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发育性协调障碍:SPECT 显示的小脑-大脑网络紊乱。

Developmental coordination disorder: disruption of the cerebello-cerebral network evidenced by SPECT.

机构信息

Department of Neurology, ZNA Middelheim General Hospital, Lindendreef 1, 2020 Antwerp, Belgium.

出版信息

Cerebellum. 2010 Sep;9(3):405-10. doi: 10.1007/s12311-010-0177-6.

Abstract

Little is known about the neurobiological substrate of developmental coordination disorder (DCD), a neuro-developmental syndrome with significant, negative impact on the motor, cognitive and affective level throughout lifespan. This paper reports the clinical, neurocognitive and neuroradiological findings of a 19-year-old patient with typical DCD. As demonstrated by mild ataxia and a close semiological correspondence with the recently acknowledged 'cerebellar cognitive affective syndrome', clinical and neurocognitive investigations unambiguously indicated functional disruption of the cerebellum. Structural MRI of the brain confirmed cerebellar involvement revealing a slight anterior/superior asymmetry of vermal fissures consistent with rostral vermisdysplasia. Although this abnormality of vermal fissuration is generally considered an incidental neuroradiological finding without any clinical relevance, a potentially subtle impact on the developmental level has never been formally excluded. In addition to a generally decreased perfusion of the cerebellum, a quantified Tc-99m-ECD SPECT disclosed functional suppression of the anatomoclinically suspected supratentorial regions involved in the execution of planned actions, visuo-spatial processing and affective regulation. Based on these findings, it is hypothesised that the cerebellum is crucially implicated in the pathophysiologcial mechanisms of DCD, reflecting disruption of the cerebello-cerebral network involved in the execution of planned actions, visuo-spatial cognition and affective regulation.

摘要

目前对于发育性协调障碍(DCD)的神经生物学基础知之甚少,这是一种神经发育综合征,对整个生命周期的运动、认知和情感水平都有重大的负面影响。本文报告了一名 19 岁典型 DCD 患者的临床、神经认知和神经影像学发现。由于存在轻度共济失调,并且与最近公认的“小脑认知情感综合征”具有密切的半综合征对应关系,临床和神经认知研究明确表明小脑功能障碍。大脑结构 MRI 证实了小脑的参与,显示出蚓部裂缝的轻微前/上不对称,与颅尾蚓部发育不良一致。尽管这种蚓部裂缝的异常通常被认为是没有任何临床相关性的偶然神经影像学发现,但它对发育水平的潜在细微影响从未被正式排除。除了小脑总体灌注减少外,定量 Tc-99m-ECD SPECT 还显示了解剖临床怀疑参与计划动作执行、视空间处理和情感调节的额顶叶区域的功能抑制。基于这些发现,假设小脑在 DCD 的病理生理机制中起着至关重要的作用,反映了参与计划动作执行、视空间认知和情感调节的小脑-大脑网络的破坏。

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