Rigid Spine Syndrome in Children

Context: Rigid spine syndrome is rare in children. It is commonly observed in the end stage of the muscular dystrophies. Another entity called primary rigid spine or spinal muscular dystrophy syndrome, rigidity of spine is noted at the beginning of a muscle disease. The two conditions should be differentiated. Evidence Acquisition: The first child with primary rigid spine syndrome was observed in 1996. Twelve children were observed over the last eighteen years and were followed up regularly in the hospital under study. The children were diagnosed with rigid spine syndrome if there was restriction of the neck movements, particularly on the bending of the spine. The diagnosis was based on clinical features, biochemical tests, electrophysiological findings, imaging and muscle biopsy. Genetic tests were not available in the past, but were done recently in three children. Results: Twelve children (nine malesandthree females) were observed. Tenchildrenhadprimary rigid spineandtwohadsecondary rigid spine syndrome. Conclusions: The current study described twelve children with rigid spine syndrome from Oman; ten with primary rigid spine (rigid spinal muscular dystrophy) and two with secondary rigid spine syndrome. The condition is rare in children. Early neck stiffness is the key to diagnosis.