Vinck A, Maassen B, Mullaart R, Rotteveel J
Department of Medical Psychology, Radboud University Medical Centre, Nijmegen, The Netherlands.
J Neurol Neurosurg Psychiatry. 2006 Sep;77(9):1083-6. doi: 10.1136/jnnp.2005.075887. Epub 2006 May 11.
Spina bifida is a multifaceted neurological condition with complex neuropsychological sequelae. The cognitive outcome in spina bifida has frequently been attributed to the severity of the hydrocephalus. However, because of complex neuropathology, the influence of hydrocephalus alone does not sufficiently explain the deficits in the cognitive profile in spina bifida. To date, little is known of the role of Arnold-Chiari-II malformation (ACM) in the cognitive profile of these patients. Aim of the current study is to delineate the specific contribution of the ACM in spina bifida by comparing children with ACM and those without ACM. 46 children between 6 and 15 years of age underwent a neuropsychological assessment covering intelligence and a wide range of cognitive functions, such as visuo-motor processing, attention, memory, word fluency and speed of information processing. Comparisons were made between patients with ACM (ACM+) and those without ACM (ACM-); all children with ACM+ also had hydrocephalus. Confounding effects of global cognitive impairment were excluded, such that groups were matched on verbal IQ. Because of complex neuropathology, which is inherent to spina bifida, the method applied was based on a comparison of cognitive profiles of the study group with profiles of patients with cerebellar damage and hydrocephalus found in the literature. Impaired visual analysis and synthesis, verbal memory, and verbal fluency, even after correction for global cognitive impairment, were observed in children with ACM. The hypothesis that in addition to impairment in visual analysis and synthesis, which are related to both hydrocephalus and ACM, specific deficiencies in verbal memory and fluency may be attributed to ACM is supported.
脊柱裂是一种具有复杂神经心理学后遗症的多方面神经系统疾病。脊柱裂患者的认知结果常常被归因于脑积水的严重程度。然而，由于复杂的神经病理学原因，仅脑积水的影响并不能充分解释脊柱裂患者认知特征方面的缺陷。迄今为止，关于阿诺德 - 奇阿里Ⅱ型畸形（ACM）在这些患者认知特征中的作用知之甚少。本研究的目的是通过比较患有ACM和未患有ACM的儿童，来描绘ACM在脊柱裂中的具体作用。46名6至15岁的儿童接受了涵盖智力和广泛认知功能的神经心理学评估，如视觉运动处理、注意力、记忆、词汇流畅性和信息处理速度。对患有ACM的患者（ACM +）和未患有ACM的患者（ACM -）进行了比较；所有ACM +的儿童也患有脑积水。排除了整体认知障碍的混杂影响，使两组在言语智商上相匹配。由于脊柱裂固有的复杂神经病理学，所应用的方法基于将研究组的认知特征与文献中发现的小脑损伤和脑积水患者的特征进行比较。在患有ACM的儿童中，即使在纠正了整体认知障碍之后，仍观察到视觉分析与综合、言语记忆和言语流畅性受损。这一假设得到了支持，即除了与脑积水和ACM都相关的视觉分析与综合受损之外，言语记忆和流畅性方面的特定缺陷可能归因于ACM。
