Prevalence of idiopathic normal pressure hydrocephalus: A prospective, population-based study

Abstract

Background: Idiopathic normal pressure hydrocephalus (iNPH) causing gait impairment, dementia and urinary incontinence among the elderly, is probably under-diagnosed and under-treated. Despite being known since the 1960s, there is still a lack of prospective, population-based studies on the prevalence of iNPH. Such studies are warranted to minimize selection bias and estimate the true prevalence of the disease.

Methods: The prevalence of iNPH was determined in a randomly selected sample of residents, aged 65 years and older, in the Swedish county of Jämtland. Out of 1,000 individuals invited to participate, 673 (67.3%) completed a questionnaire with seven questions on iNPH symptoms. A subgroup, with and without self-reported symptoms, participated in clinical and radiological evaluations and were diagnosed according to international guidelines. Measurement of cerebrospinal fluid opening pressure was not performed as it was considered too invasive.

Results: Those who reported at least two symptoms in the questionnaire (n = 117) and 51 randomly selected individuals with 0-1 symptom participated in further examinations. Out of them, 25 individuals received the diagnosis probable iNPH according to American-European guidelines (except for the criterion of CSF opening pressure) corresponding to a prevalence of 3.7%. The prevalence of iNPH was four times higher among those aged 80 years and older (8.9%) than among those aged 65-79 years (2.1%) (p <0.001). The difference in prevalence between men (4.6%) and women (2.9%) was not significant (p = 0.24). When iNPH was diagnosed according to the Japanese guidelines the prevalence was 1.5.

Conclusions: In this prospective, population-based study the prevalence of iNPH was 3.7% among individuals 65 years and older, and more common in the higher age group, 80 years and above. INPH should be increasingly recognized since it is a fairly common condition and an important cause of gait impairment and dementia among the elderly that can be effectively treated by shunt surgery.

Fig 1. Screening questionnaire (“yes” or “no” answers) in English (bold) and Swedish (italics).

Fig 2. Flow chart of sample selection.

Note: ^a Gait disturbance mandatory. ^b Including 49 individuals reporting two symptoms of incontinence and cognition but no gait disturbance. ^c Randomly selected, including five individuals reporting two symptoms on incontinence and cognition but no gait disturbance.

Fig 3. Prevalence of iNPH (possible iNPH excluded) in relation to age and sex with the American-European and Japanese guidelines, respectively.