Iizuka O, Soma Y, Otsuki M, Endo K, Tanno Y, Tsuji S
Department of Neurology, Niigata University, Japan.
No To Shinkei. 1997 Sep;49(9):841-5.
We report a patient of posterior cortical atrophy (PCA) with progressive memory disturbance and incomplete Bálint's syndrome consisting of optic ataxie (ataxie optique type) and visual inattention without psychic paralysis of fixation of gaze. The patients is a 58-year-old woman who noticed memory disturbance at 53 years old. Neurological deficit at 54 years old was detected only in the domain of memory, and mild diffuse brain atrophy was revealed on MRI. Memory disturbance progressed gradually, and at the age of 58 she was noticed to have visual disorder. Neuropsychological examination revealed severe memory disorder, incomplete Bálint's syndrome, transcortical sensory aphasia, mild ideational apraxia, and severe constructional apraxia. Visual inattention was too severe to evaluate visual acuity and visual field. MRI showed moderate dilatation of bilateral lateral ventricles, especially in their posterior horns, with atrophy of bilateral temporo-parieto-occipital lobes and hippocampus. IMP-SPECT revealed a diffuse decrease of cerebral blood flow in the bilateral temporo-parieto-occipital region, predominantly in the parietal regions. We believe that she is still in the early phase of PCA, and that psychic paralysis of fixation of gaze, visual agnosia will be noted in several years. Our patient represents an example of early stage PCA from neuropsychological and MRI findings.
我们报告了一例后皮质萎缩(PCA)患者,该患者有进行性记忆障碍,且患有不完全性巴林特综合征,表现为视觉性共济失调(光学性共济失调型)和视觉不注意,无凝视固定性精神麻痹。患者为一名58岁女性,53岁时出现记忆障碍。54岁时仅在记忆领域检测到神经功能缺损,MRI显示轻度弥漫性脑萎缩。记忆障碍逐渐进展,58岁时发现有视觉障碍。神经心理学检查显示严重记忆障碍、不完全性巴林特综合征、经皮质感觉性失语、轻度观念性失用症和严重结构性失用症。视觉不注意严重到无法评估视力和视野。MRI显示双侧侧脑室中度扩张,尤其是后角,双侧颞顶枕叶和海马萎缩。IMP-SPECT显示双侧颞顶枕叶区域脑血流弥漫性减少,主要在顶叶区域。我们认为她仍处于PCA的早期阶段,几年后将出现凝视固定性精神麻痹、视觉失认。从神经心理学和MRI检查结果来看,我们的患者代表了早期PCA的一个例子。