Program in Neurosciences and Mental Health, Department of Psychology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
Cerebellum. 2010 Dec;9(4):484-98. doi: 10.1007/s12311-010-0191-8.
Spina bifida meningomyelocele (SBM), a congenital neurodevelopmental disorder, involves dysmorphology of the cerebellum, and its most obvious manifestations are motor deficits. This paper reviews cerebellar neuropathology and motor function across several motor systems well studied in SBM in relation to current models of cerebellar motor and timing function. Children and adults with SBM have widespread motor deficits in trunk, upper limbs, eyes, and speech articulators that are broadly congruent with those observed in adults with cerebellar lesions. The structure and function of the cerebellum are correlated with a range of motor functions. While motor learning is generally preserved in SBM, those motor functions requiring predictive signals and precise calibration of the temporal features of movement are impaired, resulting in deficits in smooth movement coordination as well as in the classical cerebellar triad of dysmetria, ataxia, and dysarthria. That motor function in individuals with SBM is disordered in a manner phenotypically similar to that in adult cerebellar lesions, and appears to involve similar deficits in predictive cerebellar motor control, suggests that age-based cerebellar motor plasticity is limited in individuals with this neurodevelopmental disorder.
脊髓脊膜膨出(SBM)是一种先天性神经发育障碍,涉及小脑的畸形,其最明显的表现是运动功能缺陷。本文综述了 SBM 中几个运动系统的小脑神经病理学和运动功能,这些系统与当前的小脑运动和定时功能模型有关。SBM 患儿和成人在躯干、上肢、眼睛和言语发音器官方面存在广泛的运动缺陷,与成人小脑损伤观察到的缺陷大致一致。小脑的结构和功能与多种运动功能相关。虽然 SBM 中的运动学习通常得到保留,但那些需要预测信号和精确校准运动时间特征的运动功能受损,导致运动协调不流畅以及经典的小脑三联征(运动失调、共济失调和构音障碍)受损。SBM 个体的运动功能表现出与成人小脑损伤相似的紊乱方式,并且似乎涉及到类似的预测性小脑运动控制缺陷,这表明该神经发育障碍患者的基于年龄的小脑运动可塑性是有限的。