Headache in patients with Paget's Disease of Bones

Paget's disease of bone (PDB) is a focal remodeling disorder of unknown origin. Head bones are frequently involved and patients may suffer from deformities and specific symptoms, mainly headache, or neurological deficits, such as deafness. Several pathophysiological entities can give rise to headache in PDB patients. Clinical, laboratory, radiological and scintigraphic findings contribute to distinguish headache related to PDB from other causes of headache. Up to the present, treatment with intravenous zolendronic acid has been reported to improve symptoms of PDB and induce long-standing biochemical and scintigraphic remission. In this review, the main strands of literature about epidemiology, pathophysiology, clinical characteristics and treatment of PDB-related headache are drawn together.