Hydrocephalus-dementia-complex in Paget's disease

The clinical triad of gait disturbances, memory impairment and urinary incontinence is associated with a communicating hydrocephalus in the 'normal pressure hydrocephalus'-syndrome. The authors present a case of H.D.C. (hydrocephalus-dementia-complex) in Paget's disease with an identical syndrome, but with obstructive hydrocephalus, causing a triventricular dilatation. Today, each case of mental deterioration in Paget's disease, should be immediately observed and neurosurgical intervention kept in view. An X-ray of the skull, a CT-scan of the brain and a cisternography are performed as routine procedure. If there are indications of involvement of the basis of the skull, hydrocephalus and/or disturbed pattern of the tracer-migration around the convexities, associated with a certain degree of dementia, impaired gait or urinary incontinence, a ventriculo-subcutaneous drain should be inserted. If clinical improvement follows, a ventriculatrial shunt is indicated. The post-operative clinical outcome seems to be dependent on some clinical and technical factors. The most eventful outcome is observed in cases where the clinial triad, described in patients with a classical 'normal pressure hydrocephalus'-syndrome, is associated with an obstructive hydrocephalus, due to a stenosis of the Sylvian aqueduct.