Normal pressure hydrocephalus—an overview of pathophysiological mechanisms and diagnostic procedures

Skalický, Petr; Mládek, Arnošt; Vlasák, Aleš; Lacy, Patricia de; Beneš, Vladimı́r; Bradáč, Ondřej

doi:10.1007/s10143-019-01201-5

Cited by 69 publications

(58 citation statements)

References 124 publications

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“…Abnormal CSF dynamics has been a major research focus in the etiology of iNPH 11 . It was believed to be the initiating factor that contributes to the subsequent ventriculomegaly and neurological deficits in iNPH 6,13 . Mechanisms concerning the disturbance of CSF pulsatility and normal CSF drainage have been proposed.…”

Section: Abnormal Csfmentioning

confidence: 99%

“…Typical brain imaging of iNPH displays ventriculomegaly, periventricular hyperintensities, wide Sylvian fissures, narrowed subarachnoid space, and cortical sulci at the high convexity 5 . In contrast to the secondary normal pressure hydrocephalus with known etiology, the exact cause of iNPH is unknown 6 . Its specific pathogenesis also remains elusive, although several mechanisms have been proposed to contribute to the development of iNPH 7 .…”

Section: Introductionmentioning

confidence: 99%

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Pathogenesis and pathophysiology of idiopathic normal pressure hydrocephalus

Wang

Zhang

et al. 2020

CNS Neurosci Ther

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First defined in 1965, idiopathic normal pressure hydrocephalus (iNPH)is a surgically reversible neurological disorder in adults. It is characterized by dementia, gait disturbance, and urinary incontinence (known as Hakim's triad). 1,2 INPH is not a rare clinical entity. The prevalence of iNPH has been estimated to be 10 per 100 000 to 22 per 100 000 overall, with 1.30% in those aged ≥65 years and 5.9% in those aged ≥80 years. 3,4 One of the core features of iNPH is that the cerebrospinal fluid (CSF) pressure of an iNPH patient is within normal ranges. 2 Typical brain imaging of iNPH displays ventriculomegaly, periventricular hyperintensities, wide Sylvian fissures, narrowed subarachnoid space, and cortical sulci at the high convexity. 5 In contrast to the secondary normal pressure hydrocephalus with known etiology, the exact cause of iNPH is unknown. 6 Its specific pathogenesis also remains elusive, although several

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Section: Abnormal Csfmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pathogenesis and pathophysiology of idiopathic normal pressure hydrocephalus

Wang

Zhang

et al. 2020

CNS Neurosci Ther

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“…A corpus callosum angle of 40° or more and a lack of subarachnoid space over the high convexity (DESH-disproportionately enlarged subarachnoid space hydrocephalus) are among the key neuroradiological features [8], together with a CSF opening pressure ranging from 5 to 18 mmHg (or 70-245 mmH 2 O) [8]. Specific tests for iNPH include the lumbar infusion test, CSF drainage (tap test) by lumbar puncture or lumbar drain [15][16][17][18][19][20][21]. The results of the elaborate diagnostics correlate with the primary shunt response [21,22].…”

Section: Introductionmentioning

confidence: 99%

“…Specific tests for iNPH include the lumbar infusion test, CSF drainage (tap test) by lumbar puncture or lumbar drain [15][16][17][18][19][20][21]. The results of the elaborate diagnostics correlate with the primary shunt response [21,22].…”

Section: Introductionmentioning

confidence: 99%

Secondary deterioration in patients with normal pressure hydrocephalus after ventriculoperitoneal shunt placement: a proposed algorithm of treatment

Gutowski

Rot

Fritsch

et al. 2020

Fluids Barriers CNS

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Background: After ventriculoperitoneal shunt surgery for idiopathic normal pressure hydrocephalus (iNPH) with adjustable gravitational valves, a certain proportion of patients develop secondary clinical worsening after initial improvement of clinical symptoms. The aim of this study was to analyze this group of patients with secondary deterioration and to evaluate the performed shunt management. Methods: For this investigation, we retrospectively reviewed our NPH registry for patients included between 1999 and 2013 with a decrease by a minimum of two points in the Kiefer score in the first year of follow up and an increase of two points in the Kiefer score between the second and the fifth year after shunt surgery (secondary deterioration). Then, we analyzed the patient's shunt management (adapting the valve pressure setting, shuntography, valve replacement, catheter replacement, implant an adjustable gravitational unit). Additionally, we searched for risk factors for secondary deterioration. Results: Out of 259 iNPH patients, 53 (20%) patients showed secondary deterioration on an average of 2.7 (2-4 years) years after shunt surgery. Fourteen (26%) patients with secondary deterioration improved after shunt or valve management and 58% remained without clinical benefit after management. We had a drop-out rate of 15% due to incomplete datasets. Our shunt management reduced the rate of secondary deterioration from 20 to 15%. On the basis of our findings, we developed an algorithm for shunt management. Risk factors for secondary deterioration are the age of the patient at the time of shunting, newly diagnosed neurodegenerative diseases, and overdrainage requiring adjusting the valve to higher-pressure levels. Conclusion: Twenty percent of patients with iNPH were at risk for secondary clinical worsening about 3 years after shunt surgery. About one-fourth of these patients benefited for additional years from pressure level management and/or shunt valve revision. Our findings underline the need for long-term follow-ups and intensive shunt management to achieve a favorable long-term outcome for patients with iNPH and VPS.

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“…Nevertheless, the ocular motor dysfunction can be missing in the early stages of PSP, especially in non-Richardson's phenotypes [2]. On the other side, the urinary dysfunction is a classical feature of iNPH, but can be a non-specific and frequent finding in a population older than 60 years [3].…”

Section: Introductionmentioning

confidence: 99%

Magnetic resonance parkinsonism indices and interpeduncular angle in idiopathic normal pressure hydrocephalus and progressive supranuclear palsy

et al. 2020

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Purpose The clinical presentation of idiopathic normal pressure hydrocephalus (iNPH) may overlap with progressive supranuclear palsy (PSP). The Magnetic Resonance Parkinsonism Index (MRPI), MRPI 2.0, and the interpeduncular angle (IPA) have been investigated to differentiate PSP from healthy controls (HC) and other parkinsonisms. We aimed to assess equivalences and differences in MRPI, MRPI 2.0, and IPA in iNPH, PSP, and HC groups. Methods We retrospectively recruited 99 subjects (30 iNPH, 32 PSP, 37 HC) from two institutions. MRI exams, acquired on either 1.5 T or 3 T scanners, included 3D T1-weighted images to measure MRPI, MRPI 2.0, and IPA. Inter-and intra-rater reliability was investigated with the intra-class correlation coefficient (ICC), and the two one-sided t tests (TOST) procedure was used to assess these markers in iNPH, PSP, and HC. Results For all the three measures, intra-rater and inter-rater ICC were excellent (range = 0.91-0.93). In the comparison of iNPH and PSP with HC, differences for MRPI and MRPI 2.0 (p < 0.01 in all cases) and no equivalence (p = 1.00 in all cases) were found at TOST. iNPH and PSP MRPI showed no difference (p = 0.06) and no equivalence (p = 0.08). MRPI 2.0 was not equivalent (p = 0.06) and not different (p = 0.09) in the same two populations. PSP and HC IPA proved equivalent (p < 0.01) while iNPH IPA was different (p < 0.01) and not equivalent (p = 0.96 and 0.82) from both PSP and HC. Conclusion MRPI and MRPI 2.0 significantly overlap in iNPH and PSP, with risk of misdiagnosis, and for this reason may not be helpful in the differential diagnosis.

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Normal pressure hydrocephalus—an overview of pathophysiological mechanisms and diagnostic procedures

Cited by 69 publications

References 124 publications

Pathogenesis and pathophysiology of idiopathic normal pressure hydrocephalus

Pathogenesis and pathophysiology of idiopathic normal pressure hydrocephalus

Secondary deterioration in patients with normal pressure hydrocephalus after ventriculoperitoneal shunt placement: a proposed algorithm of treatment

Magnetic resonance parkinsonism indices and interpeduncular angle in idiopathic normal pressure hydrocephalus and progressive supranuclear palsy

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