Abstract
BackgroundSpinocerebellar ataxia (SCA) and multiple system atrophy-cerebellar type (MSA-C) often present with similar clinical manifestations in the beginning. Magnetic resonance spectroscopy (MRS) has been proved to be a useful tool to help differentiate different types of SCA and MSA-C on cross-sectional studies. However, longitudinal changes of the MRS metabolites in these subjects have never been reported. The purpose of this study was to track the longitudinal evolution of the MRS metabolites in these patients and to ascertain the correlation between clinical severity measured by Scale of the Assessment and Rating of Ataxia (SARA) and MRS metabolites.ResultsSignificant reductions of NAA/Cr and NAA/Cho in the cerebellar hemispheres in all patients and lower Cho/Cr in the cerebellar hemispheres in patients with SCA2 or MSA-C were found at all times. At initial assessments, patients with MSA-C or SCA2 tended to have lower NAA/Cr and Cho/Cr in the cerebellar hemispheres than those with SCA3 or SCA6. At follow-ups, patients with SCA2 or MSA-C had a lower NAA/Cr in cerebellar hemispheres than those with SCA3 or SCA6. Patients with MSA-C had a lower NAA/Cr in the vermis and Cho/Cr in the cerebellar hemispheres than those with SCA2 at the start, and had a lower NAA/Cr in cerebellar hemispheres than those with SCA2 at follow-ups.ConclusionCharacteristic patterns of neurodegenerative evolution were observed in patients with disparate SCAs and MSA-C using MRS and SARA. A continual impairment of neuronal integrity was observed in all groups of patients. The longitudinal changes of MRS metabolites and SARA scores were most striking in patients with SCA2 and MSA-C. Although the changes in the metabolites on MRS may still be used to help understand the pathophysiology of ataxia disorders, they are short of being a good biomarker.
Highlights
Spinocerebellar ataxia (SCA) and multiple system atrophy-cerebellar type (MSA-C) often present with similar clinical manifestations in the beginning
There was no significant difference between patient groups in terms of disease duration, SARA scores at the time when the first or the second Magnetic resonance spectroscopy (MRS) assessment was performed, or the time interval in between two assessments
The correlation between the changes of MRS metabolites and SARA scores The changes of NAA/Cr (R = −0.332, P < 0.001 in the cerebellar hemispheres; R = −0.521, P < 0.001 in the vermis) and Cho/Cr (R = −0.380; P < 0.001 in the cerebellar hemispheres; R = −0.529, P < 0.001 in the vermis) were inversely correlated with the changes of SARA scores (Figure 3). To our knowledge, this is the first study designed to evaluate the longitudinal changes of metabolites on MRS in conjunction with the clinical severity measured with SARA in patients with ataxia syndromes
Summary
Spinocerebellar ataxia (SCA) and multiple system atrophy-cerebellar type (MSA-C) often present with similar clinical manifestations in the beginning. Magnetic resonance spectroscopy (MRS) has been proved to be a useful tool to help differentiate different types of SCA and MSA-C on cross-sectional studies. Multiple system atrophy-cerebellar type (MSA-C) is the most common sporadic cerebellar ataxia with a rapidly used to measure the clinical severity and progression of diseases with ataxia. Magnetic resonance spectroscopy (MRS) provides a measure of brain chemistry and has been widely used to non-invasively evaluate in vivo changes of the metabolites in the brain. Choline (Cho) is a measure of cellular turnover, reflecting the content of cytosolic glycerolphosphocholine and phosphocholine and representing the products of membrane phosphotidylcholine breakdown, precursors of choline and acetylcholine synthesis [10]. A reduction in Cho reflects impairment in the production of cell membranes, the precursor of neurotransmitter acetylcholine and acetylcholine itself. NAA/ Cho has been used as a marker for cerebral metabolism [6,11,12]
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
