后部皮质萎缩:1例报告及文献复习

徐旭华, 汤可娜

神经病学与神经康复学杂志 ›› 2025, Vol. 21 ›› Issue (1) : 26-33.

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神经病学与神经康复学杂志 ›› 2025, Vol. 21 ›› Issue (1) : 26-33. DOI: 10.12022/jnnr.2024-0219
病例报告

后部皮质萎缩:1例报告及文献复习

  • 徐旭华,汤可娜
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Posterior cortical atrophy: a case report and literature review

  • XU Xuhua, TANG Kena
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摘要

目的报道1例后部皮质萎缩(posterior cortical atrophy,PCA)患者的临床特征、诊断过程和治疗方案,并结合文献复习,提高临床医生对该病的认识。

方法对 1 例 PCA 患者的临床资料进行分析,结合文献复习疾病的临床特征,诊断方法,鉴 别诊断和治疗策略。

结果患者,男性,67岁,以记忆力下降3年余为主诉就诊。临床表现为进行性视空间障碍、视知觉障碍和Gerstmann综合征。神经心理学评估显示整体认知功能波动性下降,早期记忆力相对保留。头颅MRI显示顶叶、枕叶及颞叶萎缩,以顶叶萎缩最为突出。脑脊液检查显示 Aβ1-42/Aβ1-40 比值降低和磷酸化 Tau 蛋白(181)升高。诊断为阿尔茨海默病 PCA。给予乙酰胆碱酯酶抑制剂和N-甲基-D-天冬氨酸受体拮抗剂治疗。

结论PCA 是一种以视觉和空间认知功能障碍为主要特征的神经退行性疾病,临床表现独特,易被误诊或漏诊。提高对PCA的认识,结合临床表现、神经心理学评估、影像学检查和生物标志物检测,有助于早期诊断和干预。

Abstract

Objective: To report the clinical features, diagnostic process, and treatment strategy of a patient with posterior cortical atrophy (PCA), and to enhance the awareness of peers on this subject through a literature review.

Methods: We analyzed the clinical data of a PCA patient and summaried the literature on the clinical characteristics, diagnostic methods, differential diagnosis, and treatment strategies of the disease.

Results: A 67-year-old male patient presented with a chief complaint of memory decline for over 3 years. The clinical manifestations included progressive visuospatial deficits, visual perceptual impairments, and Gerstmanns syndrome. The neuropsychological assessment of the patient revealed there's a global fluctuating cognitive decline accompanied by relatively preserved early memory. Brain MRI of the patient showed the atrophy in the parietal, occipital, and temporal lobes, among which the parietal lobe being most prominently affected. The analysis of cerebrospinal fluid(CSF) demonstrated the Aβ1-42/Aβ1-40 ratio decreased and the phosphorylated Tau protein (181) increased in the patient's CSF. Overall, the patient was diagnosed with Alzheimer's disease-related posterior cortical atrophy. Treatment strategies of this patient included applying acetylcholinesterase inhibitors and Nmethyl-D-aspartate receptor antagonists.

Conclusion: PCA is a neurodegenerative disorder primarily characterized by visual and spatial cognitive impairments. Its unique clinical presentation makes it prone to misdiagnosis or underdiagnosis. Improving awareness of PCA and combining clinical manifestations, neuropsychological assessments, imaging studies, and biomarker detection can facilitate early diagnosis and intervention.

关键词

后部皮质萎缩  /   / 阿尔茨海默病  /   / 视空间障碍  /   / Gerstmann综合征

Key words

Posterior cortical atrophy / Alzheimer's disease / Visuospatial deficits / Gerstmanns syndrome

引用本文

导出引用
徐旭华, 汤可娜. 后部皮质萎缩:1例报告及文献复习[J]. 神经病学与神经康复学杂志. 2025, 21(1): 26-33 https://doi.org/10.12022/jnnr.2024-0219
XU Xuhua, TANG Kena. Posterior cortical atrophy: a case report and literature review[J]. Journal of Neurology and Neurorehabilitation. 2025, 21(1): 26-33 https://doi.org/10.12022/jnnr.2024-0219

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