Abstract
Cerebral amyloid angiopathy (CAA) is characterised by the progressive accumulation of β-amyloid (Aβ) in the walls of cerebral capillaries and arteries representing a major cause of haemorrhagic stroke including lobar intracerebral haemorrhage (ICH) and convexity subarachnoid haemorrhage (SAH). Haemorrhaging from CAA predominantly involves smaller arteries rather than arterial aneurysm. Restricted bleeding into the subarachnoid space in CAA results in asymptomatic or mild symptomatic SAH. Herein, we present an autopsied case of massive SAH related to CAA. An 89-year-old male with a history of mild Alzheimer’s disease (AD) and advanced pancreatic cancer with liver metastasis developed sudden onset of coma. Head CT illustrated ICH located in the right frontal lobe and right insula, as well as SAH bilaterally spreading from the basal cistern to the Sylvian fissure, with hydrocephalus and brain herniation. He died about 24 h after onset and the post-mortem examination showed no evidence of arterial aneurysm. The substantial accumulation of Aβ in the vessels around the haemorrhagic lesions led to the diagnosis of ICH related to CAA and secondary SAH, which may have been aggravated by old age and malignancy. This case suggests that CAA can cause severe SAH resembling aneurysmal origin and thus may be overlooked when complicated by atypical cerebral haemorrhage.
Highlights
Cerebral amyloid angiopathy (CAA) is a cerebrovascular amyloidosis and a known cause of haemorrhagic stroke
The presented case was CAA-related intracerebral haemorrhage (ICH) accompanied by massive subarachnoid haemorrhage (SAH), the volume of parenchymal haematoma was relatively small (Patel et al, 2009)
Severe SAH resembling that of aneurysmal origin is rare in CAA (Ohshima et al, 1990; Charidimou et al, 2015; Ni et al, 2015; Raposo et al, 2018)
Summary
Cerebral amyloid angiopathy (CAA) is a cerebrovascular amyloidosis and a known cause of haemorrhagic stroke. Bleeding into the subarachnoid space is common in CAA, presenting as convexity subarachnoid haemorrhage (SAH) in acute and superficial siderosis in the chronic phase. Pancreatic cancer with liver metastasis was diagnosed 8 months before admission, and palliative care had been performed He had a history of mild AD at the age of 88 and brainstem haemorrhage at 82. Head CT showed right frontal lobe and insula ICH adjacent to the brain surface (Figure 1). He was diagnosed as ICH with secondary SAH and subsequently died approximately 24 h after onset. ICH with subarachnoid haemorrhage (SAH) extension was observed in the right hemisphere. Which led to the pathological diagnoses of ICH related to CAA with SAH extension. Written informed consent for autopsy and the publication was obtained from his legal representatives
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