Survival in treated idiopathic normal pressure hydrocephalus

J Neurol. 2020 Mar;267(3):640-648. doi: 10.1007/s00415-019-09598-1. Epub 2019 Nov 11.


Objective: To describe survival and causes of death in 979 treated iNPH patients from the Swedish Hydrocephalus Quality Registry (SHQR), and to examine the influence of comorbidities, symptom severity and postoperative outcome.

Methods: All 979 patients operated for iNPH 2004-2011 and registered in the SHQR were included. A matched control group of 4890 persons from the general population was selected by Statistics Sweden. Data from the Swedish Cause of Death Registry was obtained for patients and controls.

Results: At a median 5.9 (IQR 4.2-8.1) year follow-up, 37% of the iNPH patients and 23% of the controls had died. Mortality was increased in iNPH patients by a hazard ratio of 1.81, 95% CI 1.61-2.04, p < 0.001. More pronounced symptoms in the preoperative ordinal gait scale and the Mini-mental State Examination were the most important independent predictors of mortality along with the prevalence of heart disease. Patients who improved in both the gait scale and in the modified Rankin Scale postoperatively (n = 144) had a similar survival as the general population (p = 0.391). Deaths due to cerebrovascular disease or dementia were more common in iNPH patients, while more controls died because of neoplasms or disorders of the circulatory system.

Conclusions: Mortality in operated iNPH patients is 1.8 times increased compared to the general population, a lower figure than previously reported. The survival of iNPH patients who improve in gait and functional independence is similar to that of the general population, indicating that shunt surgery for iNPH, besides improving symptoms and signs, can normalize survival.

Keywords: Cognitive disorders; Cohort studies; Gait disorders; Hydrocephalus; Prognosis.

MeSH terms

  • Aged
  • Cerebrospinal Fluid Shunts / mortality*
  • Comorbidity
  • Female
  • Humans
  • Hydrocephalus, Normal Pressure / mortality*
  • Hydrocephalus, Normal Pressure / surgery*
  • Male
  • Risk Factors
  • Sweden / epidemiology