Shamitha Goonewardene is a Junior Medical Officer currently working at Manning Base Hospital, New South Wales, Australia. He obtained his MBBS degree fro Monash University and his main interests lie in the fields of Cardiology, Neurology and Nephrology.
A Case Report Of Congophilic Angiopathy Related Dementia
A 74-year-old male was referred to Geriatric Medicine clinic with a 12-month history of progressive short-term cognitive deficits and word finding difficulties. This is on a background of essential hypertension, coronary artery disease and stable depression. On neurocognitive testing, he scored 16/30 on MMSE and 10/30 on MoCA indicative of severe cognitive impairment. Cerebral imaging revealed mild cortical atrophy. No reversible causes of dementia were identified. He was diagnosed as probable Alzheimer’s dementia (AD) and a trial of acetylcholinesterase inhibitors (Donepezil) was recommended. A follow up MRI brain scan however revealed cortical infarction and haemorrhage involving both frontal lobes and multiple areas of microhaemorrhages. These findings were consistent with cerebral amyloid angiopathy (CAA).
Given this patient’s high risk of spontaneous lobar haemorrhage, aspirin was ceased. Over the next 9-month period, his cognition and language significantly declined and he lost a further 12-points on his MMSE score.
CAA is an age-related small vessel disease due to amyloid deposits in the media of the cerebral cortex and leptomeninges. It can occur in association with AD and is an important cause of lobar haemorrhage in the elderly. CAA presents as a diverse clinical spectrum ranging from transient isolated focal neurological episodes to rapid progressive cognitive decline. The radiological correlate is incidental microbleeds on MRI, as in our case. T2-weighed gradient-echo MRI sequences are the gold standard. The number of microbleeds is one useful prognostic factor. Importantly, there is no clinical association between CAA and systemic amyloidosis.
Our current understanding of CAA is still evolving. To date, there are no specific treatments available. However, minimising the risk of recurrent intracerebral haemorrhages is central to the management of CAA. This involves optimal blood pressure control and avoidance of antiplatelet and anticoagulant therapies, unless the competing benefits override their potential harm.