Paediatric acquired demyelinating syndromes: incidence, clinical and magnetic resonance imaging features

Abstract

Objective:Changing trends in multiple sclerosis (MS) epidemiology may first be apparent in the childhood population affected with first onset acquired demyelinating syndromes (ADSs). We aimed to determine the incidence, clinical, investigative and magnetic resonance imaging (MRI) features of childhood central nervous system ADSs in the British Isles for the first time.Methods:We conducted a population active surveillance study. All paediatricians, and ophthalmologists (n = 4095) were sent monthly reporting cards (September 2009–September 2010). International Paediatric MS Study Group 2007 definitions and McDonald 2010 MS imaging criteria were used for acute disseminated encephalomyelitis (ADEM), clinically isolated syndrome (CIS) and neuromyelitis optica (NMO). Clinicians completed a standard questionnaire and provided an MRI copy for review.Results:Card return rates were 90%, with information available for 200/222 positive notifications (90%). After exclusion of cases, 125 remained (age range 1.3–15.9), with CIS in 66.4%, ADEM in 32.0% and NMO in 1.6%. The female-to-male ratio in children older than 10 years (n = 63) was 1.52:1 (p = 0.045). The incidence of first onset ADS in children aged 1–15 years old was 9.83 per million children per year (95% confidence interval [CI] 8.18–11.71). A trend towards higher incidence rates of ADS in children of South Asian and Black ethnicity was observed compared with White children. Importantly, a number of MRI characteristics distinguished ADEM from CIS cases. Of CIS cases with contrast imaging, 26% fulfilled McDonald 2010 MS diagnostic criteria.Conclusions:We report the highest surveillance incidence rates of childhood ADS. Paediatric MS diagnosis at first ADS presentation has implications for clinical practice and clinical trial design.

Pediatric transverse myelitis

“…8 Although males are more likely to present with ATM (male:female ratio 1.1-1.6:1), a female preponderance is seen among teenagers in regions at high risk for MS and NMO (United States, Canada, Europe, and parts of Australia). [6][7][8][9][10][11][12] A bimodal age distribution is observed in children under 5 and older than 10 years of age. 3,8,9,[11][12][13] There is no difference in ethnicity prevalence.…”

“…ATM accounts for one-fifth of children experiencing a first ADS. 6,7 TM is more common in adults, but children account for 20% of cases. 8 Although males are more likely to present with ATM (male:female ratio 1.1-1.6:1), a female preponderance is seen among teenagers in regions at high risk for MS and NMO (United States, Canada, Europe, and parts of Australia).…”

Recovery From Central Nervous System Acute Demyelination in Children

“…1 Most prospective cohort studies of ADS in children have focused on the genetic, environmental, and neuroimaging predictors of multiple sclerosis (MS). [2][3][4][5] Relatively less is known regarding the severity of the acute incident demyelinating event and the predictors of residual attack-related physical disability.…”

Therapeutic Approach to the Management of Pediatric Demyelinating Disease: Multiple Sclerosis and Acute Disseminated Encephalomyelitis

“…In pediatric ADS cohorts, optic neuritis constitutes 22-36 %, transverse myelitis 3-22 %, and ADEM 19-32 % of cases. Other monofocal or polyfocal presentations (21-46 %) comprise the remainder of ADS cases [1][2][3]5].…”