Ishiwata Akiko, Kimura Kazumi
Departments of Neurological Science, Graduate School of Medicine, Nippon Medical School.
J Nippon Med Sch. 2016;83(2):87-92. doi: 10.1272/jnms.83.87.
Posterior cortical atrophy (PCA) is a rare neurodegenerative disorder that has cerebral atrophy in the parietal, occipital, or occipitotemporal cortices and is characterized by visuospatial and visuoperceptual impairments. The most cases are pathologically compatible with Alzheimer's disease (AD). We describe a case of PCA in which a combination of imaging methods, in conjunction with symptoms and neurological and neuropsychological examinations, led to its being diagnosed and to AD being identified as its probable cause. Treatment with donepezil for 6 months mildly improved alexia symptoms, but other symptoms remained unchanged. A 59-year-old Japanese woman with progressive alexia, visual deficit, and mild memory loss was referred to our neurologic clinic for the evaluation of right homonymous hemianopsia. Our neurological examination showed alexia, constructional apraxia, mild disorientation, short-term memory loss, and right homonymous hemianopsia. These findings resulted in a score of 23 (of 30) points on the Mini-Mental State Examination. Occipital atrophy was identified, with magnetic resonance imaging (MRI) showing left-side dominance. The MRI data were quantified with voxel-based morphometry, and PCA was diagnosed on the basis of these findings. Single photon emission computed tomography with (123)I-N-isopropyl-p-iodoamphetamine showed hypoperfusion in the corresponding voxel-based morphometry occipital lobes. Additionally, the finding of hypoperfusion in the posterior associate cortex, posterior cingulate gyrus, and precuneus was consistent with AD. Therefore, the PCA was considered to be a result of AD. We considered Lewy body dementia as a differential diagnosis because of the presence of hypoperfusion in the occipital lobes. However, the patient did not meet the criteria for Lewy body dementia during the course of the disease. We therefore consider including PCA in the differential diagnoses to be important for patients with visual deficit, cognitive impairment, and cerebral atrophy in the parietal, occipital, or occipitotemporal cortices. A combination of imaging methods, including MRI and single photon emission computed tomography, may help identify probable causes of PCA.
后部皮质萎缩(PCA)是一种罕见的神经退行性疾病,其特征为顶叶、枕叶或枕颞叶皮质出现脑萎缩,并伴有视觉空间和视觉感知障碍。大多数病例在病理上与阿尔茨海默病(AD)相符。我们描述了一例PCA病例,通过综合运用多种成像方法,并结合症状、神经学和神经心理学检查,最终得以确诊,并确定AD为其可能病因。使用多奈哌齐治疗6个月后,失读症状略有改善,但其他症状未变。一名59岁的日本女性,出现进行性失读、视力缺陷和轻度记忆力减退,因右侧同向性偏盲被转诊至我们的神经科门诊进行评估。我们的神经学检查发现患者存在失读、结构失用、轻度定向障碍、短期记忆力减退和右侧同向性偏盲。这些表现使得简易精神状态检查表(Mini-Mental State Examination)得分为23分(满分30分)。通过磁共振成像(MRI)发现枕叶萎缩,且左侧更为明显。利用基于体素的形态学测量法对MRI数据进行量化分析,基于这些结果诊断为PCA。单光子发射计算机断层扫描(123)I-N-异丙基-p-碘安非他明显示,基于体素的形态学测量法对应的枕叶存在灌注不足。此外,后部联合皮质、后扣带回和楔前叶灌注不足的表现与AD相符。因此,该PCA被认为是由AD引起的。由于枕叶存在灌注不足,我们将路易体痴呆作为鉴别诊断。然而,在病程中该患者不符合路易体痴呆的诊断标准。因此,我们认为对于出现视力缺陷、认知障碍以及顶叶、枕叶或枕颞叶皮质脑萎缩的患者,将PCA纳入鉴别诊断十分重要。包括MRI和单光子发射计算机断层扫描在内的多种成像方法的联合应用,可能有助于确定PCA的可能病因。