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Cerebrovascular pathology in cerebral amyloid angiopathy presenting as intracerebral haemorrhage

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Abstract

Cerebral amyloid angiopathy (CAA) is the second most common cause of non-traumatic intracerebral haemorrhage (ICH) accounting for 12–15% of lobar haemorrhages in the elderly. Definitive diagnosis of CAA requires histological evaluation. We aimed to evaluate the spectrum of cerebrovascular changes in CAA-related ICH. Between 2011 and 2015, biopsy-confirmed cases of CAA were retrieved and clinical, radiological and pathological features were reviewed. The spectrum of vascular alterations was evaluated and amyloid deposition was graded in accordance with the Greenberg and Vonsattel scale. Seven cases of sporadic CAA [5 males and 2 females] were diagnosed, none of whom were suspected to have CAA pre-operatively. Six presented with large intracerebral haematoma (ICH) requiring neurosurgical intervention (age range: 56–70 years) and one had episodic headache and multiple microhaemorrhages requiring a diagnostic brain biopsy (45 years). In the presence of large ICH, vascular amyloid deposits were of moderate to severe grade (grade 4 in 4, grades 2 and 3 in 1 case each) with predominant involvement of medium (200–500 μm) to large (> 500 μm) leptomeningeal vessels. Fibrinoid necrosis was noted in four. Two were hypertensive and on antiplatelet agents. β-Amyloid plaques were detected in two, one of whom had symptomatic dementia. MRI performed in 3 of 6 cases with ICH did not reveal any microhaemorrhages. Amyloid deposits in small (50–200 μm) to medium (200–500 μm) calibre intracortical vessels produced parenchymal microhemorrhages. Histopathological examination of ICH is essential for diagnosing CAA. The vascular calibre rather than grade of amyloid deposits dictates size of the bleed. Presence of co-morbidities such as antiplatelet agents may predispose to haemorrhage.

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Acknowledgements

We would like to acknowledge Dr. Natarajan M, Neurosugeon, K G Hospital, Coimbatore, Tamil Nadu, India for referring a case for histopathological diagnosis.

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Authors and Affiliations

  1. Department of Neuropathology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, Karnataka, 560029, India

    Rajalakshmi Poyuran, Anita Mahadevan, B. N. Nandeesh & Yasha T. Chickabasaviah

  2. Department of Neurosurgery, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, 560029, Karnataka, India

    Arivazhagan Arimappamagan & K. V. L. Narasinga Rao

  3. Department of Neurology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, 560029, Karnataka, India

    Madhu Nagappa

  4. Department of Neuroimaging and Interventional Radiology, National Institute of Mental Health & Neurosciences (NIMHANS), Bangalore, 560029, Karnataka, India

    Jitender Saini

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  1. Rajalakshmi Poyuran
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Contributions

Author 1, Rajalakshmi Poyuran, -analysed the cases; performed data collection, analysis and interpretation; drafted the manuscript and reviewed the literature.

Author 2, Anita Mahadevan, conceived and designed the study, performed data interpretation and analysis and critically reviewed and finalized the manuscript.

Authors 3 and 7, Arimappamagan Arivazhagan and K V L Narasinga Rao, are neurosurgeons who operated and managed the patients and performed clinical data acquisition, analysis and review of the manuscript with intellectual inputs.

Authors 4 and 8, Nandeesh BN and Yasha T Chickabasaviah, performed diagnosis of cases, data analysis and interpretation; reviewed the manuscript and provided inputs.

Author 5, Madhu Nagappa, is a neurologist who managed the patients, provided and interpreted clinical data, performed clinicopathological analysis and reviewed the manuscript with intellectual inputs.

Author 6, Jitender Saini, is a neuroradiologist who carried out neuroimaging studies, reviewed imaging findings and analysed and interpreted the neuroimaging data and reviewed the manuscript providing critical inputs.

All the authors gave final approval for publication and agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Corresponding author takes full responsibility for the work as a whole, including the study design, access to data and the decision to submit and publish the manuscript.

Corresponding author

Correspondence to Anita Mahadevan.

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Poyuran, R., Mahadevan, A., Arimappamagan, A. et al. Cerebrovascular pathology in cerebral amyloid angiopathy presenting as intracerebral haemorrhage. Virchows Arch 474, 235–245 (2019). https://doi.org/10.1007/s00428-018-2505-z

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