Normal pressure hydrocephalus (NPH) is an uncommon and poorly understood condition that most often affects people over the age of 60. 17. 25 (3): 569-576. 6. For each of these four measures, the effect of the MPRAGE protocol was also less than 0.1 of the standard deviation of that measure. 35 (12): 2311-8. 1996;198(2):523-529. All participants had high-resolution T1-weighted MR images acquired by using an MPRAGE sequence. Average gray matter volume in AD was significantly lower than in NPH or HC volunteers (Tukey honest significant difference test, P < .01). J Neuropsychiatry Clin Neurosci. Furthermore, our study groups were imaged by using different protocols, but we corrected the bias caused by this technical limitation. Onset of gait abnormality before dementia predicts good surgical outcome, Alzheimer’s disease comorbidity in normal pressure hydrocephalus: prevalence and shunt response, Outcome of shunting in idiopathic normal-pressure hydrocephalus and the value of outcome assessment in shunted patients, Categorical data analysis, Brain ventricular size in healthy elderly: comparison between Evans index and volume measurement, https://doi.org/10.1148/radiol.2017161216, Open in Image
Normal pressure hydrocephalus: significance of MRI in a potentially treatable dementia. ■ Clinically useful simple cutoffs may be used as part of a screening tool for patients with NPH versus patients who do not have NPH at MR imaging. Adachi M, Kawanami T, Ohshima F et-al. magnetization-prepared rapid gradient echo, Journal of Clinical Neuroscience, Vol. Data collection and sharing for ADNI supported by National Institutes of Health (U01 AG024904), including the National Institute on Aging, the National Institute of Biomedical Imaging and Bioengineering, and through contributions from Alzheimer’s Association; Alzheimer’s Drug Discovery Foundation; BioClinica; Biogen Idec; Bristol-Myers Squibb; Eisai; Elan Pharmaceuticals; Eli Lilly and Company; F. Hoffmann-La Roche and its affiliated company Genentech; GE Healthcare; Innogenetics; IXICO; Janssen Alzheimer Immunotherapy Research and Development; Johnson and Johnson Pharmaceutical Research and Development; Medpace; Merck; Meso Scale Diagnostics; NeuroRx Research; Novartis Pharmaceuticals Corporation; Pfizer; Piramal Imaging; Servier; Synarc; and Takeda Pharmaceutical Company. 402, Current Gerontology and Geriatrics Research, Vol. American journal of neuroradiology. We used the cutoff of 0.5 to predict the outcome. *Number of patients with NPH who remain in the analysis. It occurs if the normal flow of CSF throughout the brain and spinal cord is blocked in some way. The plots show, A, gray matter, B, white matter, C, ventricles, and, D, hippocampi. 26. The underlying cause remains controversial. By using T1-weighted MPRAGE sequences at the midsagittal plane, a reformatted coronal section was generated at the level of the posterior commissure, perpendicular to a plane that intersected the anterior and posterior commissure (Fig 1). The regression model that used volumetric predictors of gray matter and white matter was 94.3% accurate. The purpose of this study was to construct a radiological scale, composed of morphological signs of iNPH, and compare it … Cerebrospinal fluid dynamics and shunt responsiveness in patients with normal-pressure hydrocephalus. 72, Journal d'imagerie diagnostique et interventionnelle, Magnetic Resonance in Medical Sciences, Vol. Our results support this result. American journal of neuroradiology. Functional ambulation performance is a quantitative, well-validated composite gait measure on the basis of step length, symmetry, and velocity, and the scores range from 95 to 100 in healthy adults (20,21). A classic neurological sign in NPH is magnetic gait, where the patients' feet appear to be magnetically attached to the floor. Ventricular volumes in NPH are on average well above 100 mL (4–6). Favorable prognostic factors include 3. A survey of 284 physicians. We also showed that the predictive model on the basis of combined CA and EI measures demonstrated comparable accuracy to our previously proposed model, which was based on ventricular and gray matter volumes (11) in the distinction between NPH and non-NPH. 7. (2012) Neurologia medico-chirurgica. Normal pressure hydrocephalus: new concepts on etiology and diagnosis. Kitagaki H, Mori E, Ishii K, Yamaji S, Hirono N, Imamura T. CSF spaces in idiopathic normal pressure hydrocephalus: morphology and volumetry. Normal-pressure hydrocephalus. AJNR Am J Neuroradiol. A second theory suggests it results from weakening of the ventricular wall due to periventricular white matter ischemic damage 3. AIC = Akaike information criterion. Bradley WG. The combination of ventriculomegaly, Sylvian fissure widening, and crowding at the vertex has been termed disproportionately enlarged subarachnoid space hydrocephalus (DESH), reflecting disproportionality between the superior and inferior CSF spaces 1,23,27. Diagnostic Nuclear Medicine. The majority of cases are idiopathic. Unfortunately, the diagnostic sensitivity and specificity of EI and CA are controversial (10,12,17–19), and therefore their role has remained limited. Analysis of variance was used to compare mean values of individual variables across study groups. Functional ambulation performance is a quantitative, well-validated composite gait measure on the basis of step length, symmetry, and velocity, and the scores range from 95 to 100 in healthy adults (20,21). On imaging, it can be characterized both on CT and MRI by enlarged lateral and third ventricles out of proportion to the cortical sulcal enlargement. Ambarki et al (28) reported that EI poorly predicts ventricular volume and questioned the use of EI alone as a marker of enlarged ventricles. The utility of these tests for diagnosis and their ability to provide useful prognostic information pertaining to shunting have varied widely. The systematic bias (Vlocal − VADNI) because of the use of different T1-weighted MPRAGE sequences was estimated to be 5.9 mL for gray matter, −1.6 mL for white matter, 0 mL for ventricle, and 0.24 mL for hippocampus. 29. The finding of DESH without neurologic symptoms has been termed asymptomatic ventriculomegaly with features of idiopathic normal pressure hydrocephalus on MRI (AVIM) 1. Images of control patients (both HC patients and patients with AD from ADNI) were acquired with a variety of 1.5-T and 3-T MR imaging systems that used the same T1-weighted MPRAGE sequence. 408, Parkinsonism & Related Disorders, Vol. This task greatly benefits from volumetric segmentation. Aqueductal Stroke Volume: Comparisons with Intracranial Pressure Scores in Idiopathic Normal Pressure Hydrocephalus. Prominent periventricular hyperintensity consistent with transependymal flow of CSF. As the name suggests, mean CSF opening pressure is within the normal range (<18 cmH2O or 13 mmHg) 3. 1996;198 (2): 523-9. 10.4 Imaging 10.4.1 Modalities 10.4.1.1 Computed Tomography Mori E, Ishikawa M, Kato T, Kazui H, Miyake H, Miyajima M, Nakajima M, Hashimoto M, Kuriyama N, Tokuda T, Ishii K, Kaijima M, Hirata Y, Saito M, Arai H. Guidelines for management of idiopathic normal pressure hydrocephalus: second edition. Stein SC, Langfitt TW. 22. The sensitivity and specificity for this cutoff were 87.0% (94 of 108) and 89.5% (188 of 210), respectively. This causes the ventricles to enlarge, putting pressure on the brain. Patients with NPH present with a gradually progressive disorder. Normal pressure hydrocephalus (NPH) happens when too much fluid builds up in a person’s brain without increasing pressure in their brain tissue. 36 (9): 1623-30. Clinically useful cutoffs were obtained to identify and distinguish NPH from the non-NPH patient groups of AD and HC (Fig 3). These measures were tested by using multivariable logistic regression models to determine which combination of metrics is most accurate in diagnosis. Similar to Alzheimer disease (AD), the prevalence of NPH increases with age, reaching about 6% in those over 80 (3). Figure 3: Scatterplot demonstrates the cutoff values of CA and EI that separate patients with NPH from patients who do not have NPH (ie, patients with AD and HC volunteers). This study compared the accuracy of traditional measures of NPH, namely CA and EI, with segmentation metrics for diagnosis. Ringstad G, Emblem KE, Geier O, Alperin N, Eide PK. 2006;24 (8): 568-72. Changes in aqueductal CSF stroke volume and progression of symptoms in patients with unshunted idiopathic normal pressure hydrocephalus. Radiat Med. ■ Volumetric assessment is superior to the combination of CA and EI in a three-way analysis that discriminates between NPH, AD, and HC (overall classification accuracy, 88.7% vs 71.7%). Each ADNI MR imaging examination had to pass screening for blurring, ghosting, and flow artifacts; image homogeneity; signal-to-noise ratio; susceptibility artifacts; and gray-white cerebrospinal fluid contrast. (2010) Cerebrospinal fluid research. Scand. Background: Various radiological markers have been proposed for diagnostics in idiopathic normal pressure hydrocephalus (iNPH). From the Dept of Radiology, Brigham and Women’s Hospital, Boston, Mass (N.M.); Riverside Regional Medical Center, Newport News, Va (H.P. Raftopoulos C, Deleval J, Chaskis C, et al. ); Dept of Radiology, University of Maryland Medical Center, Baltimore, Md (Y.S. {"url":"/signup-modal-props.json?lang=us\u0026email="}, {"containerId":"expandableQuestionsContainer","displayRelatedArticles":true,"displayNextQuestion":true,"displaySkipQuestion":true,"articleId":6306,"mcqUrl":"https://radiopaedia.org/articles/normal-pressure-hydrocephalus/questions/1358?lang=us"}. For comparison between 1.5-T versus 3-T magnetic field strength, we randomly selected five individuals from the ADNI database who underwent two MR examinations within 6 months: one examination with a 1.5-T imager (Avanto; Siemens) and the second with a 3-T imager (Prisma; Siemens). MRS has not been shown to have any added value for differentiating idiopathic NPH from other types of dementia, nor does it help in patient selection for VP shunting 22. A Picture Archiving and Communications in Medicine multiplanar reconstruction tool (Intellispace PACS Enterprise v4.4.516; Philips Healthcare, Amsterdam, the Netherlands) was used for three-dimensional reformatting. 2008;29 (1): 1-3. We used local three-dimensional MPRAGE sequence for 3-T imaging (repetition time msec/echo time msec/inversion time msec, 2100–2200/2.3–4.0/1100; flip angle, 9°–12°; 256 × 256 × 192 matrix; voxel size, 0.8–1.0 mm; and bandwidth, 200/260 Hz/pixel) and 1.5-T imaging (2100–2200/3.8–4.0/1100; flip angle, 12°; 256 × 256 × 160 matrix; voxel size, 0.1–1.0 mm; and bandwidth, 160/200 Hz/pixel). ■ Callosal angle (CA) and Evans index (EI) combined provide good accuracy (average area under the curve, 0.96) to differentiate patients with normal-pressure hydrocephalus (NPH) from patients who do not have NPH (ie, patients with Alzheimer disease [AD] and healthy control [HC] volunteers) and may serve as an accurate screening tool for NPH. 2000;21 (9): 1586-90. If the address matches an existing account you will receive an email with instructions to reset your password. AJNR Am J Neuroradiol 36:831–838 CrossRef PubMed Google Scholar Bradley WG, Kortman KE, Burgoyne B (1986) Flowing cerebrospinal fluid in normal and hydrocephalic states: appearance on … Figure 2: Sample sagittal (left), posterior coronal (middle), and midtransaxial (right) segmentation masks overlaid on T1-weighted MR images of shunt-responsive NPH, definitive AD, and HC volunteers. To assess the diagnostic performance of the callosal angle (CA) and Evans index (EI) measures and to determine their role versus automated volumetric methods in clinical radiology. Is it related to a normal pressure hydrocephalus . Scollato A, Tenenbaum R, Bahl G et-al. J. Larry Jameson, Dennis L. Kasper, Dan Louis Longo, et al. and family’s subjective impression of gait improvement) (n = 101); (b) had available preoperative 1.5-T or 3-T MR examination performed locally that included high-resolution magnetization-prepared rapid gradient-echo (MPRAGE) sequence (n = 60); (c) successfully completed ventricular shunt placement procedure at our institution and subsequently demonstrated significant clinical improvement (n = 54); and (d) were free of comorbidities such as cerebrovascular disease, coexisting intracranial mass lesion, or previous craniectomy identified by a neuroradiologist (A.G.). 10. Multivariable logistic regression models were used to predict NPH (27). Communicating hydrocephalus with reversible cognitive impairment. This is an idiopathic form of communicating hydrocephalus, which features clinically as a triad of dementia, incontinence & gait disturbances. 2013;34 (6): 1168-74. Nuclear medicine is less important in diagnosing NPH. ; experimental studies, N.M., H.P., B.A.A., C.S., Y.S., J.G., O.G., H.R. This study was approved by the institutional research board with a waiver of consent and was Health Insurance Portability and Accountability Act–compliant. Gradually deteriorating neurologic condition with developing characteristic clinical triad, which is called the "triad of Hakim-Adams" is typical for elderly-onset normal pressure hydrocephalus (NPH). Marked cerebrospinal fluid void: indicator of successful shunt in patients with suspected normal-pressure hydrocephalus. 19, No. NPH First described in 1965 by Hakim and Adams. 22. We excluded participants who did not demonstrate this positive response to shunt to minimize confounding of comorbid neurodegenerative disorders. 1997;40 (1): 67-73. It's important to make a correct diagnosis because, unlike Alzheimer’s disease, the … Gait disorders are the initial manifestations; other symptoms appear as the disease progresses (2). 4, © 2021 Radiological Society of North America, For the Alzheimer’s Disease Neuroimaging Initiative, Symptomatic occult hydrocephalus with “normal” cerebrospinal-fluid pressure. The observers were two resident physicians (H.P. For patients from ADNI, we used T1-weighted MPRAGE sequences performed with a 3-T imager (2300/2.98/853; flip angle, 9°; 256 × 256 × 192 matrix; nonisotropic 1.0 × 1.0 × 1.2 mm voxels; and bandwidth, 240 Hz/pixel) and a 1.5-T imager (2400/3.5/1000; flip angle, 8°; 192 × 192 × 160 matrix; 1.2 × 1.2 × 1.25 mm voxels; and bandwidth, 180 Hz/pixel). J. Neurol. Al-Zain FT, Rademacher G, Lemcke J et-al. 23. Table 2 Results from Two Types of Multivariate Logistic Regression Models to Distinguish NPH from Non-NPH Patients. Sci. What do physicians know about normal pressure hydrocephalus and when did they know it? 18. Measures of white matter and hippocampus were not statistically significant predictors. 83 (993): 747-52. A formula (Excel; Microsoft, Redmond, Wash) to calculate probability of NPH was directly derived from the regression model. Additionally, we classified patients as with either AD or NPH, and we did not attempt to distinguish the poorly characterized group of patients with comorbid AD and NPH (4,24). Springer. Figure 4: Box-and-whisker plots of segmentation parameters across the three patient groups. ■ Segmentation metrics may be used as a secondary tool after CA and EI in equivocal cases for more resource intensive but more accurate distinction of NPH from AD or HC. Normal-pressure hydrocephalus: evaluation with cerebrospinal fluid flow measurements at MR imaging. The special clinical problem of symptomatic hydrocephalus with normal cerebrospinal fluid pressure. Normal-pressure hydrocephalus remains a controversial entity with often ambiguous imaging findings. Patients who meet one of the criteria should be categorized as probable NPH. (1998) Journal of neurosurgery. Harrison's Principles of Internal Medicine: Cardinal manifestations and presentation of diseases. We then matched the AD and HC groups with the NPH group for sex and sample size.For the groups, we obtained 34 patients with AD and 36 HC volunteers. Interestingly, when we tested the accuracy of the volumetric model by progressively eliminating patients with the largest ventricles, there was no statistically significant change between the overall volumetric model and the volumetric model based on the NPH group with smaller ventricles. For CA, a cutoff of 100° was 88.7% (282 318) accurate for identification of patients with NPH, and measurements below the cutoff suggested NPH. Acta Neurol. 83, Journal of the Neurological Sciences, Vol. The model that used CA and EI demonstrated 89.6%–93.4% accuracy and average area under the curve of 0.96 in differentiating patients with NPH from patients without NPH (ie, Alzheimer disease and healthy control). 28. In patients with NPH, CSF shunt placement is effective for improving these symptoms . 15. (2008) European radiology. ; statistical analysis, N.M., B.A.A., B.E.D., Y.S., H.R. 11. Shearing forces are exerted on the periventricular white matter as the ventricles enlarge. 14. The incidence is much higher in elderly populations. Bradley WG, Scalzo D, Queralt J et-al. Author contributions: Guarantors of integrity of entire study, N.M., B.A.A., A.L., A.E.G. This is sometimes confusingly referred to as secondary normal pressure hydrocephalus 3. A treatable syndrome, Diagnosis and management of idiopathic normal-pressure hydrocephalus, Incidence of idiopathic normal-pressure hydrocephalus in Northern Spain, Intracranial cerebrospinal fluid measurement studies in suspected idiopathic normal pressure hydrocephalus, secondary normal pressure hydrocephalus, and brain atrophy, Intracranial compartment volumes in normal pressure hydrocephalus: volumetric assessment versus outcome, Comparison of CSF distribution between idiopathic normal pressure hydrocephalus and Alzheimer disease, Prevalence of idiopathic normal-pressure hydrocephalus, Normal-pressure hydrocephalus: evaluation with cerebrospinal fluid flow measurements at MR imaging, Noninvasive MRI assessment of intracranial compliance in idiopathic normal pressure hydrocephalus, Brain MRI as a predictor of CSF tap test response in patients with idiopathic normal pressure hydrocephalus, Differentiating shunt-responsive normal pressure hydrocephalus from Alzheimer disease and normal aging: pilot study using automated MRI brain tissue segmentation, Evans’ index revisited: the need for an alternative in normal pressure hydrocephalus, An encephalographic ratio for estimating ventricular enlargement and cerebral atrophy, Clinical impact of the callosal angle in the diagnosis of idiopathic normal pressure hydrocephalus, Study of INPH on neurological improvement (SINPHONI). These objective cutoffs are clinically relevant, and research showed that visual assessment alone exhibits poor diagnostic accuracy for NPH (12,27). Ventricular volume differences across the three groups were statistically significant (F = 93.7; P < .0001). The mainstay of treatment is the placement of a ventricular shunt. Nervenarzt. VNT = ventricular volume. A classic neurological sign in NPH is magnetic gait, where the patients' feet appear to be magnetically attached to the floor. The most recent advance in shunt valve design is the development of adjustable or programmable valves. Hashimoto M, Ishikawa M, Mori E, Kuwana N. Diagnosis of idiopathic normal pressure hydrocephalus is supported by MRI-based scheme: a prospective cohort study. The current larger patient sample confirms our previous work (11) with highly accurate three-way differential diagnosis that uses the volumetric model. For EI, a cutoff of 0.32 was 81.4% (259 of 318) accurate for identification of patients with NPH, and measurements above the cutoff suggested NPH. A 25-year-old male asked: Is a prominent cistern magma dangerous in any way? AJNR Am J Neuroradiol. ). Normal pressure hydrocephalus (NPH) can be difficult to diagnose because the symptoms start gradually and are similar to those of more common conditions, such as Alzheimer's disease. Abnormal gait: Earliest feature and most responsive to treatment; bradykinetic, broad-based, magnetic, and shuffling gait 2. 20. Radiology. Imaging appearances are nonspecific. Unable to process the form. Unlike the model based on the volumetric measures, the model based on CA and EI performs poorly in classifying both patients with AD and HC volunteers. One of the outcomes of the logistic regression model is the estimated probability of NPH for each patient. 89 (5): 742-7. (2017) Journal of neurosurgery. This article is focused on idiopathic cases. A German epidemiologic study found that normal-pressure hydrocephalus had a prevalence of 0.41% (4:982 individuals) in the over-65 age group. We examined the ADNI database to ensure all participants met the remaining requirement a. Bradley WG (2015) CSF flow in the brain in the context of normal pressure hydrocephalus. For example, when neuroradiologists were asked to independently evaluate T1-weighted brain magnetic resonance (MR) images as either of patients with NPH, patients with AD, or elderly healthy control (HC) volunteers, the diagnostic accuracy was 68%–78%, and the agreement was only fair (intraclass correlation coefficient [ICC], 0.51; 95% confidence interval: 0.34, 0.66) (11). This combined process took an average of 4 minutes per case. Since normal pressure hydrocephalus (NPH) was first described as a clinical entity, its diagnosis has been aided by a variety of radiologic procedures and examinations. The slow enlargement of the ventricles means that the fluid pressure in the brain may not be as high as in other types of hydrocephalus. The term normal pressure hydrocephalus was based on the finding that all 3 patients reported by Hakim and Adams showed low CSF pressures at lumbar puncture, namely 150, 180, and 160 mm H … N.M. and H.R. [Idiopathic normal-pressure hydrocephalus. CA and EI may serve as a screening tool to help the radiologist differentiate patients with NPH from patients without NPH, which would allow for designation of patients for further volumetric assessment. 1970 Aug; 96 (2):347–358. Ventricular enlargement out of proportion to sulcal atrophy. The normal systolic-diastolic pressure wave of the CSF causes three peaks: P1 (systolic pressure), P2 (viscoelastic response of the vessels, parenchyma, and dural sac, i.e., the compliance), and P3 (venous systolic wave). 25. Proc. (2005) Neurosurgery. Table 4 Simulated Exclusion of Patients with NPH with Largest Ventricular Volume. The sensitivity and specificity of this cutoff were 71.3% (77 of 108) and 86.7% (182 of 210), respectively. ICC was used to assess interobserver variability. The British journal of radiology. 30. Fifteen patients with NPH were previously reported (11) by using a voxel-based segmentation method rather than the subvoxel vertex-based tissue segmentation method used in this study. 4, Journal of the Neurological Sciences, Vol. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. ; and manuscript editing, N.M., H.P., A.M.F., B.A.A., B.E.D., Y.S., J.G., O.G., H.R., A.E.G. In future studies, we plan to incorporate sulcal morphologic analysis into our predictive model and discriminate between AD, NPH, and comorbid categories. It can sometimes develop after an injury or a stroke, but in most cases the cause is unknown. The classical clinical findings of normal pressure hydrocephalus are 1-3 1. urinary incontinence 2. deterioration in cognition (dementia) 3. gait disturbances These can be remembered with the unkind mnemonic Wet, Wacky, and Wobbly. All patients with NPH were imaged by using one of several local MR imaging systems (Siemens AG, Erlangen, Germany) 1–5 months before shunt placement. We now know that brain interstitial fluid is the source of 30-40% of CSF2 and 20-40% is absorbed by cranial and spinal nerve sheaths and at the cribriform plate rather than arachnoid granulations.2,3 He… EI is a component of the NPH diagnostic criteria in both the United States (16) and Japan (14). Cognitive recovery in idiopathic normal pressure hydrocephalus. However, our statistical model did compute the probabilities of AD and NPH, which allows future studies to address this limitation and consider an additional comorbid AD and NPH group. The patient on the left (a), reveals normal involution of the brain characterized by deepening of the sulci and prominence of the gyri together with a prominent ventricular system. Normal pressure hydrocephalus (NPH) is characterized by gait disturbance, mental deterioration, and urinary incontinence in patients with an enlarged ventricular system and normal CSF pressure .An important pathophysiological feature of NPH is dysfunctional CSF dynamics with reduced absorption through the arachnoid villi, compensatory CSF flow into the periventricular white matter, … ■ The range for discrimination between patients with NPH and patients who do not have NPH on the basis of combination of CA and EI is 89.6%–93.4% depending on the reader, and it is comparable to 94.3% accuracy of volumetric assessment. Bradley WG, Scalzo D, Queralt J, et al. There is a general agreement that NPH is complex and underdiagnosed, and a detailed characterization of its severity is lacking (2). The final sample size was 36 patients. *Data in parentheses are 95% confidence interval. As such, the investigators within the ADNI contributed to the design and implementation of ADNI and/or provided data but did not participate in analysis or writing of this report. Periventricular hypodensity (on CT) or high T2-FLAIR signal (on MRI) is supportive of changes in brain water content seen in NPH, but these findings can also be seen with microvascular ischemic changes or demyelination 2,25. The Canadian Institutes of Health Research provided funds to support ADNI clinical sites in Canada. Participants in the ADNI were used as both patients with AD and HC volunteers (Table 1). Note.—Data are mean ± standard deviation unless otherwise indicated an injury or a stroke, but in most the. Focal dilation and paradoxical collapse of cortical fissures and sulci in patients with idiopathic normal pressure hydrocephalus and when they. 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