Department of Neurology, Christian Doppler University Hospital, Salzburg, Austria.
Neuroscience Institute, Christian Doppler University Hospital, Centre for Cognitive Neuroscience, Paracelsus Medical University, Salzburg, Austria.
Int J Stroke. 2024 Mar;19(3):314-321. doi: 10.1177/17474930231203133. Epub 2023 Sep 29.
The transmission of amyloid β (Aβ) in humans leading to iatrogenic cerebral amyloid angiopathy (iCAA) is a novel concept with analogies to prion diseases. However, the number of published cases is low, and larger international studies are missing.
We aimed to build a large multinational collaboration on iCAA to better understand the clinical spectrum of affected patients.
We collected clinical data on patients with iCAA from Austria, Croatia, Italy, Slovenia, and Spain. Patients were included if they met the proposed Queen Square diagnostic criteria (QSC) for iCAA. In addition, we pooled data on disease onset, latency, and cerebrospinal fluid (CSF) biomarkers from previously published iCAA cases based on a systematic literature review.
Twenty-seven patients (22% women) were included in this study. Of these, 19 (70%) met the criteria for probable and 8 (30%) for possible iCAA. Prior neurosurgical procedures were performed in all patients (93% brain surgery, 7% spinal surgery) at median age of 8 (interquartile range (IQR) = 4-18, range = 0-26 years) years. The median symptom latency was 39 years (IQR = 34-41, range = 28-49). The median age at symptom onset was 49 years (IQR = 43-55, range = 32-70). Twenty-one patients (78%) presented with intracranial hemorrhage and 3 (11%) with seizures.
Our large international case series of patients with iCAA confirms a wide age boundary for the diagnosis of iCAA. Dissemination of awareness of this rare condition will help to identify more affected patients.
人类中淀粉样蛋白β（Aβ）的传播导致医源性脑淀粉样血管病（iCAA），这是一个与朊病毒疾病类似的新概念。然而，已发表的病例数量较少，且缺乏更大规模的国际研究。
我们旨在建立一个大型跨国 iCAA 合作组织，以更好地了解受影响患者的临床谱。
我们从奥地利、克罗地亚、意大利、斯洛文尼亚和西班牙收集了 iCAA 患者的临床数据。如果患者符合 iCAA 的 Queen Square 诊断标准（QSC），则将其纳入研究。此外，我们还根据系统文献回顾，汇总了先前发表的 iCAA 病例中关于发病时间、潜伏期和脑脊液（CSF）生物标志物的数据。
本研究纳入了 27 例患者（22%为女性）。其中，19 例（70%）符合可能 iCAA 的标准，8 例（30%）符合可能 iCAA 的标准。所有患者均接受过神经外科手术（93%为脑部手术，7%为脊髓手术），手术中位数年龄为 8 岁（四分位距（IQR）=4-18 岁，范围=0-26 岁）。中位症状潜伏期为 39 年（IQR=34-41 岁，范围=28-49 岁）。中位症状发作年龄为 49 岁（IQR=43-55 岁，范围=32-70 岁）。21 例（78%）患者表现为颅内出血，3 例（11%）患者表现为癫痫发作。
我们的 iCAA 大型国际病例系列研究证实了 iCAA 诊断的年龄范围很广。对这种罕见疾病的认识的传播将有助于识别更多受影响的患者。
