Focal cerebral arteriopathy in adults: A single centre experience

Background and ObjectivesFocal cerebral arteriopathy (FCA) is a recognized cause of pediatric stroke, but its presentation in adults is poorly defined, with limited cohorts and scarce advanced imaging data. We aimed to describe the clinical spectrum, radiological features, treatment strategies, and outcomes of adult FCA in the largest single-center cohort to date. MethodsWe retrospectively reviewed consecutive adults (>18 years) with ischemic stroke admitted between 2017 and 2024. FCA was defined as unilateral focal stenosis/irregularity of anterior circulation arteries (terminal ICA, M1, M2, A1) after excluding mimics such as vasculitis, dissection, embolic occlusion, and intracranial atherosclerosis. All underwent MRI with contrast-enhanced vessel wall imaging and confirmation on a second modality. Clinical, radiological, and outcome data were collected. Severity was scored using the FCA Severity Score (FCASS). ResultsOf 2,237 stroke admissions, 47 patients (2.1%) met criteria for FCA. Median age was 30 years, with near-equal sex distribution. Hemiparesis with or without aphasia predominated, and strokes were generally mild (median NIHSS 3, mRS 1.2). Preceding clustered TIAs occurred in one-third, and 32% reported new unilateral headache. Infarcts often involved lenticulostriate and MCA watershed territories. Median FCASS was 4. Vessel wall imaging showed concentric enhancement in 74.5%. All patients received antiplatelets; 49% received additional immunosuppression, most often IV methylprednisolone. Over a median 6-month follow-up (mean 12.4), no stroke recurrences occurred, though 5 patients showed radiological progression and 2 developed contralateral stenosis. Functional outcomes were favorable, with 87% achieving mRS 0-2. DiscussionAdult FCA is uncommon but clinically distinct, marked by mild strokes, clustered TIAs, and concentric vessel wall enhancement. The course was largely monophasic, with favorable functional outcomes and no recurrent strokes, regardless of immunosuppressive therapy. Radiological progression was rare but included contralateral involvement, raising the possibility of overlap with unilateral moyamoya disease. Vessel wall imaging is valuable for diagnosis, and longer follow-up is needed to clarify pathogenesis and treatment strategies.