Paget's disease of bone - Signs and symptoms

Paget's disease is rare in patients under 25 years and increases in frequency with increasing age once. It is believed that Paget's disease develops in people after the fifth decade of life and is most often diagnosed in people 60 years. Paget's disease incidence in people over 80 years is 10%. There is also a juvenile form of Paget's disease, different from the adult.Paget's disease is a localized disease that can be monostatica-affects only one bone. This represents one third of all cases of the disease. It can be polistatica-affect two or more bones. Paget's disease does not extend from one bone to another, and we are rare localizations after initial diagnosis, in contrast lesions continue to progress if not treated. Most people are asymptomatic, the disease is detected incidentally them to abnormally high levels of alkaline phosphatase or characteristic radiographic abnormalities.When symptoms are pain is the most common accusation. Others present with symptoms that include pathological fracture, congestive heart failure, gained deafness, and weakness disestezii nerve root compression.
Physical Exam
Physical examination may be normal in patients with Paget's disease, however, signs of damage that may be present include heat skeletal growth, bone deformities, and secondary osteoarthritis hiperexpansiunea skull. Hearing loss may be apparent during a physical exam. Because these patients and should be examined for the presence of gouty arthritis tofilor. The spine is the second in locating the disease. Develop stenosis or lumbar kyphosis. If the thoracic spine disease a patient has spinal cord compression with loss of nerve function.
Paget's disease of the skull may be asymptomatic. A third of patients with head size or growth experimeteaza without deformation: boselare front, big jaw, with headache, deafness and nerve damage with loss of sensation often. The problem is most common neurological deafness VIII cranial nerve compression and dysfunction of the cochlear. Tinnitus and deafness are the most common symptoms of damage to the skull. Deafness may be driving by affecting osicioarelor middle ear and auditory nerve compression senzoneurala cochlear damage or mixed. Vertigo and tinnitus occurring with a frequency of 25% in these patients.If the facial bones are affected patient may have facial deformities airway collapse. Paget's bone fracture is a severe complication and can occasionally be traumatic or spontaneous. Because it is a long bone, the femur is the most affected. Paget bone fractures heal normally.
Back and neck pain are common accusations. Weakening of the bones of the skull leading to platibazie, clogging the backbone of the skull base.Progressive pain, limb paresthesia, weight imbalance bladder or bowel incontinence can be caused by compression of the spinal cord or spinal nerve by platibazie or vertebral fractures. Nausea, dizziness, syncope, ataxia, incontinence and dementia may be seen in hidrocefalus, basilar invagination and syndromes or brain cerebelare comprensive.People with Paget disease are asymptomatic, though a minority of affected persons accused various symptoms, including bone pain, the most common symptom, secondary osteoarthritis when Paget's disease affects an area adjacent joints, bone deformation, local excessive heat through compression hipervascularizare and neurological complications nerve tissue. Paget's disease can be monostotic, but is more frequently multifocal, with predilection for the axial skeleton: spine, pelvis, femur, sacrum and skull. Any bone can be affected, however. After onset Paget's disease of the bone does not extend to the bone, but can become progressive and worsen pre-existing location.
Evolution and complications
Paget disease morbidity may be extensive and result in bone pain, osteoarthritis and fractures. Increasing mortality associated with Paget's disease is due to disease complications, especially those associated with fracture and sarcoma. Most patients with Paget's disease who develop sarcoma die within three years after diagnosis. Cause excessive bleeding after Hipervascularizarea bone fractures or surgery and is associated with potentially severe consequences.
Paget's bone sarcoma
Development of a secondary sarcoma in Paget's bone is the most lethal complication occurs in 1% of patients with Paget's disease.These sarcomas are aggressive and multicentric. 5-year survival rate is zero. Histologically most shows osteosarcoma with malignant fibrous histiocitom remaining tumors.Chemotherapeutic regimens are relatively ineffective and very toxic. Surgery is usually palliative, however appendicular skeleton diagnosed before metastasis, surgical treatment improves the chance of survival. Paget's sarcoma presenting with pain or pathologic fractures. Multiple giant cell tumors are rarely observed in association with Paget's disease. Common site is the skull and facial bones. These lesions respond well to steroid therapy.