A nationwide survey of pediatric acquired demyelinating syndromes in Japan

Abstract

Objective:To investigate the clinical and epidemiologic features of pediatric acquired demyelinating syndromes (ADS) of the CNS in Japan.Methods:We conducted a nationwide survey and collected clinical data on children with ADS aged 15 years or younger, who visited hospitals between 2005 and 2007.Results:Among 977 hospitals enrolled, 723 (74.0%) responded to our inquiries and reported a total of 439 patients as follows: 244 with acute disseminated encephalomyelitis (ADEM), 117 with multiple sclerosis (MS), 14 with neuromyelitis optica (NMO), and 64 with other ADS. We collected and analyzed detailed data from 204 cases, including those with ADEM (66), MS (58), and NMO (10). We observed the following: (1) the estimated annual incidence rate of pediatric ADEM in Japan was 0.40 per 100,000 children (95% confidence interval [CI], 0.34–0.46), with the lowest prevalence in the north; (2) the estimated prevalence rate of MS was 0.69 per 100,000 children (95% CI, 0.58–0.80), with the lowest prevalence in the south; (3) NMO in Japan was rare, with an estimated prevalence of 0.06 per 100,000 children (95% CI, 0.04–0.08); and (4) the sex ratio and mean age at onset varied by ADS type, and (5) male/female ratios correlated with ages at onset in each ADS group.Conclusions:Our results clarify the characteristic clinical features of pediatric ADS in the Japanese population.

Myelin oligodendrocyte glycoprotein and aquaporin‐4 antibodies are highly specific in children with acquired demyelinating syndromes

“…Relapsing cases were assigned the following diagnostic categories: (1) multiple sclerosis, fulfilling the 2013 International Pediatric Multiple Sclerosis Study Group consensus criteria and the 2010 revised McDonald criteria; (2) NMOSD, fulfilling the 2015 Wingerchuk criteria; (3) multiphasic disseminated encephalomyelitis and ADEM‐optic neuritis fulfilling the 2013 International Pediatric Multiple Sclerosis Study Group consensus criteria; and (4) recurrent demyelination in a single central nervous system (CNS) area without evidence of clinically silent disease (for example relapsing optic neuritis).…”

“…Two studies looking at the prevalence of AQP4‐Ab in paediatric patients presenting with a first episode of demyelination have identified these in only 0.7% (2/279) to 4.5% (3/64) of children, which may reflect the rarity of NMOSD in certain populations. A recent nationwide study of Japanese children identified AQP4‐Ab in 1 out of 12 patients with multiple sclerosis and three out of six patients with NMOSD who were tested . Nevertheless, the severity of NMOSD and the role for specific therapies prompts testing for AQP4‐Ab in all children with ADS.…”

“…A recent nationwide study of Japanese children identified AQP4-Ab in 1 out of 12 patients with multiple sclerosis and three out of six patients with NMOSD who were tested. 5 Nevertheless, the severity of NMOSD and the role for specific therapies prompts testing for AQP4-Ab in all children with ADS.…”

Pediatric versus adult MS: similar or different?

“…In addition to numerous studies carried out in NorthAmerica and Europe, recent studies have been published on pediatric cohorts of selected populations (Hispanic North-Americans) [9] or from other countries such as Turkey, Kuwait, Iran, Brazil, Marroch, Korea and Japan [10][11][12][13][14][15][16][17], indicating an increased interest in this area of clinical research. Many papers have been recently published giving a better definition of incidence, clinical aspects, pathophysiology, immunology, neuroimaging and treatment: a summary of these studies is provided by a recent publication of the International Pediatric MS Study Group [18].…”

Fulminant Acute Disseminated Encephalomyelitis: A Remarkable Outcome with Cyclophosphamide

“…ADEM is a demyelinating disorder of the CNS with an estimated annual incidence of 0.23 to 0.4 of 100,000 children. [8][9][10][11] In 2007 the International Pediatric Multiple Sclerosis Society Group (IPMSSG) published consensus definitions for demyelinating disorders of childhood, including ADEM, 12 which was updated in 2013. 13 The clinical presentation has reportedly a latent period between anteceding viral infection and onset of symptoms of approximately 12 days, 14,15 during which prodromal symptoms can include fever, malaise, headache, nausea, and vomiting progressing to encephalopathy and coma.…”