Background/Purpose: Imaging findings in large-medium vessel Childhood Onset Primary and Secondary Angiitis of the Central Nervous System (cPACNS & cSACNS) are well described, however, structural changes of individual cerebral vessels after treatment has not been described. Our aim was to describe clinical outcomes and vessel progression using structural magnetic resonance angiography (MRA).

Methods: Children before age 16 with acute onset neuro-deficit and MRA findings suggestive of vasculitis-irregularity and/or narrowing of medium to large-sized cerebral vessels with repeated MRA studies, evaluated by pediatric rheumatologists and neurologists at KK Women’s and Children’s Hospital from January 2011 – December 2012, were included. Patient demographic and clinical data were analysed. Median and interquartile range (IQR) were used for descriptive data.

Results: 7 patients were identified, 4 – cPACNS and 3 – cSACNS (2 – Takayasu Arteritis (TA), 1 – post-varicella CNS vasculitis). Median onset age was 9.0 years (IQR 6.4-14.0) with 5 males. Median follow-up duration was 21 months (IQR 7.0-31.0) with 3-6 months duration for repeated MRI/MRA (median numbers of MRI/MRA of 4 (IQR 2.0-6.0)). Motor deficits (hemiparesis) were present in 6/7, status epilepticus in 2/7 and aphasia and/or dysarthria in 4/7. Patient characteristics and distribution/sizes of cerebral vessels involved, along with the clinical outcomes and vessel progression after therapy is depicted in Table 1.

Upon a median follow-up of 21 months, all patients improved or had resolution of their initial motor deficits. They had also returned to school although 3/7 had continued cognitive impairment. None had clinical relapse despite further reduction of affected vessels (patient 3 at month 3, patient 1 at month 6 and patient 2 at month 9). All main arteries (MCA-M1, ACA-A1, ICA) were not recanalized at the end of the follow-up.

Conclusion: Despite our small cohort and regardless of the cause, after almost 2 years of follow-up, main arteries including MCA-M1 and ACA-A1, did not seem to recanalize. Interval improvements on MRA seemed to be limited to smaller M2 and M3 branches. Clinical outcomes, especially motor deficits may not follow the cerebral vessel course over time, however. Due to the rarity of diseases, multicenter with larger cohort studies are needed to confirm our initial observations and this is ongoing in our region.

Table 1: Demographic, Clinical and Vessel Progression in Children with Angiitis of the CNS