ACR Meeting Abstracts

ACR Meeting Abstracts

  • Home
  • Meetings Archive
    • ACR Convergence 2022
    • ACR Convergence 2021
    • ACR Convergence 2020
    • 2020 ACR/ARP PRSYM
    • 2019 ACR/ARP Annual Meeting
    • 2018 ACR/ARHP Annual Meeting
    • 2017-2009 Meetings
    • Download Abstracts
  • Keyword Index
  • Advanced Search
  • Your Favorites
    • Favorites
    • Login
    • View and print all favorites
    • Clear all your favorites
  • Meeting Resource Center

Abstract Number: 274

Childhood Onset Angiitis of the Central Nervous System: What Outcomes Can We Expect?

Lena Das1, Sook Fun Hoh2, Terrence Thomas3 and Thaschawee Arkachaisri4, 1Pediatric Rheumatology, KK Women's and Children's Hospital, Singapore, Singapore, 2Nursing, KK Women's and Children's Hospital, Singapore, Singapore, 3Pediatric Neurology, KK Women's and Children's Hospital, Singapore, Singapore, 4Rheumatology & Immunology, KK Women's and Children's Hospital and Duke-NUS Graduate Medical School, Singapore, Singapore

Meeting: 2012 ACR/ARHP Annual Meeting

Keywords: central nervous system involvement, pediatrics and vasculitis

  • Tweet
  • Email
  • Print
Session Information

Session Title: Pediatric Rheumatology - Clinical and Therapeutic Aspects: Pediatric Systemic Lupus Erythematosus, Pediatric Vasculitis and Pediatric Myositis

Session Type: Abstract Submissions (ACR)

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

Patient

Age (years)

Gender

Type*

Presentation

Treatment

Follow-up Time (months)

Initial MRA

MRA Outcome

Clinical Outcome**

1

6.4

M

P

R hemiparesis, GCS 15.

Aspirin, TCM

25

L MCA (M1)

L MCA (M1), ICA

No MD. No CI.

2

14

M

P

L hemiparesis, dysarthria, GCS 14.

Steroid pulses, CTX, MMF

17

R MCA (M1, M2)

R MCA (M1, M2), ICA

Improved MD. No CI.

3

9

M

P

 

R hemiparesis, aphasia, GCS 15.

Steroid pulses, CTX, MMF

21

L MCA,  ACA (A1), ICA

No change

Improved MD. Improved CI. Mild aphasia.

4

8

F

S

R hemiparesis, aphasia, GCS 11.

Steroid pulses, CTX

7

L MCA (M1, M3, M4)

L MCA (M1)

No MD. No CI.

5

12

M

S

L hemiparesis, dysarthria, GCS 15.

Steroid pulses, CTX

3

R ICA, L ACA (A1)

Improved R ICA, L ACA

No MD. No CI.

6

1.75

M

S

Status epilepticus, GCS 12.

 Acyclovir

54

L MCA (M1), ICA

L MCA (M1), ICA

Improved MD. Improved CI. Ongoing seizures.

7

14

F

P

L hemiparesis , status epilepticus, GCS 6.

Steroid pulses, CTX, MMF

31

L MCA (M1)

L MCA (M1)

No MD. Improved CI.

*Type: P Primary, S Secondary; GCS Glasgow Coma Scale; MCA Middle cerebral artery; ACA anterior cerebral artery; ICA internal carotid artery; TCM Traditional Chinese Medicine; CTX Cyclophosphamide; *Clinical Outcome: MD Motor Deficit; CI Cognitive Impairment

 


Disclosure:

L. Das,
None;

S. F. Hoh,
None;

T. Thomas,
None;

T. Arkachaisri,
None.

  • Tweet
  • Email
  • Print

« Back to 2012 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/childhood-onset-angiitis-of-the-central-nervous-system-what-outcomes-can-we-expect/

Advanced Search

Your Favorites

You can save and print a list of your favorite abstracts during your browser session by clicking the “Favorite” button at the bottom of any abstract. View your favorites »

ACR Pediatric Rheumatology Symposium 2020

© COPYRIGHT 2023 AMERICAN COLLEGE OF RHEUMATOLOGY

Wiley

  • Home
  • Meetings Archive
  • Advanced Search
  • Meeting Resource Center
  • Online Journal
  • Privacy Policy
  • Permissions Policies
  • Cookie Preferences