Skull Monostotic Paget's Disease of Bone - Case Report and Review of the Literature

Abstract

Paget's disease of bone is a metabolic skeleton disorder with different epidemiological patterns: increased incidence up to 3% of the Northern Caucasian population over the age of 55 or a very rare occurrence as in Asia. We present a case of a 69 years old female patient with diffuse bone pain, suspected of osteoporosis after a DXA investigation of bones. She has developed progressive skull deformation over the last 17 years and bilateral sensoneural deafness. On admission in our hospital, she had alkaline phosphatase serum levels five times higher than the upper normal value and low values of 25 hydroxy vitamin D. Parathormone and calcium levels were in normal range. Radiologic findings confirmed the monostotic type of Paget's disease. Other biomarkers of bone remodeling: serum osteocalcin for bone formation and serum beta Cross Laps for bone resorption, showed moderately increased levels. High dose therapy with alendronate and alphacalcidol were given with the normalization of bone markers after 9 months of sustained treatment. The vitamin D deficiency of this patient is an independent, associated disease, producing a decrease of bone mineral density as shown by DXA measurement. The case presentation and the review of the medical literature on this subject are intended to remind clinicians of the importance of appropriate diagnosis and therapy in this disease, as well as the importance of establishing the prevalence of Paget's disease of bone in Romania.