Diagnosis and Treatment of Idiopathic Normal Pressure Hydrocephalus

Williams, Michael A.; Malm, Jan

doi:10.1212/con.0000000000000305

Cited by 160 publications

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“…1 The most common symptoms of iNPH are summarized in a clinical triad: gait disturbance, dementia, and urinary incontinence. 58 However, these symptoms are not pathognomonic and may also be present in vascular dementia, Alzheimer disease (AD), and Parkinson disease (PD), among many other less common diseases. The exact underlying pathophysiological mechanism of this disorder has remained ill defined, and several theories supporting the combination of CSF circulation disturbances along with other cerebrovascular disorders have been proposed.…”

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“…The exact underlying pathophysiological mechanism of this disorder has remained ill defined, and several theories supporting the combination of CSF circulation disturbances along with other cerebrovascular disorders have been proposed. 1,21,28,36,41,58 The diagnosis of NPH is based on a patient's clinical status, imaging findings, and the natural history of the disease. The imaging criteria for NPH are mainly ventricular enlargement (i.e., with an Evans index > 0.3), lack of any anatomical obstruction of the CSF circulation, enlarged temporal horns, and a corpus callosum (CC) angle of greater than 40° on brain CT or MRI scans.…”

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“…The imaging criteria for NPH are mainly ventricular enlargement (i.e., with an Evans index > 0.3), lack of any anatomical obstruction of the CSF circulation, enlarged temporal horns, and a corpus callosum (CC) angle of greater than 40° on brain CT or MRI scans. 58 Other diagnostic methods are a radionuclide cisternogram showing delayed clearance (i.e., of 48-72 hours) of the infused radiotracer over the cerebral convexities, a brain cine MRI study revealing increased ventricular flow rate, and a SPECT scan indicating decreased periventricular perfusion. 25,31,57 Recently, several clinical investigators have employed diffusion tensor imaging (DTI) techniques to detect and quantify any WM alterations in patients with iNPH.…”

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The role of diffusion tensor imaging and fractional anisotropy in the evaluation of patients with idiopathic normal pressure hydrocephalus: a literature review

Siasios

Kapsalaki²,

Fountas³

et al. 2016

FOC

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OBJECTIVE Diffusion tensor imaging (DTI) for the assessment of fractional anisotropy (FA) and involving measurements of mean diffusivity (MD) and apparent diffusion coefficient (ADC) represents a novel, MRI-based, noninvasive technique that may delineate microstructural changes in cerebral white matter (WM). For example, DTI may be used for the diagnosis and differentiation of idiopathic normal pressure hydrocephalus (iNPH) from other neurodegenerative diseases with similar imaging findings and clinical symptoms and signs. The goal of the current study was to identify and analyze recently published series on the use of DTI as a diagnostic tool. Moreover, the authors also explored the utility of DTI in identifying patients with iNPH who could be managed by surgical intervention. METHODS The authors performed a literature search of the PubMed database by using any possible combinations of the following terms: “Alzheimer's disease,” “brain,” “cerebrospinal fluid,” “CSF,” “diffusion tensor imaging,” “DTI,” “hydrocephalus,” “idiopathic,” “magnetic resonance imaging,” “normal pressure,” “Parkinson's disease,” and “shunting.” Moreover, all reference lists from the retrieved articles were reviewed to identify any additional pertinent articles. RESULTS The literature search retrieved 19 studies in which DTI was used for the identification and differentiation of iNPH from other neurodegenerative diseases. The DTI protocols involved different approaches, such as region of interest (ROI) methods, tract-based spatial statistics, voxel-based analysis, and delta-ADC analysis. The most studied anatomical regions were the periventricular WM areas, such as the internal capsule (IC), the corticospinal tract (CST), and the corpus callosum (CC). Patients with iNPH had significantly higher MD in the periventricular WM areas of the CST and the CC than had healthy controls. In addition, FA and ADCs were significantly higher in the CST of iNPH patients than in any other patients with other neurodegenerative diseases. Gait abnormalities of iNPH patients were statistically significantly and negatively correlated with FA in the CST and the minor forceps. Fractional anisotropy had a sensitivity of 94% and a specificity of 80% for diagnosing iNPH. Furthermore, FA and MD values in the CST, the IC, the anterior thalamic region, the fornix, and the hippocampus regions could help differentiate iNPH from Alzheimer or Parkinson disease. Interestingly, CSF drainage or ventriculoperitoneal shunting significantly modified FA and ADCs in iNPH patients whose condition clinically responded to these maneuvers. CONCLUSIONS Measurements of FA and MD significantly contribute to the detection of axonal loss and gliosis in the periventricular WM areas in patients with iNPH. Diffusion tensor imaging may also represent a valuable noninvasive method for differentiating iNPH from other neurodegenerative diseases. Moreover, DTI can detect dynamic changes in the WM tracts after lumbar drainage or shunting procedures and could help identify iNPH patients who may benefit from surgical intervention.

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The role of diffusion tensor imaging and fractional anisotropy in the evaluation of patients with idiopathic normal pressure hydrocephalus: a literature review

Siasios

Kapsalaki²,

Fountas³

et al. 2016

FOC

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“…Yıllık görülme insidansı 1,8/100,000 olarak bildirilmiştir (5). Görülme sıklığı açısından cinsiyet farkı yoktur (6).…”

Section: Bulgularunclassified

İdiopatik Normal Basınçlı Hidrosefali Hastalarında Kraniyal MRG Bulguları

Yılmaz¹

2018

Akd Med J

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ÖZAmaç: Bu makalenin amacı, normal basınçlı hidrosefalide az bilinen veya göz ardı edilen kraniyal MRG bulgularının vurgulanmasıdır.Gereç ve Yöntemler: 2014-2017 yılları arasında, nöroloji tarafından idiopatik normal basınçlı hidrosefali tanısı konmuş hastalardan; yapılan boşaltıcı lomber ponksiyonda pozitif sonuç elde edilip ventriküloperitoneal şant takılan 9 hasta, boşaltıcı lomber ponksiyonda anlamlı klinik düzelme elde edilememesi üzerine şant takılmasından vazgeçilen 3 hasta ve boşaltıcı lombar ponksiyonu kabul etmeyen 4 hastanın preoperatif kraniyal MRI bulguları retrospektif olarak incelendi.Bulgular: İdiopatik normal basınçlı hidrosefali tanısı konan 8 erkek, 8 kadın toplam 16 hastaincelendi. Ortalama yaş 73,6 (64-89) olarak saptandı. Hastaların %68,75'inde çok belirgin serebral atrofi, %100'ünde temporal hornlarda genişleme, %75'inde silvian fissürde genişleme vardı. Korpus kallozum incelmesi %100, ventrikül çatısında dalgalanma (bulging) %68,75 oranında saptandı. Ortalama kallozal açı 83,4 derece, şant takılıp fayda gören hastalarda ise 71,6 dereceydi. Evans indeksi ortalama 0.4, 3.ventrikül ortalama çapı 1,49 cm, aquaduct çapı ortalama 2,66 mm olarak ölçüldü. Periventriküler hiperintensite %81,25, beyaz cevher hiperintensitesi %62,5 oranında saptandı. Singulat sulkus bulgusu %37,5, mezensefalon bulgusu %81,25, hidrosefaliyle orantısız subaraknoid alan genişlemesi %81,25 oranında saptandı.Sonuç: Literatürde farklı görüşler olmakla birlikte; preoperatif MR'da 40-90 derecelik kallozal açı, temporal hornların dilatasyonu, hidrosefaliyle orantısız genişlemiş bazal subaraknoid alanlar, periventriküler hiperintensite ve boşaltıcı lombar ponksiyondan fayda görülmesi, şanttan faydalanım açısından pozitif belirteçtir.Anahtar Sözcükler: İdiopatik normal basınçlı hidrosefali, ventriküloperitoneal şant, kraniyal MRG ABSTRACT Objective: The aim of this article is to emphasize the findings of minimally known or ignored cranial MRI in normal pressure hydrocephalus. Material and Methods:We retrospectively evaluated the preoperative cranial MRI findings of 9 patients where a ventriculoperitoneal shunt was inserted after a positive result withthe lumbar tap test performed by the author, 3 patients where it was decided not to place a shunt as there was no significant clinical improvement on the lumbar tap test performed by the author, and 4 patients who did not accept a lumbar tap test, among our patients diagnosed with idiopathic normal pressure hydrocephalusby the Neurology Department between 2014 and 2017.Results: A total of 16 patients consisting of 8 males and 8 females with a diagnosis of normal pressure hydrocephalus were evaluated. The mean age was 73.6 (64-89) years. Marked cerebral atrophy was found in 68.75% of the patients, temporal horn dilatation in 100% and Sylvian fissure dilatation in 75%. Corpus callosum thinning was found at a rate of 100% and ventricular roof bulging at 68.75%. The mean callosal angle was 83.4 degrees, decreasing to 71.6 degrees in patients who had benefited from a shunt. The mean Eva...

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“…Normal pressure hydrocephalus (NPH) is a clinical condition with enlarged intra-cerebral ventricles (Hakim & Adams, 1965), and symptoms of gait disturbance, enuresis and cognitive reduction (Fisher, 1982;Williams & Malm, 2016). The small step, shuffling gait is an early dominant symptom, which may be due to direct pressure on the midbrain gait center from an enlarged third ventricle (Lee, Yong, Ahn, & Huh, 2005).…”

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confidence: 99%

Improved lumbar infusion test analysis for normal pressure hydrocephalus diagnosis

Ryding

Kahlon²,

Reinstrup

2018

Brain and Behavior

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ObjectivesConstant infusion lumbar infusion test (LIT) is an important way to find which patients, of those with signs and symptoms corresponding to normal pressure hydrocephalus (NPH) who will improve from shunt operation. LIT is a stress test on the ability for cerebrospinal fluid re‐absorbtion. The aim of this study is to show how the information from LIT can be improved by quantitative analysis and avoidance of methodological pitfalls.Material and methodsThe potential pitfalls, and the analysis method, are described in detail. The analysis was applied on pre‐operative constant infusion LIT from 31 patients operated for NPH, with known outcome. The pre‐ and post‐operative walking speed was used to grade pathology progression or improvement.ResultsThe maximal, plateau, intra‐spinal pressure at constant infusion LIT is an ambivalent indicator for NPH: while low maximal pressure indicates no cerebrospinal fluid (CSF) absorbtion pathology, too high pressure (≥47 mmHg) may mean no diagnosis, because of stenosis of the Sylvian aqueduct. When subjects with too high intra‐spinal pressure were excluded, the new analysis gave a couple of diagnostic volume parameters, of which one appears to be an optimal LIT parameter for identifying NPH patients with 14% better accuracy than plateau pressure.ConclusionBy avoiding methodological pitfalls, and optimal analysis of the results from lumbar infusion test, the number of NPH patients who do not have a successful outcome after shunt operation may be further decreased.

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Diagnosis and Treatment of Idiopathic Normal Pressure Hydrocephalus

Cited by 160 publications

References 53 publications

The role of diffusion tensor imaging and fractional anisotropy in the evaluation of patients with idiopathic normal pressure hydrocephalus: a literature review

The role of diffusion tensor imaging and fractional anisotropy in the evaluation of patients with idiopathic normal pressure hydrocephalus: a literature review

İdiopatik Normal Basınçlı Hidrosefali Hastalarında Kraniyal MRG Bulguları

Improved lumbar infusion test analysis for normal pressure hydrocephalus diagnosis

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