A nationwide survey of pediatric acquired demyelinating syndromes in Japan

Y Yamaguchi; H Torisu; R Kira; Y Ishizaki; Y Sakai; M Sanefuji; T Ichiyama; A Oka; T Kishi; S Kimura; M Kubota; J Takanashi; Y Takahashi; H Tamai; J Natsume; S Hamano; S Hirabayashi; Y Maegaki; M Mizuguchi; K Minagawa; H Yoshikawa; J Kira; S Kusunoki; T Hara

doi:10.1212/WNL.0000000000003318

A nationwide survey of pediatric acquired demyelinating syndromes in Japan

Neurology. 2016 Nov 8;87(19):2006-2015. doi: 10.1212/WNL.0000000000003318. Epub 2016 Oct 14.

Authors

Y Yamaguchi¹, H Torisu², R Kira¹, Y Ishizaki¹, Y Sakai¹, M Sanefuji¹, T Ichiyama¹, A Oka¹, T Kishi¹, S Kimura¹, M Kubota¹, J Takanashi¹, Y Takahashi¹, H Tamai¹, J Natsume¹, S Hamano¹, S Hirabayashi¹, Y Maegaki¹, M Mizuguchi¹, K Minagawa¹, H Yoshikawa¹, J Kira¹, S Kusunoki¹, T Hara¹

Affiliations

¹ From the Department of Pediatrics (Y.Y., H. Torisu, R.K., Y.I., Y.S., M.S., T.H.) and Department of Neurology, Neurological Institute (J.K.), Graduate School of Medical Sciences, Kyushu University, Fukuoka; Department of Pediatrics (H. Torisu), Fukuoka Dental College Medical and Dental Hospital, Fukuoka; Department of Pediatrics (T.I.), Yamaguchi University Graduate School of Medicine, Ube; Department of Pediatrics (A.O.), Kyorin University School of Medicine, Hachioji; Department of Pediatrics (T.K.), Tokyo Women's Medical University, Tokyo; Department of Child Development Pediatrics (S. Kimura), Kumamoto University Graduate School, Kumamoto; Division of Neurology (M.K.), National Center for Child Health and Development, Tokyo; Department of Pediatrics (J.T.), Kameda Medical Center, Kamogawa; National Epilepsy Center (Y.T.), Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka; Department of Pediatrics (H. Tamai), Osaka Medical College, Takatsuki; Department of Pediatrics (J.N.), Nagoya University Graduate School of Medicine, Nagoya; Department of Neurology (S. Hamano), Saitama Children's Medical Center, Saitama; Department of Neurology (S. Hirabayashi), Nagano Children's Hospital, Azumino; Division of Child Neurology (Y.M.), Faculty of Medicine, Tottori University, Yonago; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, The University of Tokyo; Department of Pediatrics (K.M.), Hokkaido Medical Center for Child Health and Rehabilitation, Sapporo; Department of Pediatric Neurology (H.Y.), Nagaoka Habilitation and Medical Center for Severely Handicapped Children, Nagaoka; and Department of Neurology (S. Kusunoki), Kinki University Faculty of Medicine, Osaka-Sayama, Japan.
² From the Department of Pediatrics (Y.Y., H. Torisu, R.K., Y.I., Y.S., M.S., T.H.) and Department of Neurology, Neurological Institute (J.K.), Graduate School of Medical Sciences, Kyushu University, Fukuoka; Department of Pediatrics (H. Torisu), Fukuoka Dental College Medical and Dental Hospital, Fukuoka; Department of Pediatrics (T.I.), Yamaguchi University Graduate School of Medicine, Ube; Department of Pediatrics (A.O.), Kyorin University School of Medicine, Hachioji; Department of Pediatrics (T.K.), Tokyo Women's Medical University, Tokyo; Department of Child Development Pediatrics (S. Kimura), Kumamoto University Graduate School, Kumamoto; Division of Neurology (M.K.), National Center for Child Health and Development, Tokyo; Department of Pediatrics (J.T.), Kameda Medical Center, Kamogawa; National Epilepsy Center (Y.T.), Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka; Department of Pediatrics (H. Tamai), Osaka Medical College, Takatsuki; Department of Pediatrics (J.N.), Nagoya University Graduate School of Medicine, Nagoya; Department of Neurology (S. Hamano), Saitama Children's Medical Center, Saitama; Department of Neurology (S. Hirabayashi), Nagano Children's Hospital, Azumino; Division of Child Neurology (Y.M.), Faculty of Medicine, Tottori University, Yonago; Department of Developmental Medical Sciences (M.M.), Graduate School of Medicine, The University of Tokyo; Department of Pediatrics (K.M.), Hokkaido Medical Center for Child Health and Rehabilitation, Sapporo; Department of Pediatric Neurology (H.Y.), Nagaoka Habilitation and Medical Center for Severely Handicapped Children, Nagaoka; and Department of Neurology (S. Kusunoki), Kinki University Faculty of Medicine, Osaka-Sayama, Japan. htorys@pediatr.med.kyushu-u.ac.jp.

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.

MeSH terms

Child
Child, Preschool
Demyelinating Diseases / classification
Demyelinating Diseases / diagnostic imaging
Demyelinating Diseases / drug therapy
Demyelinating Diseases / epidemiology*
Female
Follow-Up Studies
Humans
Immunosuppressive Agents / therapeutic use
Japan / epidemiology
Magnetic Resonance Imaging
Male
Methylprednisolone / therapeutic use
Retrospective Studies
Steroids / therapeutic use
Surveys and Questionnaires

Substances

Immunosuppressive Agents
Steroids
Methylprednisolone