Developmental Imaging and Biophysics Section, UCL GOS Institute of Child Health, 30 Guilford Street, London WC1N 1EH, United Kingdom; Department of Neurosurgery, Great Ormond Street Hospital for Children, Great Ormond Street, London WC1N 3JH, United Kingdom.
Developmental Imaging and Biophysics Section, UCL GOS Institute of Child Health, 30 Guilford Street, London WC1N 1EH, United Kingdom.
Eur J Radiol. 2018 Nov;108:43-51. doi: 10.1016/j.ejrad.2018.09.007. Epub 2018 Sep 10.
Post-operative paediatric cerebellar mutism syndrome (pCMS) occurs in around 25% of children undergoing surgery for cerebellar and fourth ventricular tumours. Reversible mutism is the hallmark of a syndrome which comprises severe motor, cognitive and linguistic deficits. Recent evidence from advanced neuroimaging studies has led to the current theoretical understanding of the condition as a form of diaschisis contingent on damage to efferent cerebellar circuitry. Tractography data derived from diffusion MRI studies have shown disruption of the dentato-rubro-thalamo-cortical tract in patients with pCMS, and perfusion studies have indicated widespread supratentorial regions which may give rise to the florid signs and symptoms of pCMS. Given the difficulties in predicting pCMS from standard structural MRI, this review discusses findings from quantitative MRI modalities which have contributed to our understanding of this debilitating syndrome, and considers the goals and challenges which lie ahead in the field.
术后小儿小脑缄默症(pCMS)发生于约 25%接受小脑和第四脑室肿瘤手术的儿童。可逆性缄默是一种综合征的标志,包括严重的运动、认知和语言缺陷。来自先进神经影像学研究的最新证据使人们目前对这种疾病的理论认识为一种牵涉到传出小脑回路损伤的去抑制现象。来自弥散 MRI 研究的轨迹数据显示,pCMS 患者的齿状核-红核-丘脑-皮质束中断,灌注研究表明广泛的幕上区域可能导致 pCMS 的明显体征和症状。鉴于从标准结构 MRI 预测 pCMS 存在困难,本综述讨论了定量 MRI 方式的研究结果,这些结果有助于我们理解这种使人衰弱的综合征,并考虑了该领域的目标和挑战。