ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 1661

The Spectrum of childhood Inflammatory Brain Diseases; An Increasingly Recognised Field

Marinka Twilt1, Eyal Muscal2, Tania Cellucci3, Pavla Dolezalova4, Rob Forsyth5, Susan Kim6, David A. Cabral7, Johannes Roth8, Adam Kirton9, Rolando Cimaz10, Jasmin Kummerle-Deschner11, Annet van Royen-Kerkhof12, Jürgen Brunner13, Gaelle Chedeville14, Jordi Anton15, Angela Robinson16, Mary Toth17, Tilmann Kallinich18, Claudia Bracaglia19, Alexis Boneparth20, Shehla Sheikh21 and Susanne M. Benseler21, 1Division of Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada, 2TCH Pediatric Rheum Center, Baylor College of Medicine, Houston, TX, 3Rheumatology, Hamilton Health Sciences, McMaster, Hamilton, ON, Canada, 4Pediatric Rheumatology Unit, Department of Pediatrics and Adolescent Medicine, General University Hospital in Prague, Prague, Czech Republic, 5Newcastle upon Tyne's NHS foundation Trust, Newcastle upon Tyne, United Kingdom, 6Division of Immunology, Boston Children's Hospital, Boston, MA, 7University of British Columbia, Vancouver, BC, Canada, 8University of Ottawa, Ottawa, ON, Canada, 9Neurology, Alberta Children's Hospital, Calgary, AB, Canada, 10Rheumatology Unit, A. Meyer Children's Hospital, Florence, Italy, 11University Childrens Clinic Tuebingen, Tuebingen, Germany, 12Department of Pediatric Immunology & Rheumatology, Wilhelmina Children’s Hospital, University Medical Center Utrecht, Utrecht, Netherlands, 13pediatric Rheumatology, University Children's Hospital, Innsbruck, Austria, 14Pediatric Rheumatology, Montreal Children's Hospital, Montreal, QC, Canada, 15Rheumatology, Hospital Sant Joan de Deu, Barcelona, Spain, 16Pediatrics, Rainbow Babies & Children's Hospital / Case Medical Center, Cleveland, OH, 17Rheumatology, Akron Children's Hospital, Akron, OH, 18Charite, University Medicine Berlin, Berlin, Germany, 19Department of Pediatric Medicine, Division of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS, Rome, Italy, 20Pediatrics, The Children's Hospital at Montefiore, Bronx, NY, 21Rheumatology, The Hospital for Sick Children, Toronto, ON, Canada

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: ,

Session Information

Title: Vasculitis II

Session Type: Abstract Submissions (ACR)

Background/Purpose:

Childhood Inflammatory Brain diseases are potentially life-threatening diseases leading to severe neurological deficits if not treated. The spectrum of childhood inflammatory brain diseases is rapidly expanding, and recent evidence shows the presence of inflammatory markers in previously thought to be vasculopathies, such as Moyamoya. We reviewed all pediatric patients enrolled in the international BrainWorks database.

Methods:

All pediatric patients enrolled through a BrainWorks center were identified in the BrainWorks database. Patients were eligible for inclusion if predetermined information at the baseline visit was available. This included, information on demographic information, such as age at diagnosis and gender, diagnosis at enrollment, clinical, laboratory, neuroradiology and if applicable brain biopsy.

Results:

In total 370 (190 boys, 180 girls) children were enrolled in BrainWorks with complete dataset at baseline. The mean age at diagnosis was 9.33 years (boys 9.67 years, girls 9.07 years). The diagnosis included were; Non-progressive large vessel CNS vasculitis n=108 (68 boys, 40 girls, mean age 8.15 years), Small vessel CNS vasculitis n = 76 (26 boys, 50 girls, mean age 11 years), Progressive large vessel CNS vasculitis n=25 (17 boys, 8 girls, mean age 10.3 years), NMDAR-encephalitis n=42 (15 boys, 27 girls, mean age 9.9 years), secondary CNS vasculitis due to underlying systemic disease n=33 (16 boys, 17 girls), and Moyamoya n=33 (18 boys, 15 girls). Other diagnosis included CNS vasculitis due to infection n=20, ADEM n=5, MS n=2, Rasmussen n=2, and other vasculopathies/vasculitis (n-21).

Focal deficits at presentation were more commonly seen in patients with large vessel CNS vasculitis and infantile Moyamoya, while diffuse deficits were seen more in children with small vessel CNS vasculitis and NMDAR encephalitis. Seizures were seen in all inflammatory brain diseases, but were more frequently present in NMDAR encephalitis and small vessel CNS vasculitis (80% and 61%) compared to the large vessel CNS vasculitis subtypes and Moyamoya.

Conclusion:

Childhood inflammatory Brain diseases encompasses many different diagnosis. The most frequent diagnosis are the different subtypes of childhood CNS vasculitis, however NMDAR encephalitis and Moyamoya are increasingly recognized and diagnosed.

Disclosure:

M. Twilt,
None;

E. Muscal,
None;

T. Cellucci,
None;

P. Dolezalova,
None;

R. Forsyth,
None;

S. Kim,
None;

D. A. Cabral,
None;

J. Roth,
None;

A. Kirton,
None;

R. Cimaz,
None;

J. Kummerle-Deschner,
None;

A. van Royen-Kerkhof,
None;

J. Brunner,
None;

G. Chedeville,
None;

J. Anton,

Research grant,

2,

Consulting fees ,

5,

Speakers’ bureau ,

8;

A. Robinson,
None;

M. Toth,
None;

T. Kallinich,
None;

C. Bracaglia,
None;

A. Boneparth,
None;

S. Sheikh,
None;

S. M. Benseler,
None.

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