title: Rigid Spine Syndrome among Children in Oman.
abstract: OBJECTIVE
  Rigidity of the spine is common in adults but is rarely observed in children. The aim of this study was to report on rigid spine syndrome (RSS) among children in Oman.
  
  
  METHODS
  Data on children diagnosed with RSS were collected consecutively at presentation between 1996 and 2014 at the Sultan Qaboos University Hospital (SQUH) in Muscat, Oman. A diagnosis of RSS was based on the patient's history, clinical examination, biochemical investigations, electrophysiological findings, neuro-imaging and muscle biopsy. Atrophy of the paraspinal muscles, particularly the erector spinae, was the diagnostic feature; this was noted using magnetic resonance imaging of the spine. Children with disease onset in the paraspinal muscles were labelled as having primary RSS or rigid spinal muscular dystrophy. Secondary RSS was classified as RSS due to the late involvement of other muscle diseases.
  
  
  RESULTS
  Over the 18-year period, 12 children were included in the study, with a male-to-female ratio of 9:3. A total of 10 children were found to have primary RSS or rigid spinal muscular dystrophy syndrome while two had secondary RSS. Onset of the disease ranged from birth to 18 months of age. A family history was noted, with two siblings from one family and three siblings from another (n = 5). On examination, children with primary RSS had typical features of severe spine rigidity at onset, with the rest of the neurological examination being normal.
  
  
  CONCLUSIONS
  RSS is a rare disease with only 12 reported cases found at SQUH during the study period. Cases of primary RSS should be differentiated from the secondary type.
authors.affiliation: Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Roshan
authors.initials: R
authors.lastname: Koul
authors.affiliation: Radiology & Molecular Imaging, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Dilip
authors.initials: D
authors.lastname: Sankhla
authors.affiliation: Pathology, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Suad
authors.initials: S
authors.lastname: Al-Jahdhami
authors.affiliation: Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Renjith
authors.initials: R
authors.lastname: Mani
authors.affiliation: Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Rana A
authors.initials: RA
authors.lastname: Rahim
authors.affiliation: Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Saif
authors.initials: S
authors.lastname: Al-Yaarubi
authors.affiliation: Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Hussein
authors.initials: H
authors.lastname: Al-Kindy
authors.affiliation: Genetics, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Khalid
authors.initials: K
authors.lastname: Al-Thihli
authors.affiliation: Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman.
authors.forename: Amna
authors.initials: A
authors.lastname: Al-Futaisi
keywords: Children; Magnetic Resonance Imaging; Oman; Rigid Spine Muscular Dystrophy; Rigid Spine Syndrome; Selenoprotein