Rigid Spine Syndrome among Children in Oman
Journal Title: Sultan Qaboos University Medical Journal - Year 2015, Vol 15, Issue 3
Abstract
Objectives: Rigidity of the spine is common in adults but is rarely observed in children. Te 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 fndings, 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 classifed 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 maleto-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. Conclusion: 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 and Affiliations
Roshan Koul| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman, Dilip Sankhla| Departments of Radiology & Molecular Imaging, Sultan Qaboos University Hospital, Muscat, Oman, Suad Al-Jahdhami| Departments of Pathology, Sultan Qaboos University Hospital, Muscat, Oman, Renjith Mani| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman, Rana A. Rahim| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman, Saif Al-Yaarubi| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman, Hussein Al-Kindy| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman, Khalid Al-Tihli| Departments of Genetics, Sultan Qaboos University Hospital, Muscat, Oman, Amna Al-Futaisi| Departments of Child Health, Sultan Qaboos University Hospital, Muscat, Oman
Keywords
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