Jambaqué I, Mottron L, Ponsot G, Chiron C
Hôpital Saint Vincent de Paul, Service de Neuropédiatrie, Université René Descartes, Paris, France.
J Neurol Neurosurg Psychiatry. 1998 Oct;65(4):555-60. doi: 10.1136/jnnp.65.4.555.
Autistic disorder is a developmental handicap with an unknown neurological basis. Current neuropsychological models for autism suggest an abnormal construction of visual perceptual representation or a deficit in executive functions. These models predict cerebral lesions in the temporo-occipital or frontal regions of autistic patients. The present study aimed at studying the presence of symptoms of autism and visual agnosia in a 13 year old girl who had a right temporo-occipital cortical dysplasia that was surgically removed at the age of 7.
Neuropsychological evaluation included Wechsler and Kaufman intelligence scales, a test of word fluency, digit span, Corsi block, California verbal learning, Trail making, Benton facial recognition, Snoodgrass object recognition tests, Rivermead face learning subtest, and developmental test of visual perception. The ADI-R was used to show current and retrospective diagnosis of autistic disorder. Neuroimagery included brain MRI, single photon emission computed tomography (SPECT), and PET.
Brain MRI showed a right occipital defect and an abnormal hyperintensity of the right temporal cortex. PET and SPECT disclosed a left frontal hypometabolism together with the right occipital defect. Neuropsychological testing showed a visual apperceptive agnosia and executive function deficits. Psychiatric study confirmed the diagnosis of autistic disorder.
Although the possibility that autism and visual agnosia were dissociable factors in this patient cannot be excluded, the finding of both deficits supports the possibility that occipito-temporal lesions can predispose to the development of autism.
自闭症谱系障碍是一种神经学基础不明的发育障碍。目前针对自闭症的神经心理学模型表明,视觉感知表征构建异常或执行功能存在缺陷。这些模型预测自闭症患者颞枕叶或额叶区域存在脑部病变。本研究旨在对一名13岁女孩进行研究,该女孩患有右侧颞枕叶皮质发育异常,7岁时接受了手术切除,研究其是否存在自闭症和视觉失认症症状。
神经心理学评估包括韦氏和考夫曼智力量表、词汇流畅性测试、数字广度测试、科西方块测试、加利福尼亚言语学习测试、连线测试、本顿面部识别测试、斯诺德格拉斯物体识别测试、里弗米德面部学习子测试以及视觉感知发育测试。使用孤独症诊断访谈修订版(ADI-R)来显示自闭症谱系障碍的当前和回顾性诊断。神经影像学检查包括脑部磁共振成像(MRI)、单光子发射计算机断层扫描(SPECT)和正电子发射断层扫描(PET)。
脑部MRI显示右侧枕叶缺损以及右侧颞叶皮质异常高信号。PET和SPECT显示左侧额叶代谢减低以及右侧枕叶缺损。神经心理学测试显示存在视觉认知性失认症和执行功能缺陷。精神病学研究确诊为自闭症谱系障碍。
尽管不能排除自闭症和视觉失认症在该患者中是可分离因素的可能性,但两种缺陷的发现支持枕颞叶病变可能易导致自闭症发展的可能性。