Idiopathic normal pressure hydrocephalus: historical context and a contemporary guide

Idiopathic normal pressure hydrocephalus (NPH) was described in 1965 as a syndrome in which hydrocephalus develops but with a normal cerebrospinal fluid (CSF) pressure, causing shunt-responsive gait apraxia, cognitive impairment and urinary incontinence. Not all patients respond to shunting despite having the clinical syndrome with appropriate radiological features. This has led to considerable debate over subsequent decades regarding idiopathic NPH. It is now understood that asymptomatic communicating hydrocephalus can develop in many healthy older people, and that over time this can develop into a symptomatic state that sometimes responds to CSF shunting, but to a variable extent. This review looks at the historical background of NPH, the use of predictive tests, the current state of clinical evidence for the diagnosis and treatment of idiopathic NPH and the possible underlying causes, to provide a contemporary practical guide for assessing patients with the radiological features of idiopathic NPH.