THIS rare and interesting condition, the exact nature of which is still unknown, was first described by Sir James Paget in 1876. It begins usually after the age of 40. The youngest case that the authors have been able to find reported is that of a patient in Dr. LeWald's series who was 31 years of age when the disease was first noted. The essential feature of the disease is an associated softening and overgrowth of bone, with persistent bone pains forming the chief subjective symptom in the earlier stages. As to clinical features, the bones principally affected are the skull, the vertebrae, and the bones of the leg, but almost any bone may be involved. As a rule the disease manifests itself in a number of bones, but in rare cases it may be confined to one, the diagnosis being then a matter of great difficulty. The bones of the lower limbs are usually the first to be affected, but in a number of cases the disease has been known to commence in the skull. The softened bones of the leg, having to bear the weight of the body, become bent; the femur bends outward, the tibia forward. The patient, therefore, becomes bow-legged. In addition to the bowing there may be a marked twisting, so that, as DaCosta remarks, “The femur comes to look as though it had been grasped by the hands of a giant, bent into a bow, then twisted.” Other factors, in addition to that of body weight, must be responsible for the deformity, for the bones of the arm also become bent, although to a lesser degree. In this case the curve is backward. Associated with the bending, or even before it occurs, there is a characteristic thickening of the bone. The bone pains, which are so constant a feature of the disease, are felt particularly in the legs, seldom in the arms or head. They may be continuous or periodic and may appear many months before any gross lesion can be detected. Enlargement of the skull is almost always present at some stage of the disease. It may be the first sign to appear, and may attract attention through the patient noticing that he has to buy hats of ever-increasing size. The deformity in typical cases is so characteristic that it can be recognized at a glance. The head becomes a triangle, with the base above, the face usually escaping almost completely. The enlargement is due to an enormous deposit of bone on the outside of the cranium; there is no endocranial thickening. To those cases in which the facial bones and the bones of the skull are thickened whilst the other bones escape, the name “leontiasis ossea” is given. The vertebral column is almost always involved, and marked kyphosis develops in the dorsal and lower cervical regions. As a result of the kyphosis, the patient shrinks in height, a condition which is aggravated by the bowing of the legs. There are cases in which a man has lost as much as a foot in stature. The clavicles may be affected, and, in exceptional cases, the bones of the hands and feet.
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