Abstract: Exploration of Association of Menarche with Age at Onset of Pediatric Nmo (2016 CMSC Annual Meeting)

EG06
Exploration of Association of Menarche with Age at Onset of Pediatric Nmo

Thursday, June 2, 2016

Exhibit Hall

Tanuja Chitnis, MD , Massachusetts General Hospital for Children, Boston, MA

Jennifer Graves, MD, PhD , UCSF Pediatric MS Center, San Francisco, CA

Cody S Olsen, MS , Neurology, University of Utah, Salt Lake City, UT

Jayne Ness, MD , Alabama Pediatric MS Center, Birmingham, AL

Lauren Krupp, MD , Lourie Center for Pediatric MS, Stony Brook Children's Hospital, Stony Brook, NY

Anita Belman, MD , Lourie Center for Pediatric MS, Stony Brook Children's Hospital, Stony Brook, NY

Moses Rodriguez, MD , Department of Neurology, Mayo Clinic, Rochester, MN

Jan Tillema, MD , Pediatric MS Center, Mayo Clinic, Rochester, MN

Tim Lotze, MD, PhD , Texas Children's Hospital, Houston, TX

Mark Gorman, MD , Massachusetts General Hospital, Partners Pediatric MS Center, Boston, MA

Leslie Benson, MD , Boston Children's Hospital, Boston, MA

Bianca Weinstock-Guttman, MD , New York State Multiple Sclerosis Consortium, Buffalo, NY

Greg Aaen, MD , Department of Child Neurology, Loma Linda University, Loma Linda, CA

John Rose, MD , Department of Neurology University of Utah and Neurovirology Research Laboratory VASLCHCS, Imaging and Neuroscience Center, Salt Lake City, UT

T. Charles Casper, PhD , Department of Pediatrics, University of Utah, Salt Lake City, UT

Emmanuelle Waubant, MD, PhD , UCSF Pediatric MS Center, San Francisco, CA

Tanuja Chitnis, MD , Massachusetts General Hospital for Children, Boston, MA

Background: Neuromyelitis optica (NMO) is a female predominant autoimmune neurological disease. Although puberty is a key period of sexual differentiation, little is known about the onset of NMO in relation to the onset of puberty.

Objectives: To assess the effect of age at puberty onset on the age of onset of NMO in children and adolescents.

Methods: This is a multicenter cohort study of 84 pediatric NMO patients collected at 9 clinical centers across the U.S between 2011-2015. Clinical data are recorded in a standardized database forms managed by the University of Utah. 381 Pediatric controls were collected at the same centers as part of a case:control MS study. Age at menarche and Tanner staging is recorded at clinical visits. Patients were classified as NMO IgG positive or negative.

Results: Of the 84 pediatric NMO patients, 65 were girls and 19 were boys. The sex ratio of patients with onset <10 years was 3.7 girls:1 boy and for onset 10 years and older it was 3.25 girls:1 boy. 48% of NMO-IgG+ girls, and 52% of NMO-IgG negative girls had first symptoms of NMO prior to menarche. Mean age at menarche was younger in post-menarcheal-onset NMO girls (11.3 years versus 12.0 years, p=0.04 from Van Elteren’s test adjusting for race/ethnicity), compared to controls collected at the same centers. Age at menarche and age at onset were not correlated in all girls (Spearman’s r=0.21, p=0.21), but were positively correlated among post-menarcheal-onset girls (Spearman’s rho=0.42, p=0.03). In girls, 29 had Tanner stage data within 12 months of onset, with 8 stage 1, 3 stage 2, 5 stage 3, 9 stage 4, and 4 stage 5. In the boys, 6 had Tanner staging measured within 12 months of first symptom onset. Of these, 1 was Tanner stage (pubic hair) 2, 3 were stage 3, and 1 each were stages 4 and 5.

Conclusions: Approximately half of pediatric girls with NMO had onset prior to menarche, regardless of NMO IgG status. There is a female predominance both pre- and post-pubertal-onset patients. Further studies are required to explore the role of sexual dimorphism and sex hormones in NMO.

EG06 Exploration of Association of Menarche with Age at Onset of Pediatric Nmo

EG06
Exploration of Association of Menarche with Age at Onset of Pediatric Nmo