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      Diagnosis of idiopathic normal pressure hydrocephalus is supported by MRI-based scheme: a prospective cohort study


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          Idiopathic normal pressure hydrocephalus (iNPH) is a treatable neurological syndrome in the elderly. Although the magnetic resonance imaging (MRI) findings of tight high-convexity and medial subarachnoid spaces and the ventriculo-peritoneal (VP) shunt with programmable valve are reportedly useful for diagnosis and treatment, respectively, their clinical significance remains to be validated. We conducted a multicenter prospective study (Study of Idiopathic Normal Pressure Hydrocephalus on Neurological Improvement: SINPHONI) to evaluate the utility of the MRI-based diagnosis for determining the 1-year outcome after VP shunt with the Codman-Hakim programmable valve.


          Twenty-six centers in Japan were involved in this study. Patients aged between 60 and 85 years with one or more of symptoms (gait, cognitive, and urinary problems) and MRI evidence of ventriculomegaly and tight high-convexity and medial subarachnoid spaces received VP shunt using the height/weight-based valve pressure-setting scheme. The primary endpoint was a favorable outcome (improvement of one level or more on the modified Rankin Scale: mRS) at one year after surgery, and the secondary endpoints included improvement of one point or more on the total score of the iNPH grading scale. Shunt responder was defined by more than one level on mRS at any evaluation point in one year.


          The full analysis set included 100 patients. A favorable outcome was achieved in 69.0% and 80.0% were shunt responders. When measured with the iNPH grading scale, the one-year improvement rate was 77.0%, and response to the surgery at any evaluation point was detected in 89.0%. Serious adverse events were recorded in 15 patients, three of which were events related to surgery or VP shunt. Subdural effusion and orthostatic headache were reported as non-serious shunt-related adverse events, which were well controlled with readjustment of pressure.


          The MRI-based diagnostic scheme is highly useful. Tight high-convexity and medial subarachnoid spaces, and enlarged Sylvian fissures with ventriculomegaly, defined as disproportionately enlarged subarachnoid-space hydrocephalus (DESH), are worthwhile for the diagnosis of iNPH. This study is registered with ClinicalTrials.gov, number NCT00221091.

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          Most cited references23

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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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            Diagnostic intracranial pressure monitoring and surgical management in idiopathic normal pressure hydrocephalus: a 6-year review of 214 patients.

            To review our experience of managing idiopathic normal pressure hydrocephalus (iNPH) during the 6-year period from 2002 to 2007, when intracranial pressure (ICP) monitoring was part of the diagnostic workup. The review includes all iNPH patients undergoing diagnostic ICP monitoring during the years 2002 to 2007. Clinical grading was done prospectively using a normal pressure hydrocephalus (NPH) grading scale (scores from 3 to 15). The selection of patients for surgery was based on clinical symptoms, enlarged cerebral ventricles, and findings on ICP monitoring. The median follow-up time was 2 years (range, 0.3-6 years). Both static ICP and pulsatile ICP were analyzed. A total of 214 patients underwent the diagnostic workup, of whom 131 went on to surgery. Although 1 patient died shortly after treatment, 103 of the 130 patients (79%) improved clinically. This improvement lasted throughout the observation period. The static ICP observed during ICP monitoring was a poor predictor of the response to surgery. In contrast, among 109 of 130 patients with increased ICP pulsatility (ie, ICP wave amplitude >4 mm Hg on average and >5 mm Hg in >10% of recording time), 101 (93%) were responders (ie, increase in the NPH score of >2). Correspondingly, only 2 of 21 (10%) without increased ICP pulsatility were responders. Superficial wound infection was the only complication of ICP monitoring and occurred in 4 (2%) patients. Surgical results in iNPH were good with almost 80% of patients improving after treatment. The data indicate that improvement after surgery can be anticipated in 9 of 10 iNPH patients with abnormal ICP pulsatility, but in only 1 of 10 with normal ICP pulsatility. Diagnostic ICP monitoring had a low complication rate.
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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.

                Author and article information

                Cerebrospinal Fluid Res
                Cerebrospinal Fluid Research
                BioMed Central
                31 October 2010
                : 7
                : 18
                [1 ]Department of Neurosurgery, Noto General Hospital, Nanao, 926-8610, Japan
                [2 ]Department of Neurosurgery, Rakuwakai Otowa Hospital, Kyoto, 607-8602, Japan
                [3 ]Department of Behavioral Neurology and Cognitive Neuroscience, Tohoku University Graduate School, Sendai, 980-8575, Japan
                [4 ]Department of Neurosurgery, Tokyo Kyosai Hospital, Tokyo, 153-8934, Japan
                Copyright ©2010 Hashimoto et al; licensee BioMed Central Ltd.

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.




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