Nationwide hospital-based survey of idiopathic normal pressure hydrocephalus in Japan: Epidemiological and clinical characteristics

Nagato Kuriyama, Masakazu Miyajima, Madoka Nakajima, Michiko Kurosawa, Wakaba Fukushima, Yoshiyuki Watanabe, Etsuko Ozaki, Yoshio Hirota, Akiko Tamakoshi, Etsuro Mori, Takeo Kato, Takahiko Tokuda, Akinori Urae, Hajime Arai, Nagato Kuriyama, Masakazu Miyajima, Madoka Nakajima, Michiko Kurosawa, Wakaba Fukushima, Yoshiyuki Watanabe, Etsuko Ozaki, Yoshio Hirota, Akiko Tamakoshi, Etsuro Mori, Takeo Kato, Takahiko Tokuda, Akinori Urae, Hajime Arai

Abstract

Objectives: There have been no nationwide epidemiological studies of idiopathic normal pressure hydrocephalus (iNPH) in Japan. Therefore, a nationwide epidemiologic survey of iNPH was performed to determine the number of cases and clinical characteristics by sex and diagnostic level.

Methods: The first survey examined the numbers of cases that met the diagnostic criteria of iNPH and those who underwent shunt operations in 2012. The second survey gathered patients' details to clarify their clinical background characteristics.

Results: The estimated number of cases meeting the diagnostic criteria in 2012 was 12,900, with 6,700 undergoing shunt operations. The estimated crude prevalence was 10.2/100,000 persons. The age of onset was in the 70s in more than 50% of both men and women. Significantly higher (p < .05) frequencies of gait impairment in men and cognitive decline in women were observed as initial symptoms. At the time of definitive diagnosis, gait impairment was observed most frequently in patients with definite iNPH (77.7%). Hypertension was the most frequent comorbidity (40.0%), followed by diabetes mellitus (17.8%) and Alzheimer's disease (14.8%). Hypertension was observed more frequently in men, but diabetes was observed more frequently in women (p < .05). An LP shunt was the first-choice (55.1%) treatment of iNPH, followed by a VP shunt (43.2%).

Conclusion: This study showed that iNPH occurs most frequently in the 70s, gait impairment and cognitive decline are the most frequent initial symptoms in men and women, respectively, and hypertension and diabetes are the most frequent comorbidities in men and women, respectively.

Keywords: clinical characteristics; comorbidity; idiopathic normal pressure hydrocephalus; nationwide survey.

Figures

Figure 1
Figure 1
Age distribution of the subjects at the time of diagnosis. The age distribution of registered patients with iNPH at the time of definitive diagnosis is shown. Analysis is based on the subjects whose age at the time of diagnosis was available. Those in their 70s account for 50% or more of the registered patients, indicating the peak age of onset of this disease, followed by those in their 80s, accounting for approximately 30% of patients, and those in their 60s, accounting for 15% or less. The age distribution shows no sex‐associated characteristics or differences. These are descriptive statistics showing how the distribution by sex is similar. The box plots are based on the three‐number summary of age: minimum, median, and maximum. The segment inside the rectangle shows the median, and the bars above and below the box show the minimum and maximum, respectively

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