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Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in
Amauri Dalla Corte1,2, Carolina F M de Souza3, Maurício Anés4
1Post-Graduate Program in Medical Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil. dalacorte@gmail.com.
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.
Insights
In mucopolysaccharidoses (MPS), ventriculomegaly correlates with cerebrospinal fluid (CSF) flow and white matter lesions. Dilated perivascular spaces (PVS) are linked to enlarged ventricles and abnormal CSF flow in MPS patients.
Area of Science:
- Neurology
- Genetics
- Biochemistry
Background:
- Limited understanding of hydrocephalus incidence and prevalence in mucopolysaccharidoses (MPS).
- Difficulty distinguishing communicating hydrocephalus from brain atrophy in MPS due to overlapping features.
- Need for biomarkers to identify abnormal cerebrospinal fluid (CSF) circulation in MPS.
Purpose of the Study:
- Assess relationships between ventriculomegaly, brain/CSF volumes, and CSF flows in MPS.
- Investigate associations with CSF opening pressure and potential biomarkers for CSF circulation abnormalities.
Main Methods:
- Studied 43 MPS patients (MPS I, II, III, IV A, VI) with clinical, developmental, and MRI assessments.
- Utilized MRI for brain/CSF volumes, white matter lesion load, and CSF stroke volumes (aqueductal and cervical).
- Measured CSF opening pressure and glycosaminoglycans concentration via lumbar puncture.
Main Results:
- Ventricle enlargement scores moderately correlated with aqueductal CSF stroke volume (ACSV).
- CSF opening pressure showed no correlation with ventriculomegaly, ventricular volume, or ACSV.
- Dilated perivascular spaces (PVS) significantly associated with ventriculomegaly, ventricular volume, and elevated ACSV.
Conclusions:
- Ventricular enlargement in MPS is linked to severe phenotype, cognitive decline, white matter lesions, and enlarged PVS.
- Demonstrated associations between CSF flow dynamics and volumetric measurements.
- Volumetric measurements correlated with the degree of PVS dilation.
