Paget's disease is a common osteodystrophy characterized by abnormal bone metabolism where focal areas of bone are resorbed, then replaced in excess. It is estimated to affect 7% of males and 4% of females over the age of 55 (119). Although 85% of cases of Paget's disease are asymptomatic, typical presentations of pain, arthritis, fractures, and high-output cardiac failure are characteristic (120). Skull enlargement with bony impingement of neural foramina can lead to neuropathy, but this appears to be an uncommon occurrence. Of the cranial nerves involved, the auditory and olfactory nerves are the most commonly affected. When involvement of the facial nerve occurs, it usually presents as hemifacial spasm occurring in 7% of patients with Paget's disease. Facial paresis or paralysis has also been found in roughly 2% of Paget's patients. Diagnosis is confirmed by characteristic radiographic findings with demineralized areas giving a washed out or blurry appearance. Laboratory abnormalities showing an elevated alkaline phosphatase level are consistent with increased bone metabolism. Treatment is with antiosteoclastic agents including calcitonin, plicamycin (mithramycin), and etidronate (etidronic acid). The prognosis is considered good with the exception of sarcomatous transformation, which occurs in roughly 10% of the cases (120).
Paget' s disease is discussed in detail in Chapter 21.
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