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

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          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.

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          Most cited references30

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          The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Observations on cerebrospinal fluid hydrodynamics.

          S Hakim, R ADAMS (2015)
          Article 0 comments Cited 132 times – based on 0 reviews     Review now
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            Nationwide Epidemiologic Survey of Idiopathic Osteonecrosis of the Femoral Head

            Wakaba Fukushima, Mikihiro Fujioka, Toshikazu Kubo … (2010)
            Background Although numerous studies describe the clinical characteristics of idiopathic osteonecrosis of the femoral head (ONFH) in specific study populations, these have not been confirmed in countrywide studies. Questions/purposes We therefore determined: (1) the annual number of patients seeking medical care and number of patients newly diagnosed; and (2) the distribution of the age and gender of the patients, potential causative factors, severity of the disease, and operative procedures performed. Patients and Methods We conducted a nationwide epidemiologic survey in 2005. The survey included all orthopaedic departments in Japan by stratified random sampling according to the number of beds. Results The number of patients who sought medical care for idiopathic ONFH during 2004 was estimated to be 11,400 (95% confidence interval, 10,100–12,800). We obtained clinical information from 1502 of these patients. The peak in age distribution occurred in the 40s. Potential causative factors were systemic steroid administration (51%) and habitual alcohol use (31%). Hip replacement was the most frequently performed procedure (65%). Among patients with a history of systemic steroid administration, systemic lupus erythematosus was reported most frequently (31%) as the underlying disease. Among patients younger than 40 years, steroid use was the most prominent potential causative factor (60%), and hip replacement frequently was performed (45%). A greater proportion of patients with no history of steroid or alcohol use was observed among patients 65 years or older (41%). Conclusions In addition to the disease burden of idiopathic ONFH in Japan, our results confirmed the importance of developing preventive and treatment strategies, especially among the younger population. Level of Evidence Level IV, prognostic study. See Guidelines for Authors for a complete description of levels of evidence.
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              The value of supplemental prognostic tests for the preoperative assessment of idiopathic normal-pressure hydrocephalus.

              Anthony Marmarou, Marvin Bergsneider, Petra Klinge … (2005)
              The diagnosis and management of idiopathic normal-pressure hydrocephalus (INPH) remains unclear. Moreover, the value of supplementary tests to predict which patients would benefit from placement of a shunt has not been established. This report develops evidence-based guidelines for the use of supplementary tests as an aid in prognosis. MEDLINE searches from 1966 to the present were undertaken by use of the query NPH, normal-pressure hydrocephalus, lumbar drain, CSF [cerebrospinal fluid] tap test, and external CSF drainage in humans. This resulted in 242 articles. To provide a scientific, evidence-based review, we have chosen to restrict our analysis to clinically relevant studies usually consisting of large numbers of shunted NPH patients. Studies that did not specify INPH or secondary NPH were considered in a separate evidentiary table. Evidence-based guidelines for use in supplementary tests have been developed. A positive response to a 40- to 50-ml tap test has a higher degree of certainty for a favorable response to shunt placement than can be obtained by clinical examination. However, the tap test cannot be used as an exclusionary test because of its low sensitivity (26-61%). Determination of the CSF outflow resistance via an infusion test carries a higher sensitivity (57-100%) compared with the tap test and a similar positive predictive value of 75 to 92%. Prolonged external lumbar drainage in excess of 300 ml is associated with high sensitivity (50-100%) and high positive predictive value (80-100%). To date, a single standard for the prognostic evaluation of INPH patients is lacking. However, supplemental tests can increase predictive accuracy for prognosis to greater than 90%. Additional multicenter prospective randomized clinical trials are needed.
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                Author and article information

                Contributors
                Nagato Kuriyama: nkuriyam@koto.kpu-m.ac.jp
                Journal
                Journal ID (nlm-ta): Brain Behav
                Journal ID (iso-abbrev): Brain Behav
                Journal ID (doi): 10.1002/(ISSN)2157-9032
                Journal ID (publisher-id): BRB3
                Title: Brain and Behavior
                Publisher: John Wiley and Sons Inc. (Hoboken )
                ISSN (Electronic): 2162-3279
                Publication date (Electronic): 27 January 2017
                Publication date Collection: March 2017
                Volume: 7
                Issue: 3 ( doiID: 10.1002/brb3.2017.7.issue-3 )
                Electronic Location Identifier: e00635
                Affiliations
                [ 1 ] Department of Epidemiology for Community Health and MedicineKyoto Prefectural University of Medicine KyotoJapan
                [ 2 ] Department of NeurologyKyoto Prefectural University of Medicine KyotoJapan
                [ 3 ] Department of NeurosurgeryJuntendo University Graduate School of Medicine TokyoJapan
                [ 4 ] Department of Epidemiology and Environmental HealthJuntendo University Graduate School of Medicine TokyoJapan
                [ 5 ] Department of Public HealthOsaka City University Faculty of Medicine OsakaJapan
                [ 6 ]College of Healthcare Management FukuokaJapan
                [ 7 ] Department of Public HealthHokkaido University Graduate School of Medicine SapporoJapan
                [ 8 ] Department of Behavioral Neurology and Cognitive NeuroscienceTohoku University Graduate School of Medicine SendaiJapan
                [ 9 ] Department of Neurology, Hematology, Metabolism, Endocrinology and DiabetologyYamagata University Faculty of Medicine YamagataJapan
                [ 10 ]Mediscience Planning Inc. TokyoJapan
                Author notes
                [*] [* ] Correspondence

                Nagato Kuriyama, Department of Epidemiology for Community Health and Medicine, Kyoto Prefectural University of Medicine, Kyoto, Japan.

                Email: nkuriyam@ 123456koto.kpu-m.ac.jp

                Article
                Publisher ID: BRB3635
                DOI: 10.1002/brb3.635
                PMC ID: 5346522
                PubMed ID: 28293475
                SO-VID: 9fcd5c55-1c63-4da7-a3e1-651d8727169e
                Copyright © © 2017 The Authors. Brain and Behavior published by Wiley Periodicals, Inc.
                License:

                This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.

                History
                Date received : 09 September 2016
                Date revision received : 28 November 2016
                Date accepted : 14 December 2016
                Page count
                Figures: 1, Tables: 5, Pages: 10, Words: 8674
                Funding
                Funded by: Ministry of Health, Labour and Welfare of Japan
                Award ID: 2014‐Nanchi‐General‐052
                Categories
                Subject: Original Research
                Subject: Original Research
                Custom metadata
                source-schema-version-number 2.0
                component-id brb3635
                cover-date March 2017
                details-of-publishers-convertor Converter:WILEY_ML3GV2_TO_NLMPMC version:5.0.8 mode:remove_FC converted:12.03.2017

                ScienceOpen disciplines: Neurosciences
                Keywords: idiopathic normal pressure hydrocephalus,nationwide survey,clinical characteristics,comorbidity
                Data availability:
                ScienceOpen disciplines: Neurosciences
                Keywords: idiopathic normal pressure hydrocephalus, nationwide survey, clinical characteristics, comorbidity

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