Dataset Information

Hydrocephalic Parkinsonism: lessons from normal pressure hydrocephalus mimics.

ABSTRACT:

Background

Hydrocephalus is an under-recognized presentation of progressive supranuclear palsy (PSP) and dementia with Lewy bodies (DLB).

Methods

We describe four normal pressure hydrocephalus (NPH)-like presentations of pathology-proven PSP (n?=?3) and DLB (n?=?1) and review the literature on the hydrocephalic presentation of these atypical parkinsonisms.

Results

Despite the presence of ventriculomegaly disproportionate to the extent of parenchymal atrophy, all patients demonstrated early postural impairment and/or oculomotor abnormalities that encouraged a diagnostic revision. Hallucinations were the only early atypical manifestation of the hydrocephalic DLB presentation.

Conclusions

Early postural impairment, falls, oculomotor impairment, and/or hallucinations are inconsistent with the diagnosis of NPH and suggest PSP or DLB as the underlying NPH mimic. We postulate that previously reported cases of "dual" pathology (e.g., NPH and PSP) actually represent the hydrocephalic presentation of selected neurodegenerative disorders.

SUBMITTER: Starr BW

PROVIDER: S-EPMC4677733 | biostudies-literature | 2014

REPOSITORIES: biostudies-literature

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Publications

Hydrocephalic Parkinsonism: lessons from normal pressure hydrocephalus mimics.

Starr Brian W BW Hagen Matthew C MC Espay Alberto J AJ

Journal of clinical movement disorders 20141029

<h4>Background</h4>Hydrocephalus is an under-recognized presentation of progressive supranuclear palsy (PSP) and dementia with Lewy bodies (DLB).<h4>Methods</h4>We describe four normal pressure hydrocephalus (NPH)-like presentations of pathology-proven PSP (n = 3) and DLB (n = 1) and review the literature on the hydrocephalic presentation of these atypical parkinsonisms.<h4>Results</h4>Despite the presence of ventriculomegaly disproportionate to the extent of parenchymal atrophy, all patients de ...[more]

PMID: 26788328

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Project description:BackgroundVascular dementia (VaD) and atypical parkinsonism often present with symptoms that can resemble idiopathic normal pressure hydrocephalus (iNPH) and enlarged cerebral ventricles, and can be challenging differential diagnoses. The aim was to investigate frequencies of imaging features usually associated with iNPH and their radiological diagnostic accuracy in a sample containing the relevant differential diagnoses VaD, progressive supranuclear palsy (PSP), multiple system atrophy parkinsonian type (MSA-P), and healthy controls.MethodsNine morphological imaging features usually associated with iNPH were retrospectively investigated in MR images of 55 patients with shunt-responsive iNPH, 32 patients with VaD, 30 patients with PSP, 27 patients with MSA-P, and 39 age-matched healthy controls. Logistic regression and receiver operating characteristic curves were used to assess diagnostic accuracy, sensitivity, and specificity for each imaging finding.ResultsIn a logistic regression model using iNPH diagnosis as a dependent variable, the following imaging features contributed significantly to the model: callosal angle (OR = 0.95 (0.92-0.99), p = 0.012), Evans' index * 100 (OR = 1.51 (1.23-1.86), p < 0.001), enlarged Sylvian fissures (OR = 6.01 (1.42-25.40), p = 0.015), and focally enlarged sulci (OR = 10.18 (1.89-55.02), p = 0.007). Imaging features with 95% specificity for iNPH were: callosal angle ≤ 71°, temporal horns ≥ 7 mm, Evans' index ≥ 0.37, iNPH Radscale ≥ 9, and presence of DESH, bilateral ventricular roof bulgings or focally enlarged sulci. A simplified version of the iNPH Radscale with only four features resulted in equally high diagnostic accuracy as the original iNPH Radscale.ConclusionsThere is a notable overlap between some of the commonly used imaging markers regarding iNPH, VaD and atypical parkinsonism, such as PSP. However, this study shows that the specificity of imaging markers usually associated with iNPH was high even when comparing with these challenging differential diagnoses. The callosal angle was the single imaging feature with highest diagnostic accuracy to discriminate iNPH from its mimics. A simplified rating scale using only a few selected features could be used with retained specificity.

Project description:The glymphatic system has in previous studies been shown as fundamental to clearance of waste metabolites from the brain interstitial space, and is proposed to be instrumental in normal ageing and brain pathology such as Alzheimer's disease and brain trauma. Assessment of glymphatic function using magnetic resonance imaging with intrathecal contrast agent as a cerebrospinal fluid tracer has so far been limited to rodents. We aimed to image cerebrospinal fluid flow characteristics and glymphatic function in humans, and applied the methodology in a prospective study of 15 idiopathic normal pressure hydrocephalus patients (mean age 71.3 ± 8.1 years, three female and 12 male) and eight reference subjects (mean age 41.1 + 13.0 years, six female and two male) with suspected cerebrospinal fluid leakage (seven) and intracranial cyst (one). The imaging protocol included T1-weighted magnetic resonance imaging with equal sequence parameters before and at multiple time points through 24 h after intrathecal injection of the contrast agent gadobutrol at the lumbar level. All study subjects were kept in the supine position between examinations during the first day. Gadobutrol enhancement was measured at all imaging time points from regions of interest placed at predefined locations in brain parenchyma, the subarachnoid and intraventricular space, and inside the sagittal sinus. Parameters demonstrating gadobutrol enhancement and clearance in different locations were compared between idiopathic normal pressure hydrocephalus and reference subjects. A characteristic flow pattern in idiopathic normal hydrocephalus was ventricular reflux of gadobutrol from the subarachnoid space followed by transependymal gadobutrol migration. At the brain surfaces, gadobutrol propagated antegradely along large leptomeningeal arteries in all study subjects, and preceded glymphatic enhancement in adjacent brain tissue, indicating a pivotal role of intracranial pulsations for glymphatic function. In idiopathic normal pressure hydrocephalus, we found delayed enhancement (P < 0.05) and decreased clearance of gadobutrol (P < 0.05) at the Sylvian fissure. Parenchymal (glymphatic) enhancement peaked overnight in both study groups, possibly indicating a crucial role of sleep, and was larger in normal pressure hydrocephalus patients (P < 0.05 at inferior frontal gyrus). We interpret decreased gadobutrol clearance from the subarachnoid space, along with persisting enhancement in brain parenchyma, as signs of reduced glymphatic clearance in idiopathic normal hydrocephalus, and hypothesize that reduced glymphatic function is instrumental for dementia in this disease. The study shows promise for glymphatic magnetic resonance imaging as a method to assess human brain metabolic function and renders a potential for contrast enhanced brain extravascular space imaging.

Dataset Information

Hydrocephalic Parkinsonism: lessons from normal pressure hydrocephalus mimics.

Background

Methods

Results

Conclusions

Publications

Hydrocephalic Parkinsonism: lessons from normal pressure hydrocephalus mimics.

Similar Datasets

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