Paget's Disease of the skull

Paget's Disease

• Paget's disease is common -- approx. 3% of people over 40 yrs.
  • May rise to 10-11% of pts. over age 80.
  • Rare among Chinese.
• Clinical findings vary with severity - and location - of disease.
• Occ. associated with high output CHF.
• Viral etiology questioned.
• Has osteolytic, mixed, and sclerotic phases.
• In the skull:
  • Lytic phase is termed "osteoporosis circumscripta."
  • Often affects frontal and/or occipital regions.
  • Can progress to involve entire skull.
  • Basilar invagination secondary to bone softening.
  • Narrowing of auditory canal can lead to deafness.
  • Facial bone involvement less than in fibrous dysplasia.
• Elsewhere in skeletal system:
  • Bowing long bones.
  • Transverse Fxs.
  • Cortical thickening.
  • Coarsening of trabeculae.
• Activity of disease demonstrated by intensity of uptake on nuclear medicine bone scan.
• Small percentage of lesions can undergo sarcomatous degeneration.
• Rx:
  • Calcitonin -- inhibits bone resorption.
  • Diphosphonates -- inhibits bone resorption and mineralization by binding to hydroxyapatite crystals.
  • Mithramycin -- cytotoxic agent.
• DDx:
  • osteoblastic metastases.
  • osteitis condensans ilii in pelvis.
  • hyperostosis frontalis interna in skull.
  • fibrous dysplasia.
  • lymphoma.

You may wish to review the radiographic appearance of a NORMAL skull.

Ref:Resnick, D., Bone and Joint Imaging, W.B. Saunders, Philadelphia, PA., 1989, pp. 603-611.

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