Osteosclerosis Systemic Hereditary With Myopathy

Osteosclerosis (from ancient Greek ὀστέον - bone + σκλήρωμα - density; sclero - hardened, dense, hard) is a pathological process in which normal bone tissue is gradually replaced by coarse fibrous tissue. As a rule, osteosclerotic processes are accompanied by a pathological fracture or compression of the nerve trunks (spinal cord compression syndrome), as well as paralysis and paresis of varying severity, due to which the patient experiences limitations in motor activity (impaired walking, weakness of the arm muscles, paralysis).

**Systemic hereditary osteosclerosis with myopathy** is a medical condition that manifests itself in hereditary pathology of bone tissue and can cause disturbances in muscle function. The human skeleton consists of several parts, including vertebrae and bones formed by the fusion of the ends of long bones. With osteosclerosis, these bones become denser, resulting in fewer bones and difficulty moving them. In this article we will talk about the causes and consequences of this disease.

The causes of the disease Osteoskerosis are systemic and hereditary, with myopathy being different depending on the form: dystrophic (progressive), remodeling and mixed. The progressive form is accompanied by a painful manifestation with pain during movement, swelling of the bone area and bleeding of the soft tissues of the bone walls (due to defective tissue areas formed when the cells are unstable). Most often, the age of onset of progressive primary systemic osteosclerosis is 40-60 years, and less often - 20-30 years. The secondary form occurs with frequent microtrauma of bones, deformation of articular elements and imbalances. The deformed shape occurs only due to injury or dislocations. The remodeling appearance occurs against the background of joint diseases due to reduced calcium in the skeletal system, with the appearance of trophic disorders and neuromuscular dystrophy of the musculoskeletal fibers.