Chakravorty N K
Gerontology. 1978;24(6):459-72. doi: 10.1159/000212286.
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.
骨Paget病的常见特征,如头颅增大、因第8对脑神经受累导致的耳聋以及腿部弯曲,在医学教科书中均有详尽描述。然而,一些特征,如伴有神经功能缺损的扁平颅底、第2、5和7对脑神经受累、脑积水、痴呆、言语困难、颅骨皱纹、骨痛(伴或不伴有髋部和膝部相关骨关节炎)、心力衰竭、骨盆的三叶形畸形,在书中要么描述不足,要么未作描述。这些特征并不常见,但认识到它们很重要,因为近年来引入非常有前景的新药后取得的进展,不仅使人们对该疾病有了越来越深入的了解,也改善了骨Paget病患者的管理。