From the Stroke Research Centre (L.P., D.J.W.), Department of Brain Repair and Rehabilitation, UCL Queen Square Institute of Neurology, London, United Kingdom; Department of Neurology (L.P.), Bogomolets National Medical University, Kyiv, Ukraine; MRC Prion Unit at UCL (G.B.), Institute of Prion Diseases; National Hospital for Neurology and Neurosurgery (G.B., R.O., G.H., H.R.J.), Queen Square, University College London Hospitals NHS Foundation Trust; Neuroradiological Academic Unit (D.H.M., H.R.J.), UCL Queen Square Institute of Neurology; Lysholm Department of Neuroradiology (D.H.M.), National Hospital for Neurology and Neurosurgery; National Hospital for Neurology and Neurosurgery (V.H.), Queen Square, University College London Hospitals NHS Foundation Trust; Torbay and South Devon NHS Foundation Trust (W.K.); York and Scarborough Teaching Hospitals NHS Foundation Trust (S.D.); Queen Square Institute of Neurology and National Hospital for Neurology and Neurosurgery (M.S.Z.), University College London Hospitals NHS Foundation Trust; and Department of Neuroinflammation (M.S.Z.), UCL Queen Square Institute of Neurology, London, United Kingdom.
Neurology. 2024 Dec 24;103(12):e210084. doi: 10.1212/WNL.0000000000210084. Epub 2024 Nov 25.
Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a subtype of CAA with distinct clinical and radiologic features. Existing diagnostic criteria require the presence of characteristic asymmetrical white matter hyperintensity (WMH), together with classical hemorrhagic neuroimaging markers of CAA. There are limited data for other diagnostic neuroimaging markers of CAA-ri.
This is a case series from a specialist hospital intracerebral hemorrhage service.
We describe 4 patients with CAA-ri who had regions of sulcal hyperintensity, with or without gyral swelling at clinical presentation, but did not fulfill current diagnostic criteria because of the absence of typical asymmetric WMH on brain MRI. All 4 patients were subsequently diagnosed with CAA-ri; three later developed asymmetric WMHs with disease relapse, and 2 had pathologically proven CAA-ri; 1 patient had both.
Regions of sulcal hyperintensity, sometimes with associated gyral swelling, can be an early imaging finding in CAA-ri. These neuroimaging markers could potentially improve the accuracy of existing diagnostic criteria for CAA-ri to allow earlier diagnosis and treatment without biopsy in patients with atypical presentations.
脑淀粉样血管病相关性炎症(CAA-ri)是 CAA 的一个亚型,具有独特的临床和影像学特征。现有的诊断标准需要存在特征性的不对称性脑白质高信号(WMH),以及 CAA 的经典出血性神经影像学标志物。对于 CAA-ri 的其他诊断性神经影像学标志物,数据有限。
这是来自一家专科医院脑出血服务的病例系列。
我们描述了 4 例 CAA-ri 患者,他们在临床出现时存在脑沟高信号区域,有或没有脑回肿胀,但由于脑 MRI 上没有典型的不对称性 WMH,不符合当前的诊断标准。所有 4 例患者随后均被诊断为 CAA-ri;其中 3 例后来出现了不对称性 WMH,疾病复发,2 例有病理证实的 CAA-ri;1 例两者均有。
脑沟高信号区域,有时伴有脑回肿胀,可能是 CAA-ri 的早期影像学表现。这些神经影像学标志物可能会提高现有的 CAA-ri 诊断标准的准确性,从而允许在不进行活检的情况下,对表现不典型的患者进行更早的诊断和治疗。