelination in response to an epileptic or other stimulus. This may have implications on our understanding of the development of function systems (visual, auditory, motor, sensory, etc.) or even the more complex interactive process of learning and may open new therapeutic opportunities in pathological functional conditions previously believed to be incurable.

摘要

正常脑发育过程中 MRI 上髓鞘形成的时间进程已有详细描述。除了遗传决定的髓鞘形成过程外,环境或其他偶然因素也可能调节整体或局部的实际髓鞘形成。在许多干扰足够能量或髓鞘前体细胞供应的病理情况下,髓鞘形成延迟是众所周知的。已知也有一些罕见但特定的情况下,正常的髓鞘形成过程会加速。我们描述了一例早发性癫痫病例,在假定的癫痫灶附近的脑组织以及沿着同侧锥体束穿过脑干,都表现出明显程度的加速髓鞘形成,这一特征在文献中尚未有描述。这种远端髓鞘形成突出了功能诱导髓鞘形成的特殊系统受累模式,而不仅仅是对癫痫或其他刺激的区域髓鞘形成。这可能对我们理解功能系统(视觉、听觉、运动、感觉等)的发育甚至更复杂的学习交互过程有影响,并为以前认为无法治愈的病理性功能障碍提供新的治疗机会。

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