Rigid Spine Syndrome among XXXX in Oman.

@entity1 @entity2003 @entity121
OBJECTIVES: Rigidity of the spine is common in adults but is rarely observed in @entity1 . The aim of this study was to report on @entity2003 ( @entity2003 ) among @entity1 in Oman. METHODS: Data on @entity1 diagnosed with @entity2003 were collected consecutively at presentation between 1996 and 2014 at the Sultan Qaboos University Hospital (SQUH) in Muscat, Oman. A diagnosis of @entity2003 was based on the @entity1 '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. @entity1 with disease onset in the paraspinal muscles were labelled as having primary @entity2003 or @entity2003 . Secondary @entity2003 was classified as @entity2003 due to the late involvement of other @entity121 . RESULTS: Over the 18-year period, 12 @entity1 were included in the study, with a male-to-female ratio of 9:3. A total of 10 @entity1 were found to have primary @entity2003 or @entity2003 while two had secondary @entity2003 . 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, @entity1 with primary @entity2003 had typical features of severe @entity2003 at onset, with the rest of the neurological examination being normal. CONCLUSION: @entity2003 is a rare disease with only 12 reported cases found at SQUH during the study period. Cases of primary @entity2003 should be differentiated from the secondary type.
