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Focal cerebral arteriopathy (FCA) is a monophasic inflammatory stenosis most often affecting the supraclinoid internal carotid artery (ICA) or the proximal segment of the middle cerebral artery (MCA). It accounts for more than half of acute ischaemic stroke (AIS) cases in young children but is a less familiar entity for adult neurologists.

Over the 2 years, there were 5 cases of AIS secondary to FCA with an average age (range) of 43 (32 – 55) years. There were 2 males, and 3 females. In those aged 16-50 years, there was an estimated incidence of 2.5%. With the exception of an incidental case, 4 cases presented with non-abrupt, stuttering focal strokes affecting one hemisphere over days to months. All cases had unilateral focal vessel wall irregularity typically involving the distal ICA and proximal MCA confirmed on CT angiogram. Interval imaging demonstrated resolution in 1 case and improvement in 3 cases. No case demonstrated interval progression. Functional outcome was excellent with discharge Modified Rankin Scale ranging from 0-1. Recurrence occurred in 1 case.

FCA is a rare, and perhaps seldom recognized, cause of AIS in young adults. It has a highly characteristic presentation, with step-wise AIS events which crescendo over days to months.  It highlights the importance of initial and follow-up intracranial imaging in select cases to help differentiate from progressive arteriopathies. Evidence based treatment warrants further investigation. Prognosis is favourable. This contributes to the paucity of data on AIS secondary to FCA in adults. Results in young adults are similar to those published in the paediatric literature.