Paget’s disease of bone

Published on 02/03/2015 by admin

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Last modified 02/03/2015

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CHAPTER 12

Paget’s disease of bone

1. What is Paget’s disease of bone?

2. Discuss how Paget’s disease is diagnosed.

3. What are the clinical manifestations of Paget’s disease?

Most patients (70%-80%) with Paget’s disease are asymptomatic. This disorder is often suspected from radiographs performed for other reasons or from an unexpected elevation of the serum alkaline phosphatase concentration. The most common symptom of Paget’s disease is bone or joint pain. The pain is often described as dull and aching. Other manifestations of Paget’s disease, such as headache, bone deformity, skull enlargement, fracture, change in skin temperature over an involved bone, high-output congestive heart failure, and entrapment neuropathies that may cause hearing loss or other neurologic deficits, are much less common (Box 12-1). Neurologic deficits may arise from bony impingement on the brain or cranial nerves exiting from the skull, spinal nerve entrapment, or direct pressure of pagetic vertebrae on the spinal cord. Bone deformity is usually seen in patients with long-standing Paget’s disease. Most commonly, the skull, clavicles, and long bones are deformed and exhibit both an increase in size and an abnormal contour. There is speculation that Ludwig van Beethoven’s hearing loss, headaches, and progressive hyperostosis frontalis were the results of advanced Paget’s disease of bone.

4. What disorders are associated with Paget’s disease of bone?

5. What are the three phases of Paget’s disease of bone?

6. Describe the radiographic findings associated with the osteolytic phase of Paget’s disease.

7. What are the radiographic findings most commonly found in the osteoblastic phase of the disease?

8. What is the best radiographic evaluation to determine the extent of Paget’s disease?

The metabolic activity of osteoblastic pagetic bone lesions is most easily assessed by radionuclide scanning because these lesions avidly take up the technetium-labeled bisphosphonate. Although bone scans are diagnostically less specific than radiographic studies, they identify approximately 15% to 30% of pagetic lesions not visualized on radiographs. Conversely, when radiographs demonstrate pagetic involvement but the serum alkaline phosphatase concentration is normal and the bone scan reveals little isotope uptake at those sites, the diagnosis of relatively inactive or “burned out” Paget’s disease is most likely. Predominantly lytic bone lesions (such as osteoporosis circumscripta) may not be detected on bone scan. Computed tomography (CT) and magnetic resonance imaging (MRI) add little to the workup of patients with uncomplicated Paget’s disease.

9. Which bones are involved in Paget’s disease?

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