Surgical management of hydrocephalic dementia in Paget's disease of bone: the 6-year outcome of ventriculo-peritoneal shunting

Paget's disease of bone is a chronic progressive skeletal disorder usually occurring in the long bones and skull of older adults and elderly persons. In the skull, softening of the skull base may lead to basilar impression and consequently obstruction of the cerebrospinal fluid through the basilar cisterns, resulting ventricular enlargement in association with gait difficulties, incontinence and dementia: a syndrome resembling normal pressure hydrocephalus. The optimal management of hydrocephalus associated with Paget's disease of the skull is not well documented and is still debated. We report a patient with hydrocephalic dementia linked to Paget's disease of the skull who showed marked sustained improvement in her neurological condition after ventriculo-peritoneal shunt insertion. We have now followed this patient for 6 years. Our experience supports the view that ventricular shunting is the procedure of choice for treating hydrocephalus linked to the Paget's disease of bone and is best carried out in the early stages of the illness.