Brain MRI in mucopolysaccharidosis Effect of aging and correlation with biochemical findings

摘要: Objective: To investigate the influence of aging on conventional MRI and magnetic resonance spectroscopy (MRS) findings mucopolysaccharidosis (MPS) patients to test correlation enzyme levels, urinary glycosaminoglycans (GAG), neuroimaging findings. Methods: Sixty with MPS types I (n = 8), II 31), IV-A 4), VI 17) underwent T2, fluid-attenuated inversion recovery (FLAIR), MRS brain. For analysis variables, we measured normalized cerebral volume (NCV), CSF (NCSFV), ventricular (NVV), lesion load (NLL) FLAIR using semiautomated automated segmentation techniques. MRS, a point-resolved technique was used. Voxels were positioned at white gray matter. Statistical involved Pearson or Spearman tests for between neuroimaging, age, GAG. Results: The median age onset disease 20 months. Patients longer duration had more NLL in matter ( r 0.28, p 0.03), this difference pronounced 0.44, 0.02). Metabolites ratios NCV, NCSFV, NVV did not correlate > 0.05). variables either enzymatic activity GAG levels. lower mean NCV Conclusions: Our data showed that is extensive as increases, especially type patients. glycosaminoglycan