title: Neuroepidemiology of Porencephaly, Schizencephaly, and Hydranencephaly in Miyagi Prefecture, Japan.
abstract: BACKGROUND
  No population-based surveys of porencephaly, schizencephaly, and hydranencephaly have been conducted in Japan or other Asian countries. We performed a neuroepidemiologic analysis to elucidate the incidence of porencephaly, schizencephaly, and hydranencephaly in Miyagi prefecture, Japan, during 2007-2011.
  
  
  METHODS
  We sent inquiry forms in February 2012 to three neonatal intensive care units, 25 divisions of orthopedic surgery in municipal hospitals, 33 divisions of pediatrics including one university hospital, municipal hospitals, pediatric practitioners, and institutions for physically handicapped children located in Miyagi prefecture. These covered all clinics related to pediatric neurology and orthopedic surgery in Miyagi prefecture. In the inquiry, diagnostic criteria for porencephaly, schizencephaly, and hydranencephaly were described and representative images of magnetic resonance imaging were shown. We obtained an 82% (27 of 33) response rate from the divisions of pediatrics, a 100% (3 of 3) response rate from the neonatal intensive care units, and a 68% (17 of 25) response rate from orthopedic surgery clinics. The magnetic resonance imaging scans of each patient were retrieved and inspected.
  
  
  RESULTS
  Five, one, and two individuals developed porencephaly, schizencephaly, and hydranencephaly, respectively. The estimated incidence rates of porencephaly, schizencephaly, and hydranencephaly were 5.2 (95% confidence interval [CI], 0.6-9.8), 1.0 (95% CI, 0.0-3.1), and 2.1 (95% CI, 0.0-5.0) per 100,000 live births, respectively.
  
  
  CONCLUSIONS
  The prevalence rates of porencephaly, schizencephaly, and hydranencephaly at birth reported herein are compatible with results reported previously in the United States and European countries. The overall prevalence rate of these three diseases was 8.3 (95% CI, 2.6-14.1) per 100,000 live births.
authors.affiliation: Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan.
authors.forename: Naomi
authors.initials: N
authors.lastname: Hino-Fukuyo
authors.affiliation: Department of Neurology, Miyagi Children's Hospital, Sendai, Japan.
authors.forename: Noriko
authors.initials: N
authors.lastname: Togashi
authors.affiliation: Neonatal Intensive Care Unit in Perinatal Center, Japanese Red Cross Sendai Hospital, Sendai, Japan.
authors.forename: Ritsuko
authors.initials: R
authors.lastname: Takahashi
authors.affiliation: Neonatal Intensive Care Unit, Miyagi Children's Hospital, Sendai, Japan.
authors.forename: Junko
authors.initials: J
authors.lastname: Saito
authors.affiliation: Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, Sendai, Japan.
authors.forename: Takehiko
authors.initials: T
authors.lastname: Inui
authors.affiliation: Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, Sendai, Japan.
authors.forename: Wakaba
authors.initials: W
authors.lastname: Endo
authors.affiliation: Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, Sendai, Japan.
authors.forename: Ryo
authors.initials: R
authors.lastname: Sato
authors.affiliation: Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, Sendai, Japan.
authors.forename: Yukimune
authors.initials: Y
authors.lastname: Okubo
authors.affiliation: Department of Human Genetics, Yokohama City University Graduate School of Medicine, Yokohama, Japan.
authors.forename: Hirotomo
authors.initials: H
authors.lastname: Saitsu
authors.affiliation: Department of Pediatrics, Tohoku University School of Medicine, Sendai, Japan; Department of Pediatric Neurology, Takuto Rehabilitation Center for Children, Sendai, Japan. Electronic address: khaginoya@kha.biglobe.ne.jp.
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Haginoya
mesh_terms: D000328:Adult; D001921:Brain / Q000473:pathology; D005260:Female; D006801:Humans; D006832:Hydranencephaly / Q000453:epidemiology* / Q000473:pathology; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008279:Magnetic Resonance Imaging; D008297:Male; D065708:Porencephaly / Q000453:epidemiology* / Q000473:pathology; D015995:Prevalence; D065707:Schizencephaly / Q000453:epidemiology* / Q000473:pathology; D055815:Young Adult
keywords: epidemiology; hydranencephaly; porencephaly; prevalence rate; schizencephaly