Abstract
A 75 year-old retired primary school teacher developed multiple sequential strokes over a three month period. MRI brain showed ischaemic strokes of different ages across multiple vascular territories, involving the right genu of the corpus callosum and the left posterior limb of the internal capsule. MR angiography demonstrated multifocal intracranial arterial segmental narrowing, while CT angiography showed no evidence of vasculopathy in the remainder of the medium or large vessels of the neck, thorax or abdomen. Digital subtraction angiogram demonstrated severe intracranial arteriopathy, with multiple stenoses in the posterior, middle and anterior cerebral arteries bilaterally. FDG-PET showed no abnormal glucose uptake in the large or medium blood vessels of the body, suggesting against a systemic vasculitis with extracranial vessel involvement. Cerebrospinal fluid analysis was non-contributory. Random skin biopsies yielded no evidence of intravascular lymphoma. Given recurrent ischaemic strokes with multifocal arterial vessel changes confined to the intracranial circulation, she was diagnosed with primary angiitis (vasculitis) of the central nervous system. She was treated with six cycles of intravenous cyclophosphamide and has had no further events.
In the accompanying poster, the digital subtraction angiography images are presented demonstrating classical features of primary angiitis of the central nervous system. This submission is intended as a teaching case in neuroimaging.