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      Challenges in diagnosing normal pressure hydrocephalus: Evaluation of the diagnostic guidelines

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          Abstract

          Purpose

          To evaluate the present diagnostic guidelines of idiopathic normal pressure hydrocephalus (iNPH) in a sample from the general population.

          Methods

          A total of 168 individuals (93 females, 75 males), mean age 75 years (range 66–92) with and without symptoms of iNPH underwent a CT-scan of the brain, a neurological examination with assessment of the triad symptoms, i.e. gait disturbances, memory impairment and urgency incontinence. The participants were then diagnosed as “unlikely”, “possible” and “probable” iNPH according to the American-European and the Japanese guidelines, respectively. Separately, a senior consultant in neurology diagnosed each patient based on the overall clinical picture.

          Results

          Obtaining a diagnosis of “probable iNPH” was three times more likely according to the American-European guidelines ( n = 35) compared to the Japanese guidelines ( n = 11) or the neurologist ( n = 11). The concordance was highest (Kappa = 0.69) between the Japanese guidelines and the neurologist.

          Conclusions

          Considerable discrepancies were found when diagnosing iNPH according to two international guidelines and a neurologist, respectively. The Japanese guidelines, which include a minimum of two triad symptoms, were most concordant with the neurologist. As a step towards widely accepted, standardized diagnostic criteria, we suggest a revision of the current guidelines, preferably into one common diagnostic system.

          Highlights

          • Normal pressure hydrocephalus lacks common, standardized diagnostic criteria.

          • Considerable discrepancies were found between the two current international guidelines.

          • The highest concordance in diagnoses was between the Japanese guidelines and a neurologist.

          • We suggest a revision of the current guidelines into one common diagnostic system.

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          Most cited references25

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          Validation of Grading Scale for Evaluating Symptoms of Idiopathic Normal-Pressure Hydrocephalus

          Background/Aims: We developed an idiopathic normal-pressure hydrocephalus grading scale (iNPHGS) to classify a triad of disorders (cognitive impairment, gait disturbance and urinary disturbance) of iNPH with a wide range of severity. The purpose of this study was to assess the reliability and validity of this scale in 38 patients with iNPH. Results: The interrater reliability of this scale was high. The iNPHGS cognitive domain score significantly correlated with the cognitive test scores, including the Mini-Mental State Examination (MMSE), the gait domain score with the Up and Go Test and Gait Status Scale scores, and the urinary domain score with the International Consultation on Incontinence Questionnaire-Short Form (ICIQ-SF) score. The MMSE, Gait Status Scale and ICIQ-SF scores significantly improved in patients whose iNPHGS scores improved after CSF tapping but not in those whose iNPHGS scores did not improve after CSF tapping. Fourteen of the 38 patients received shunt operations. In these 14 patients, changes in the iNPHGS cognitive and urinary domains after CSF tapping were significantly associated with the changes after the shunt operation.
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            Diagnosis and Treatment of Idiopathic Normal Pressure Hydrocephalus.

            This article provides neurologists with a pragmatic approach to the diagnosis and treatment of idiopathic normal pressure hydrocephalus (iNPH), including an overview of: (1) key symptoms and examination and radiologic findings; (2) use of appropriate tests to determine the patient's likelihood of shunt responsiveness; (3) appropriate referral to tertiary centers with expertise in complex iNPH; and (4) the contribution of neurologists to the care of patients with iNPH following shunt surgery.
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              The value of supplemental prognostic tests for the preoperative assessment of idiopathic normal-pressure hydrocephalus.

              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
                Journal
                eNeurologicalSci
                eNeurologicalSci
                eNeurologicalSci
                Elsevier
                2405-6502
                11 April 2017
                June 2017
                11 April 2017
                : 7
                : 27-31
                Affiliations
                Department of Pharmacology and Clinical Neuroscience, Neurology, Östersund, Umeå University, Sweden
                Author notes
                [* ]Corresponding author at: Östersund's Hospital, Region Jämtland Härjedalen, Box 654, 831 27 Östersund, Sweden.Östersund's HospitalRegion Jämtland HärjedalenBox 654Östersund831 27Sweden johanna.andersson@ 123456regionjh.se
                Article
                S2405-6502(17)30011-4
                10.1016/j.ensci.2017.04.002
                5746061
                29302622
                822b7aa6-28de-4812-8836-6d44f0c242d1
                © 2017 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 28 March 2017
                : 10 April 2017
                Categories
                Original Article

                hydrocephalus,normal pressure,idiopathic normal pressure hydrocephalus,diagnosis,prevalence studies,cognitive dysfunction,gait disorders,neurologic

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