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The commonest clinical presentations of the sporadic cerebral amyloid angiopathies are with either recurrent lobar haemorrhages or with dementia.1 In addition, transient focal neurological syndromes have occasionally been reported in association with cerebral amyloid angiopathies. These may represent transient ischaemic events or possibly focal seizures related to petechial intraparenchymal haemorrhages which are not associated with neuroimaging abnormalities; large haemorrhages may follow the onset of transient symptoms by weeks or months.2 3 We report on a patient with multifocal cortical myoclonus who subsequently proved to have amyloid β-peptide cerebral amyloid angiopathy, an association not previously described. We consider possible pathogenetic interrelations of these findings.
A 65 year old woman presented with a 4 year history of involuntary movements. She had been noted to have abnormal jerking movements of her legs—for example, when climbing the stairs— which, on occasion had caused her to fall. Using an electric vacuum cleaner or hearing the telephone ring had been noted to trigger these involuntary movements. There was no history of cognitive impairment. Her medical history was unremarkable aside from treated hypothyroidism. Family history was positive for sudden death, all three of her elder siblings (one sister, two brothers) dying in their mid-60s. One had previously had angina, the other two had been healthy until the time of their deaths, which were ascribed to a “heart attack” and a “clot …