Some unusual features of Paget's disease of bone

The usual features of Paget's disease of bone, e.g. large head, deafness due to involvement of the 8th cranial nerve and bowing of the legs are all well described in text books of medicine. However, some features such as platybasia with neurological deficit, involvement of the 2nd, 5th and 7th cranial nerves, hydrocephalus, dementia, dysphasia, corrugation of the skull, bone pain (with or without associated osteo-arthrosis of hips and knees), heart failure, triradiate deformity of the pelvis have either had an inadequate or no description in books. These features are unusual, but it is important to recognise them, as the advances made since the introduction of the very promising new drugs in recent years have not only provided increasing insight into the disease, but also have improved management of patients with Paget's disease of bone.