Sheth R D, Bodensteiner J B
Department of Neurology, West Virginia, University Health Sciences Center, Morgantown 26506-9180, USA.
Pediatr Neurol. 1995 Nov;13(4):352-4. doi: 10.1016/0887-8994(95)00220-0.
Approximately 15% of all cerebral arteriovenous malformations (AVMs) present with progressive neurologic deficits, the pathogenic mechanism of which has not been established. One suggestion is that AVMs by expanding over time compress normal surrounding cerebral parenchyma and thereby cause progressive neurologic impairment. Alternatively, a vascular steal results in progressive ischemia of normal cerebral tissue. Because the area occupied by the AVM and the area of observed blood flow reductions in all reported patients have overlapped, delineating the relative contribution of local compression from that of vascular steal has not been possible. We present a 7-month-old girl and a 7-year-old boy with AVMs restricted to the diencephalon who had progressive cognitive impairment and dystrophic cerebral hemispheral calcification (in the 7-month-old girl) indicating diffuse cerebral cortical involvement remote from the AVM. These patients provide evidence for vascular steal, and not local compression, as the primary mechanism underlying a progressive neurologic course associated with some AVMs.
所有脑动静脉畸形（AVM）中约15%会出现进行性神经功能缺损，其发病机制尚未明确。一种观点认为，AVM随时间推移不断扩大，压迫周围正常脑实质，从而导致进行性神经功能损害。另一种观点则认为，盗血现象会导致正常脑组织进行性缺血。由于在所有已报道的患者中，AVM所占区域与观察到的血流减少区域存在重叠，因此无法明确局部压迫和盗血现象各自的相对作用。我们报告了一名7个月大的女孩和一名7岁大的男孩，他们的AVM局限于间脑，出现了进行性认知障碍，且7个月大的女孩出现了营养不良性脑半球钙化，提示远离AVM的大脑皮质广泛受累。这些患者为盗血现象而非局部压迫是某些AVM相关进行性神经病程的主要机制提供了证据。
