Rigid spine syndrome - Two case-reports

Rigid spine syndrome is characterized by massive spinal rigidity, usually m ost marked in the cervical region. Stiffness of the peripheral joints is so metimes present. We report two cases. Patient 1 was a la-year-old boy diagn osed at three years of age with Duchenne's muscular dystrophy because of de layed onset of walking. Contracture of the Achilles tendons, flexion contra cture of the elbows, and loss of motion of the cervical spine were the main findings during the current evaluation. Radiographs of the affected joints were normal. An electrocardiogram showed an incomplete left bundle branch block. Muscle enzyme activities were moderately elevated. A myopathic patte rn was seen on the electromyogram. A muscle biopsy showed muscle fiber atro phy with peri- and endomysial fibrosis, Patient 2 was a 39-year-old man wit h a five-year history of isolated rigidity of the cervical spine thought to be due to a spondylarthropathy. Extension was the only movement possible a t the cervical spine. The peripheral joints showed no motion range limitati on. Findings were normal from radiographs of the spine and sacroiliac joint s, an erythrocyte sedimentation rate determination. an electromyogram, and muscle enzyme activity assays. A muscle biopsy showed muscle fiber atrophy with peri- and endomysial fibrosis. Discussion. Rigid spine syndrome is rar e in rheumatological practice and can simulate a number of other muscle and joint diseases. Peri- and endomysial fibrosis may be strongly suggestive. although nonpathognomonic. Involvement of the heart governs the prognosis.