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The Value of 1H-MRS and MRI in Combined Methylmalonic Aciduria and Homocystinuria
| Content Provider | Scilit |
|---|---|
| Author | Cheng, Ailan Yao, Rong Cao, Wenjun Yu, Hong |
| Copyright Year | 2019 |
| Description | Journal: Journal of computer assisted tomography The aims of this study were to describe the brain magnetic resonance imaging (MRI) features of methylmalonic aciduria and homocystinuria and to evaluate the additional value of 1H-MRS. Twenty-eight children with methylmalonic aciduria and homocystinuria were included in this study. The control group included 21 healthy children. All the cases underwent MRI and 1H-MRS before treatment. We measured the N-acetylaspartate (NAA), choline (Cho), creatine (Cr), and myoinositol (mI) peaks in the basal ganglia regions. The NAA/Cr, Cho/Cr, mI/Cr, and NAA/Cho ratios were calculated. We also observed whether there were lactic acid peaks. We identified that NAA/Cr and NAA/Cho significantly decreased in the basal ganglia and that 3 patients showed lactate peaks, but other metabolites were not significantly altered. Hydrocephalus and diffuse supratentorial white matter edema were the primary MR findings; 7 patients had thinning of the corpus callosum, and 2 patients had subdural hematoma. Six patients showed normal brain MRI findings. Methylmalonic aciduria and homocystinuria patients with metabolite changes in the basal ganglia demonstrate compromised neuronal integrity, and anerobic metabolism occurs in acute encephalopathic episodes. 1H-MRS is a useful tool for evaluating brain damage. Hydrocephalus and diffuse supratentorial white matter edema are the main MRI features. This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. |
| Related Links | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6636799/pdf |
| Ending Page | 562 |
| Page Count | 4 |
| Starting Page | 559 |
| ISSN | 03638715 |
| e-ISSN | 15323145 |
| DOI | 10.1097/rct.0000000000000854 |
| Journal | Journal of computer assisted tomography |
| Issue Number | 4 |
| Volume Number | 43 |
| Language | English |
| Publisher | Ovid Technologies (Wolters Kluwer Health) |
| Publisher Date | 2019-01-01 |
| Access Restriction | Open |
| Subject Keyword | Journal: Journal of computer assisted tomography Clinical Neurology Methylmalonic Aciduria |
| Content Type | Text |
| Resource Type | Article |
| Subject | Radiology, Nuclear Medicine and Imaging |
