Department of Advanced Medical and Surgical Sciences, University of Campania "L. Vanvitelli", P.zza L. Miraglia 2, 80138 Naples, Italy.
Second Neurological Clinic, University of Campania "L. Vanvitelli", Isola 8, Edificio 10 Policlinico, "Federico II" via Pansini 5, 80131 Napoli, Italy.
Rev Neurosci. 2022 Jun 6;33(8):849-858. doi: 10.1515/revneuro-2022-0003. Print 2022 Dec 16.
Posterior cortical atrophy (PCA) is a neurodegenerative disorder characterized by an early prominent deficit of visual functions associated with signs and symptoms that are the expression of dysfunction of posterior brain regions. Although PCA is commonly associated with Alzheimer's disease (AD), in recent years new pathological substrates have emerged. Among them, frontotemporal lobar degeneration (FTLD) is the most commonly reported but, to date, little is known about the clinical features of PCA due to FTLD. We conducted a systematic search in the main biomedical database MEDLINE. We searched for all clinical PCA reports that assessed the pathological basis of such syndrome with at least one of the following: (1) neuropathological examination, (2) cerebrospinal fluid biomarkers, (3) amyloid-PET imaging and (4) genetic testing. Of 369 potentially eligible studies, 40 fulfilled the inclusion criteria with an overall number of 144 patients (127 PCA-AD vs. 17 PCA-FTD/non-AD). We found that hallucinations/illusions were present in none of the probable PCA-FTD/non-AD subjects while were reported in 15 out of 97 PCA-AD individuals. Optic ataxia and Parkinsonism showed a significantly greater prevalence in probable PCA FTD/non-AD than in PCA-AD whereas myoclonus and disorientation in time and space were significantly more frequent in PCA-AD than in probable PCA FTD/non-AD. We also found a predominance of a left-side pattern of atrophy/hypometabolism in the probable PCA FTD/non-AD. Clinical features such as optic ataxia, Parkinsonism, myoclonus, hallucinations and disorientation in time and space suggest the underlying pathological basis of PCA and help in leading the diagnostic protocol consequently.
后部皮质萎缩症（PCA）是一种神经退行性疾病，其特征是早期明显的视觉功能缺陷，伴有大脑后部区域功能障碍的表现和症状。尽管 PCA 通常与阿尔茨海默病（AD）相关，但近年来出现了新的病理基础。在这些基础中，额颞叶变性（FTLD）是最常见的报道，但迄今为止，由于 FTLD，对于 PCA 的临床特征知之甚少。我们在主要的生物医学数据库 MEDLINE 中进行了系统搜索。我们搜索了所有评估这种综合征病理基础的临床 PCA 报告，这些报告至少使用了以下一种方法：（1）神经病理学检查，（2）脑脊液生物标志物，（3）淀粉样蛋白-PET 成像和（4）基因测试。在 369 项可能符合条件的研究中，有 40 项符合纳入标准，共有 144 名患者（127 名 PCA-AD 与 17 名 PCA-FTD/非 AD）。我们发现，在可能的 PCA-FTD/非 AD 患者中，没有出现幻觉/错觉，而在 97 名 PCA-AD 患者中有 15 名出现幻觉/错觉。在可能的 PCA FTD/非 AD 中，视性共济失调和帕金森病的患病率明显高于 PCA-AD，而在 PCA-AD 中，肌阵挛和时间及空间定向障碍的发生率明显高于可能的 PCA FTD/非 AD。我们还发现，在可能的 PCA FTD/非 AD 中，左侧萎缩/代谢低下的模式占主导地位。视性共济失调、帕金森病、肌阵挛、幻觉和时间及空间定向障碍等临床特征提示 PCA 的潜在病理基础，并有助于相应地指导诊断方案。
