Epidemiology of Idiopathic Normal Pressure Hydrocephalus: A Systematic Review of the Literature

BACKGROUND: Idiopathic normal pressure hydrocephalus (iNPH) is an important cause of gait disturbance and cognitive impairment in elderly adults. However, the epidemiology of iNPH is relatively unknown, largely as a result of the paucity of specifically designed population studies. This systematic review aims to assess the prevalence and incidence of iNPH. METHODS: A systematic literature review on the epidemiology of iNPH was conducted using MEDLINE/PubMed searching for articles published up to June 2014. RESULTS: The inclusion criteria were met by 21 studies. Of the studies, 12 were specifically designed for detecting cases of iNPH; however, only 4 were prospective. In people >65 years old, pooled prevalence obtained from specific population studies was 1.3%, which was almost 50-fold higher than that inferred from door-to-door surveys of dementia or parkinsonism. Prevalence may be higher in assisted-living and extended-care residents, with 11.6% of patients fulfilling the criteria for suspected iNPH and 2.0% of patients showing permanent improvement after cerebrospinal fluid diversion. The only prospective population-based survey that reported iNPH incidence estimated 1.20 cases/1000 inhabitants/year, 15-fold higher than estimates obtained from studies based on hospital catchment areas. The incidence of shunt surgery for iNPH and shunt-responsive iNPH obtained from incident cases of hospital catchment areas appears to be <2 cases/100,000 inhabitants/year and 1 case/100,000 inhabitants/year, respectively. No population-based study reporting the real values for these 2 parameters could be found. CONCLUSIONS: iNPH appears to be extremely underdiagnosed. Properly designed and adequately powered population-based studies are required to characterize the epidemiology of this disease accurately.