Hydrocephalus and headaches in Paget's disease of the skull: complete relief by ventriculo-atrial shunt.

A 76 year old patient with a long history of headaches was found to have Paget's disease and communicating hydrocephalus. There were (otherwise) no neurological or musculo-skeletal manifestations of Paget's disease, but moderate impairment of intellectual function was present. Treatment with disphosphonates did not bring any significant improvement, but three days following a ventriculo-atrial shunting procedure, the patient became headache-free for the first time in several years. In the literature, patients with hydrocephalus have been shown to respond quite unevenly to atrio-ventricular shunting, but in most instances the descriptions concerned advanced cases with well-established symptoms of dementia, ataxia and incontinence. Our case is reported to stress the importance of early diagnosis and management of hydrocephalus in Paget's disease for the prevention of widespread neurological dysfunction.