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NEUROLOGY 2007;69:917-924
© 2007 American Academy of Neurology

Brain MRI in mucopolysaccharidosis

Effect of aging and correlation with biochemical findings

L. Vedolin, MD, PhD, I.V.D. Schwartz, MD, PhD, M. Komlos, MD, A. Schuch, A. C. Azevedo, MD, T. Vieira, F. K. Maeda, A. M. Marques da Silva and R. Giugliani, MD, PhD

From the Neuroradiology Department, Mãe de Deus Center and Hospital Mãe de Deus (L.V., M.K.); Medical Genetics Service, Hospital de Clínicas (I.V.D.S., A.C.A., T.V., R.G.); Department of Genetics, Federal University of Rio Grande do Sul (I.V.D.S., A.S., R.G.); and Physics Department, Pontifícia Universidade Católica do Rio Grande do Sul (F.K.M., A.M.M.d.S.), Porto Alegre, Brazil.

Address correspondence and reprint requests to Dr. Leonardo M. Vedolin, Av Nilo Peçanha, 550 ap 1301, Bela Vista, 90410.000, Porto Alegre, Brazil leonardovedolin{at}hotmail.com

Objective: To investigate the influence of aging on conventional MRI and magnetic resonance spectroscopy (MRS) findings of mucopolysaccharidosis (MPS) patients and to test the correlation of enzyme levels, urinary glycosaminoglycans (GAG), and neuroimaging findings.

Methods: Sixty patients with MPS types I (n = 8), II (n = 31), IV-A (n = 4), and VI (n = 17) underwent T2, fluid-attenuated inversion recovery (FLAIR), and MRS of the brain. For analysis of MRI variables, we measured the normalized cerebral volume (NCV), CSF volume (NCSFV), ventricular volume (NVV), and lesion load (NLL) on FLAIR using semiautomated and automated segmentation techniques. For MRS, a point-resolved spectroscopy technique was used. Voxels were positioned at the white and gray matter. Statistical analysis involved Pearson or Spearman tests for correlation between neuroimaging, age, enzyme levels, and urinary GAG.

Results: The median age at onset of the disease was 20 months. Patients with longer disease duration had more NLL in the white matter (r = 0.28, p = 0.03), and this difference was more pronounced in MPS II patients (r = 0.44, p = 0.02). Metabolites ratios in MRS, NCV, NCSFV, and NVV did not correlate with disease duration or age of the patients (p > 0.05). MRI and MRS variables in either the white or the gray matter did not correlate with enzymatic activity or GAG levels. Patients with MPS II had a lower mean NCV (p < 0.001).

Conclusions: Our data showed that white matter lesion is more extensive as disease duration increases, especially in mucopolysaccharidosis type II patients. MRI and magnetic resonance spectroscopy findings did not correlate with either enzymatic or glycosaminoglycan levels.

Received December 22, 2006. Accepted in final form April 4, 2007.

Disclosure: The authors report no conflicts of interest.






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