title: Nationwide survey on the epidemiology of syringomyelia in Japan.
abstract: BACKGROUND
  Syringomyelia is a rare disease characterized by abnormal fluid-filled cavities within the spinal cord, and is associated with Chiari malformations, arachnoiditis, or spinal cord tumors. The widespread availability of magnetic resonance imaging (MRI) in Japan has allowed for easy identification of syrinxes. The aim of this study was to survey the clinicoepidemiological characteristics of syringomyelia in Japan.
  
  
  METHODS
  A 2-stage postal survey was conducted in late 2009. The first survey aimed to estimate the number of patients with syringomyelia, and the second survey aimed to elucidate clinicoepidemiological characteristics. Diagnosis of syringomyelia was based on the findings of MRI or computed tomographic myelography.
  
  
  RESULTS
  In the first survey, we received 2133 responses from 2937 randomly selected departments and collected data of 1215 syringomyelia patients (543 men and 672 women). The total response rate for the first survey was 73%. The estimated prevalence of ambulatory syringomyelia patients in Japan was 1.94 per 100000. In the second survey, the proportion of asymptomatic syringomyelia patients was 22.7%. Chiari type I malformations and idiopathic syringomyelia were the first and second most common etiologies.
  
  
  CONCLUSIONS
  Our nationwide survey indicated that widespread MRI availability has contributed to the diagnosis of both asymptomatic and idiopathic cases.
authors.affiliation: Department of Neurology, Hokkaido University Graduate School of Medicine, Hokkaido, Japan. sakusima@med.hokudai.ac.jp
authors.forename: Ken
authors.initials: K
authors.lastname: Sakushima
authors.forename: Satoshi
authors.initials: S
authors.lastname: Tsuboi
authors.forename: Ichiro
authors.initials: I
authors.lastname: Yabe
authors.forename: Kazutoshi
authors.initials: K
authors.lastname: Hida
authors.forename: Satoshi
authors.initials: S
authors.lastname: Terae
authors.forename: Ritei
authors.initials: R
authors.lastname: Uehara
authors.forename: Imaharu
authors.initials: I
authors.lastname: Nakano
authors.forename: Hidenao
authors.initials: H
authors.lastname: Sasaki
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D002675:Child, Preschool; D005260:Female; D006306:Health Surveys / Q000639:trends; D006801:Humans; D007223:Infant; D007564:Japan / Q000453:epidemiology; D008279:Magnetic Resonance Imaging / Q000639:trends; D008297:Male; D008875:Middle Aged; D013595:Syringomyelia / Q000175:diagnosis* / Q000453:epidemiology*; D055815:Young Adult