Does Transient Cerebral Arteriopathy exist in adults? The course of unilateral intracranial large-artery arteriopathy.

The majority of childhood arterial ischaemic stroke (AIS) is caused by cerebral
arteriopathies. The increased use of vascular imaging techniques has led to the
recognition of a newly identified arteriopathic entity: *Transient Cerebral
Arteriopathy* (TCA), a unilateral intracranial non-progressive arteriopathy of
presumed inflammatory origin. To diagnose TCA, a follow-up angiogram showing
stabilisation or regression of vascular pathology is required.
In circa 30% of young adults with AIS the cause of stroke remains unknown
despite an extensive diagnostic work-up. Hitherto, TCA has only been
demonstrated in children. However, unilateral intracranial non-atherosclerotic
stenotic arterial disease is also encountered in young adults. The angiographic
course of these arteriopathies in this age group is unknown, since follow-up
vascular imaging is generally not performed.

Primary aim: to investigate the possible existence of *transient* or
non-progressive non-atherosclerotic arteriopathy as a cause of AIS in young
adults, and to describe the course of young adulthood unilateral intracranial
arterial disease.
Secondary aim: to study the possible etiological factors contributing to
unilateral intracranial large-artery arteriopathy in young adults.

From the Utrecht Stroke Database we identified a cohort of 18 (out of 773) AIS
patients between 16 and 50 years with anterior circulation unilateral
intracranial large-artery arteriopathy as demonstrated with vascular imaging
performed within three months of disease onset.
Data from the patients* medical record regarding history, examination, outcome
and laboratory results will be retrospectively collected to obtain insight into
the possible nature of the arteriopathy. The patients will undergo a clinical
follow-up examination, including assessment of functional outcome by means of
the modified Rankin scale and a magnetic resonance angiogram (MRA). The
vascular imaging at disease onset will be reviewed and compared with the
follow-up MRA to investigate the course (progression, stabilisation or
regression) of the arteriopathy. Possible atherosclerotic and prothrombotic
risk factors will be investigated to assess their relevance in the pathogenesis
of the cerebral arteriopathy.

Patients will visit the hospital twice for approximately two hours at each
visit.
We do not expect adverse events from the one-time blooddrawing nor from the MR
investigation. Some patients may feel claustrophobic during the MR.

The study may give insight in the cause of ischemic stroke in young patients
with unilateral intracranial arteriopathy.