Loading...
Please wait, while we are loading the content...
Similar Documents
Evaluation of Neonatal Acute Metabolic Crisis in Maple Syrup Urine Disease with MR Diffusion and MR Spectroscopy: Case Series and Review of the Literature
| Content Provider | Scilit |
|---|---|
| Author | Chavan, Rajendra Kadam, Sandeep Bhalke, Amit Choudhary, Sohan Lal Patil, Pushpak |
| Copyright Year | 2021 |
| Description | Magnetic resonance imaging (MRI) findings of acute metabolic crisis in maple syrup urine disease (MSUD) in neonates were reviewed. This case cohort study included six MSUD neonates imaged during acute metabolic decompensation. Specific diffusion imaging and proton spectroscopic findings were reviewed. All patients revealed extensive intramyelinic cytotoxic edema typically involving myelinated white matter structures. Brainstem, cerebellar white matter and peduncles, midbrain, posterior limbs of internal capsules, central portions of periventricular, and perirolandic white matter regions showed typical MSUD edema. Gray matter structures such as dentate nucleus and thalamus were involved in all patients. Involvement of other deep nuclei was also noted in a few patients. None of the patients showed involvement of the superficial cortex. Reduction in N-acetyl aspartate, a prominent lactate peak, and a peak representing methyl groups of amino acids were characteristic findings seen on intermediate short echo time MR spectroscopy. Our case series outlines the importance of diffusion and spectroscopy MR techniques in the diagnosis of acute neonatal MSUD metabolic crisis. |
| Related Links | http://www.thieme-connect.de/products/ejournals/pdf/10.1055/s-0041-1726312.pdf |
| Ending Page | 302 |
| Page Count | 6 |
| Starting Page | 297 |
| ISSN | 13042580 |
| DOI | 10.1055/s-0041-1726312 |
| Journal | Journal of Pediatric Neurology |
| Issue Number | 05 |
| Volume Number | 19 |
| Language | English |
| Publisher | Georg Thieme Verlag KG |
| Publisher Date | 2021-03-22 |
| Access Restriction | Open |
| Subject Keyword | Journal: Journal of Pediatric Neurology Maple Syrup Urine Disease Mr Diffusion Imaging Mr Proton Spectroscopy Acute Metabolic Crisis Intramyelinic Edema |
| Content Type | Text |
| Resource Type | Article |
| Subject | Neurology (clinical) Pediatrics, Perinatology and Child Health |
