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AJNR - American Journal of Neuroradiology

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American Journal of Neuroradiology 28:1029-1033, June-July 2007
DOI 10.3174/ajnr.A0510
© 2007 American Society of Neuroradiology

PEDIATRICS

Correlation of MR Imaging and MR Spectroscopy Findings with Cognitive Impairment in Mucopolysaccharidosis II

L. Vedolin^a, I.V.D. Schwartz^b,c, M. Komlos^a, A. Schuch^c, A.C. Puga^b, L.L.C. Pinto^b, A.P. Pires^b and R. Giugliani^b,c

^a Neuroradiology Department, Mãe de Deus Center and Hospital Mãe de Deus, Porto Alegre, Brazil
^b Medical Genetics Service, Hospital de Clínicas, Porto Alegre, Brazil
^c Department of Genetics, Federal University of Rio Grande do Sul, Porto Alegre, Brazil

Address correspondence to Leonardo M. Vedolin, Av Nilo Peçanha, 550 ap 1301 –Bela Vista –90410.000, Porto Alegre, Brazil; e-mail: leonardovedolin{at}hotmail.com

BACKGROUND AND PURPOSE: There are no reliable markers to predict neurologic outcome of patients with mucopolysaccharidosis (MPS) II. We hypothesized that brain MR imaging and MR spectroscopy are useful in depicting features related to cognitive impairment (CI) in MPS II.

MATERIALS AND METHODS: Nineteen male patients with MPS II were included in this study. They were evaluated through intelligence/developmental tests to be classified in 2 groups: patients with CI (group A) or patients without CI (group B). Brain MR imaging evaluated white matter (WM) lesions, hydrocephalus, and brain atrophy. Voxels from MR spectroscopy (point-resolved spectroscopy TE 30 ms) were positioned in the WM of the deep right frontal lobe and at the gray matter (GM) in the posterior occipital cortex across the midline. Comparison of MR imaging and MR spectroscopy findings between these 2 groups and a control group was performed.

RESULTS: The mean age of the patients was 9.6 years (group A, 7.08 years old, 12 patients; group B, 14 years old, 7 patients; P = .076). Brain atrophy and hydrocephalus were more frequently found in group A patients (P = .006 and P = .029, respectively); these patients also presented more severe WM lesions than patients from group B (P = .022). Patients from group A also had a higher myo-inositol (mIns)/creatine (Cr) ratio in the GM (P = .046) and in the WM (P = .032). The choline/Cr and N-acetylaspartate/Cr ratios were similar in both groups.

CONCLUSIONS: Our study showed that severe WM lesions, brain atrophy, hydrocephalus, and elevated mIns/Cr were more common in patients with MPS II and with CI.