67. Paget’s Disease of the Bone
Definition
Paget’s disease of the bone is a relatively common disease of middle-aged and elderly people in which there is excessive and abnormal bone remodeling. The condition results in extensively vascularized, weak, enlarged, deformed bones that are increasingly prone to subsequent complications.
Incidence
Determining the frequency of occurrence of Paget’s disease is inherently imprecise because many affected people are asymptomatic. The disease is estimated to affect approximately 3% of the population 40 years of age or older. Males are more commonly affected at a ratio of 1.8:1.
Etiology
The exact etiology of Paget’s disease remains unknown. Genetic and nongenetic contributions have been implicated, including factors related to viral infection, elevated parathyroid hormones, autoimmune deficiencies, connective tissue, and vascular disorders.
Signs and Symptoms
• Acetabulum protrusion
• Ataxia
• Back and neck pain
• Bone angulation and/or deformity
• Bone pain
• Bowel and bladder incontinence
• Cranial nerve palsies
• Dementia
• Dizziness
• Enlargement of skull and lower limbs
• Facial disfigurement
• Femur and long bone bowing
• Gait difficulties
• Hearing loss
• Limb paresis
• Malocclusion
• Muscle weakness
• Nausea
• Nonspecific headache
• Paresthesias
• Pathologic fractures
• Platybasia (also known as basilar impression: a developmental deformity of the occipital bone and upper end of the cervical spine in which the cervical spine appears to have pushed the floor of the occipital bone upward)
• Syncope
• Tibial or skull bruits
