Jambaqué I, Chiron C, Dulac O, Raynaud C, Syrota P
Neuropediatric Department, Hôpital Saint-Vincent-de-Paul, Paris, France.
Epilepsia. 1993 Jul-Aug;34(4):692-700. doi: 10.1111/j.1528-1157.1993.tb00448.x.
Visual behavior is frequently impaired at onset of West syndrome (WS). We studied the neuropsychological outcome of eight children who had cryptogenic WS and moderate to severe visual impairment at the onset of epilepsy. At the last examination, a regional cerebral blood flow study using SPECT (single photon emission computed tomography) was performed. The behavior abnormalities observed initially evolved to various defects of cognitive function. Three patients had severe mental retardation with autistic features. Two had marked speech disorders but one had global cognitive impairment. Three patients had specific visual-spatial deficits. SPECT showed perfusion defects involving the parieto-occipital areas in 6 of 8 patients. These abnormalities were restricted to parieto-occipital regions in the three patients with selective visual-spatial deficits, whereas they were associated with other perfusion defects in the three remaining patients. This study demonstrates that the visual inattention observed at the onset of WS is frequently associated with long-term cognitive and/or perfusion defects involving the parieto-occipital regions.
在韦斯特综合征（WS）发病时，视觉行为常常受损。我们研究了8名隐源性WS且癫痫发作时伴有中度至重度视力损害儿童的神经心理学转归。在最后一次检查时，使用单光子发射计算机断层扫描（SPECT）进行了局部脑血流研究。最初观察到的行为异常逐渐演变为各种认知功能缺陷。3例患者有严重智力障碍并伴有孤独症特征。2例有明显的言语障碍，但1例有全面认知障碍。3例患者有特定的视觉空间缺陷。SPECT显示8例患者中有6例存在涉及顶枕区的灌注缺陷。在3例有选择性视觉空间缺陷的患者中，这些异常仅限于顶枕区，而在其余3例患者中，它们与其他灌注缺陷相关。这项研究表明，在WS发病时观察到的视觉注意力不集中常常与涉及顶枕区的长期认知和/或灌注缺陷相关。
