Rigid Spine Syndrome: A Clinical Symptom Encompassing Various Aethiologies

The rigid spine syndrome (R.S.S.) is a unusual muscular disorder first defined in 1965 by Dubowitz [1,2] and later described by several others. It is a rare manifestation which typical features include a reduction of the spine flexion and fibrous degeneration of axial muscles. The main clinical features in this condition is a pronounced limitation of flexion of the cervical, thoracic and lumbar spine with the development of scoliosis eventually associated with contractures of others joints. Raised levels of C.K are frequently observed in R.S.S. associated with muscle disorders. Calcium is thought to have a pivotal role in controlling membrane permeability (ref.). An increase of CK, most probably due to leakage of intracellular enzyme has been observed in miscellaneous conditions presenting hypocalcemia as well (ref). Moreover a deficiency of phosphorylase a, as a cause of muscle weakness, has been well documented in this condition [1]. We further emphasize the different causes of RSS that may include myopathies or endocrine disturbances. Moreover, the unique case of pseudohypoparathyroidism associated with R.S.S. further clarify the different mechanisms which may lead to increased C.K level. Patients and Methods Patient 1