Bone mineral content in hereditary polycystic osteodysplasia associated with progressive dementia

Measurements of the bone mineral content were made in five patients with a disease characterized by progressive dementia and lipomembranous polycystic osteodysplasia. Decreased bone mineral density (g/cm3) was observed not only in the region of cysts in the distal radius but also in the diaphyses of radius and ulna. The 85Sr vertebral uptake was low in the youngest patient, normal in two and raised in another two. The observations were compatible with the hypothesis of a general metabolic disorder of the bone.