We aimed to characterize the clinicoradiographic course of isolated focal intracranial arteriopathy (FIA) in a single-center cohort of young adults with acute ischemic stroke (AIS) or transient ischemic attack (TIA).

Vasculopathy is the most common cause of stroke in children, with a significant subset due to Focal Cerebral Arteriopathy (FCA), a well-described inflammatory arteriopathy associated with preceding infection.  Focal arteriopathy in young adult stroke has also been described; while some overlap with childhood arteriopathies is suspected, the entity is poorly understood in this population.  To date, only one series has described FIA in a small sample of young adults.

We retrospectively reviewed all patients aged 18-49 admitted to a single center inpatient stroke unit between 2015-2018.  FIA was defined as a focal, non-embolic, steno-occlusion of the intracranial circulation causing AIS or TIA. 

Of 145 young adults admitted for AIS/TIA, 11 (7.6%) demonstrated FIA.  Stuttering/recurrent TIA was the presenting feature in 8/11 (72.7%) patients; 3/11 (27.3%) presented solely with AIS.  Preceding/concomitant illness was documented in 2/11 (18.2%) patients.  Focal stenosis was seen in eight patients, with possible occlusion in three.  Abnormal collaterals were seen in 5/11 patients.  Of seven patients with vessel-wall imaging (VWI), three demonstrated concentric enhancement, one demonstrated eccentric enhancement, and three demonstrated no enhancement.  Eight patients had follow-up imaging; radiographic improvement was seen in two, stability in three, and progression in three. Recurrent AIS/TIA occurred in 3/11 patients; in two patients, this was within one week of the presenting event.  All patients with recurrence had oligemia on pre-discharge perfusion imaging.  The most common suspected etiology was atherosclerosis (3/11), while an inflammatory etiology was suspected in two patients.

FIA is an uncommon cause of AIS/TIA in young adults, and may share clinicoradiographic features with childhood FCA.  VWI may help inform etiology, while persistent hypoperfusion may predict recurrent ischemic events.