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Fluids and Barriers of the CNS
Published in: Fluids and Barriers of the CNS 1/2017

Open Access 01-12-2017 | Research

Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses

Authors: Amauri Dalla Corte, Carolina F. M. de Souza, Maurício Anés, Fabio K. Maeda, Armelle Lokossou, Leonardo M. Vedolin, Maria Gabriela Longo, Monica M. Ferreira, Solanger G. P. Perrone, Olivier Balédent, Roberto Giugliani

Published in: Fluids and Barriers of the CNS | Issue 1/2017

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Abstract

Background

Very little is known about the incidence and prevalence of hydrocephalus in patients with mucopolysaccharidoses (MPS). The biggest challenge is to distinguish communicating hydrocephalus from ventricular dilatation secondary to brain atrophy, because both conditions share common clinical and neuroradiological features. The main purpose of this study is to assess the relationship between ventriculomegaly, brain and cerebrospinal fluid (CSF) volumes, aqueductal and cervical CSF flows, and CSF opening pressure in MPS patients, and to provide potential biomarkers for abnormal CSF circulation.

Methods

Forty-three MPS patients (12 MPS I, 15 MPS II, 5 MPS III, 9 MPS IV A and 2 MPS VI) performed clinical and developmental tests, and T1, T2, FLAIR and phase-contrast magnetic resonance imaging (MRI) followed by a lumbar puncture with the CSF opening pressure assessment. For the analysis of MRI variables, we measured the brain and CSF volumes, white matter (WM) lesion load, Evans’ index, third ventricle width, callosal angle, dilated perivascular spaces (PVS), craniocervical junction stenosis, aqueductal and cervical CSF stroke volumes, and CSF glycosaminoglycans concentration.

Results

All the scores used to assess the supratentorial ventricles enlargement and the ventricular CSF volume presented a moderate correlation with the aqueductal CSF stroke volume (ACSV). The CSF opening pressure did not correlate either with the three measures of ventriculomegaly, or the ventricular CSF volume, or with the ACSV. Dilated PVS showed a significant association with the ventriculomegaly, ventricular CSF volume and elevated ACSV.

Conclusions

In MPS patients ventriculomegaly is associated with a severe phenotype, increased cognitive decline, WM lesion severity and enlarged PVS. The authors have shown that there are associations between CSF flow measurements and measurements related to CSF volumetrics. There was also an association of volumetric measurements with the degree of dilated PVS.
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Metadata
Title
Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses
Authors
Amauri Dalla Corte
Carolina F. M. de Souza
Maurício Anés
Fabio K. Maeda
Armelle Lokossou
Leonardo M. Vedolin
Maria Gabriela Longo
Monica M. Ferreira
Solanger G. P. Perrone
Olivier Balédent
Roberto Giugliani
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Fluids and Barriers of the CNS / Issue 1/2017
Electronic ISSN: 2045-8118
DOI
https://doi.org/10.1186/s12987-017-0073-2

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