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A case is described of severe untreated polyostotic Paget’s disease with severe skull involvement causing hydrocephalus, and its response to treatment with intravenous bisphosphonates is presented.
An 86 year old woman presented with a femoral neck fracture as result of a fall. Her daughter gave a 20 year history of progressive deafness and a 6 month history of confusion, ataxia, and urine incontinence in the setting of untreated Paget’s disease. Examination disclosed a profoundly deaf woman with whom communication was only possible by writing, typical bony changes of the skull and both tibiae, but no papilloedema or angioid streaks on fundoscopy. A modified mini-mental state examination was 21/30. Serum alkaline phosphatase (AP) was 1477 U/l (normal 30–120). Plain radiography showed severe pagetic changes affecting both hemipelves, femora (including the site of fracture), tibiae, and the skull. Computed tomography scanning of the head showed moderate enlargement of both lateral ventricles and the third ventricle, with the fourth ventricle remaining decompressed, consistent with obstructive hydrocephalus (fig 1).
The patient declined orthopaedic intervention, magnetic resonance imaging of the brain, or neurosurgical consultation. In view of the neurological involvement her Paget’s disease was treated aggressively with three infusions of 90 mg pamidronate given weekly, and her serum AP fell to 650 U/l 4 weeks after presentation. When the effect of this seemed to be plateauing she was then given 4 mg of zoledronate intravenously (fig 2). Her serum AP fell further to 290 U/l over the next 2 months. Her daughter felt there was improvement in her hearing, but this was not documented objectively with audiometry. Unfortunately, the patient developed sudden respiratory problems and died 4 months after presentation.
Reports of about 20 cases of hydrocephalus complicating Paget’s disease have been published. Affected patients presented with ataxia, confusion, urine incontinence, and parkinsonian syndromes, and had relief of their neurological symptoms with ventricular shunting. The use of zoledronate in this case was prompted by the article by Chung and Keen, who reported an excellent response of polyostotic Paget’s disease to this drug after variable responses to various bisphosphonates and subsequent resistance to intravenous pamidronate.1 Randomised trials are underway comparing response rates between zoledronate and other bisphosphonates.
No competing interest.