Institute of Neuroradiology, University Hospital, Goethe University Frankfurt, Frankfurt am Main, Germany.
Department of Neurology, University Hospital, Goethe University Frankfurt, Frankfurt am Main, Germany.
Eur J Neurol. 2024 Oct;31(10):e16425. doi: 10.1111/ene.16425. Epub 2024 Aug 6.
Although the Boston criteria version 2.0 facilitates the sensitivity of cerebral amyloid angiopathy (CAA) diagnosis, there are only limited data about precursor symptoms. This study aimed to determine the impact of neurological and imaging features in relation to the time of CAA diagnosis.
Patients diagnosed with probable CAA according to the Boston criteria version 1.5, treated between 2010 and 2020 in our neurocentre, were identified through a keyword search in our medical database. Neuroimaging was assessed using Boston criteria versions 1.5 and 2.0. Medical records with primary focus on the clinical course and the occurrence of transient focal neurological episodes were prospectively evaluated.
Thirty-eight out of 81 patients (46.9%) exhibited transient focal neurological episodes, most often sensory (13.2%) or aphasic disorders (13.2%), or permanent deficits at a mean time interval of 31.1 months (SD ±26.3; range 1-108 months) before diagnosis of probable CAA (Boston criteria version 1.5). If using Boston criteria version 2.0, all patients receiving magnetic resonance imaging (MRI) met the criteria for probable CAA, and diagnosis could have been made on average 44 months earlier. Four patients were younger than 50 years, three of them with supporting pathology. Cognitive deficits were most common (34.6%) at the time of diagnosis.
Non-haemorrhagic MRI markers enhance the sensitivity of diagnosing probable CAA; however, further prospective studies are proposed to establish a minimum age for inclusion. As the neurological overture of CAA may occur several years before clinical diagnosis, early clarification by MRI including haemosensitive sequences are suggested.
尽管波士顿标准 2.0 版提高了脑淀粉样血管病(CAA)诊断的敏感性,但关于前驱症状的数据有限。本研究旨在确定与 CAA 诊断时间相关的神经和影像学特征的影响。
通过在我们的医学数据库中使用关键词搜索,确定了在我们的神经中心根据波士顿标准 1.5 版诊断为可能的 CAA 并在 2010 年至 2020 年期间接受治疗的患者。使用波士顿标准 1.5 版和 2.0 版评估神经影像学。前瞻性评估以临床病程和短暂性局灶性神经发作发生为主要重点的病历记录。
81 例患者中有 38 例(46.9%)出现短暂性局灶性神经发作,最常见的是感觉(13.2%)或失语障碍(13.2%),或在诊断为可能的 CAA(波士顿标准 1.5 版)前平均 31.1 个月(标准差 ±26.3;范围 1-108 个月)出现永久性缺陷。如果使用波士顿标准 2.0 版,所有接受磁共振成像(MRI)的患者均符合可能的 CAA 标准,并且平均可以提前 44 个月做出诊断。有 4 名患者年龄小于 50 岁,其中 3 名有支持性病理。诊断时最常见的是认知障碍(34.6%)。
非出血性 MRI 标志物提高了诊断可能的 CAA 的敏感性;然而,建议进一步开展前瞻性研究以确定纳入的最小年龄。由于 CAA 的神经发作可能在临床诊断前数年发生,因此建议通过包括敏感序列的 MRI 尽早明确。