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.
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.
Authors: John P Kim; Margaret R Lentz; Susan V Westmoreland; Jane B Greco; Eva M Ratai; Elkan Halpern; Andrew A Lackner; Eliezer Masliah; R Gilberto González Journal: AJNR Am J Neuroradiol Date: 2005-04 Impact factor: 3.825
Authors: Y Takahashi; K Sukegawa; M Aoki; A Ito; K Suzuki; H Sakaguchi; M Watanabe; K Isogai; S Mizuno; H Hoshi; K Kuwata; S Tomatsu; S Kato; T Ito; N Kondo; T Orii Journal: Pediatr Res Date: 2001-03 Impact factor: 3.756
Authors: Joseph Muenzer; James E Wraith; Michael Beck; Roberto Giugliani; Paul Harmatz; Christine M Eng; Ashok Vellodi; Rick Martin; Uma Ramaswami; Muge Gucsavas-Calikoglu; Suresh Vijayaraghavan; Susanne Wendt; Suzanne Wendt; Ana Cristina Puga; Antonio Puga; Brian Ulbrich; Marwan Shinawi; Maureen Cleary; Diane Piper; Anne Marie Conway; Ann Marie Conway; Alan Kimura Journal: Genet Med Date: 2006-08 Impact factor: 8.822
Authors: R Manara; E Priante; M Grimaldi; L Santoro; G Polonara; R Parini; M Scarpa Journal: AJNR Am J Neuroradiol Date: 2011-12-22 Impact factor: 3.825
Authors: Charles H Vite; Igor Nestrasil; Anton Mlikotic; Jackie K Jens; Elizabeth M Snella; William Gross; Elsa G Shapiro; Victor Kovac; James M Provenzale; Steven Chen; Steven Q Le; Shih-hsin Kan; Shida Banakar; Raymond Y Wang; Mark E Haskins; N Matthew Ellinwood; Patricia I Dickson Journal: Comp Med Date: 2013-04 Impact factor: 0.982