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Clinical, laboratory, and radiological features of cerebral amyloid angiopathy-related inflammation (CAA-ri): retrospective, observational experience of a single centre

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Abstract

Background

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a subtype of CAA with an inflammatory response to the vascular β-amyloid deposits. Reliable and non-invasive clinical diagnostic methods may allow patients to avoid the side effects of brain biopsy.

Objective

In this observational study, we retrospectively analyzed the clinical, laboratory, radiological features, treatment, and outcome of patients diagnosed with CAA-ri. The main purpose is to enhance knowledge of CAA-ri and to avoid misdiagnosis.

Methods

We described 15 consecutive patients with probable or possible CAA-ri at Henan Provincial People’s Hospital according to a validation study of proposed criteria for the diagnosis of CAA-ri. The clinical features, imaging, laboratory findings, and treatment which included the response to immunotherapy were revealed in the study.

Results

The median age of 15 patients was 67.0 years (range 48.0–90.0 years), and the male-to-female ratio was 7: 8. In our study, the most common clinical manifestations were cognitive decline (7/15, 46.7%), focal neurologic deficit (6/15, 40.0%), and headache (5/15, 33.3%). In terms of imaging results, white matter hyperintensity (WMH) lesions were rarely seen in the cerebellum and brainstem, while no hemorrhagic lesion was observed in the brainstem of all 15 patients. In addition, 12 patients (80.0%) showed improvement or stability for the clinical and radiological outcomes after immunotherapy.

Conclusion

CAA-ri should be considered as a differential diagnosis when brain MRI shows typical features in the elderly. Once the diagnosis is established, immunotherapy should be initiated as early as possible to promote neurological function recovery and reduce recurrence.

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Funding

This work was supported by grant from the National Natural Science Foundation of 643 China (No. 81873727).

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Author notes

  1. Dan Li and Weiwei Qin contributed equally to this work

Authors and Affiliations

  1. Department of Neurology, Henan Provincial People’s Hospital, People’s Hospital of Zhengzhou University, People’s Hospital of Henan University, No. 7 Weiwu Road, Jinshui District, Zhengzhou, 450003, Henan, China

    Dan Li, Weiwei Qin, Mingrong Xia, Jiewen Zhang & Weizhou Zang

  2. Department of Neurosurgery, Henan Provincial People’s Hospital, People’s Hospital of Zhengzhou University, People’s Hospital of Henan University, No. 7 Weiwu Road, 450003, Zhengzhou, China

    Yang Guo

  3. Department of Neurology, Dengfeng People’s Hospital, Dengfeng, 452470, Henan, China

    Siyuan Li

Authors
  1. Dan Li
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  2. Weiwei Qin
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  3. Yang Guo
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  4. Mingrong Xia
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Correspondence to Weizhou Zang.

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This study was approved by the local Institutional Review Board and all written informed consent was obtained from all patients included.

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Li, D., Qin, W., Guo, Y. et al. Clinical, laboratory, and radiological features of cerebral amyloid angiopathy-related inflammation (CAA-ri): retrospective, observational experience of a single centre. Neurol Sci 44, 631–638 (2023). https://doi.org/10.1007/s10072-022-06436-7

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  • DOI: https://doi.org/10.1007/s10072-022-06436-7

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