Cargando…
Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses
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...
Autores principales: | , , , , , , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2017
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5603164/ https://www.ncbi.nlm.nih.gov/pubmed/28918752 http://dx.doi.org/10.1186/s12987-017-0073-2 |
_version_ | 1783264688632496128 |
---|---|
author | Corte, Amauri Dalla de Souza, Carolina F. M. Anés, Maurício Maeda, Fabio K. Lokossou, Armelle Vedolin, Leonardo M. Longo, Maria Gabriela Ferreira, Monica M. Perrone, Solanger G. P. Balédent, Olivier Giugliani, Roberto |
author_facet | Corte, Amauri Dalla de Souza, Carolina F. M. Anés, Maurício Maeda, Fabio K. Lokossou, Armelle Vedolin, Leonardo M. Longo, Maria Gabriela Ferreira, Monica M. Perrone, Solanger G. P. Balédent, Olivier Giugliani, Roberto |
author_sort | Corte, Amauri Dalla |
collection | PubMed |
description | 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. |
format | Online Article Text |
id | pubmed-5603164 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-56031642017-09-21 Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses Corte, Amauri Dalla de Souza, Carolina F. M. Anés, Maurício Maeda, Fabio K. Lokossou, Armelle Vedolin, Leonardo M. Longo, Maria Gabriela Ferreira, Monica M. Perrone, Solanger G. P. Balédent, Olivier Giugliani, Roberto Fluids Barriers CNS Research 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. BioMed Central 2017-09-18 /pmc/articles/PMC5603164/ /pubmed/28918752 http://dx.doi.org/10.1186/s12987-017-0073-2 Text en © The Author(s) 2017 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Research Corte, Amauri Dalla de Souza, Carolina F. M. Anés, Maurício Maeda, Fabio K. Lokossou, Armelle Vedolin, Leonardo M. Longo, Maria Gabriela Ferreira, Monica M. Perrone, Solanger G. P. Balédent, Olivier Giugliani, Roberto Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses |
title | Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses |
title_full | Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses |
title_fullStr | Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses |
title_full_unstemmed | Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses |
title_short | Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses |
title_sort | correlation of csf flow using phase-contrast mri with ventriculomegaly and csf opening pressure in mucopolysaccharidoses |
topic | Research |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5603164/ https://www.ncbi.nlm.nih.gov/pubmed/28918752 http://dx.doi.org/10.1186/s12987-017-0073-2 |
work_keys_str_mv | AT corteamauridalla correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses AT desouzacarolinafm correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses AT anesmauricio correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses AT maedafabiok correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses AT lokossouarmelle correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses AT vedolinleonardom correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses AT longomariagabriela correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses AT ferreiramonicam correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses AT perronesolangergp correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses AT baledentolivier correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses AT giuglianiroberto correlationofcsfflowusingphasecontrastmriwithventriculomegalyandcsfopeningpressureinmucopolysaccharidoses |