ArticleAJNR. American journal of neuroradiology
Correlation of MR imaging and MR spectroscopy findings with cognitive impairment in mucopolysaccharidosis II.
Article in AJNR. American journal of neuroradiology. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 24 papers.
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24 citing papers in PubMed, 69 citations in OpenAlex.
- Cognitive, medical, and neuroimaging characteristics of attenuated mucopolysaccharidosis type II.Molecular genetics and metabolism · 2015Trial
- Correlation of automated volumetric analysis of brain MR imaging with cognitive impairment in a natural history study of mucopolysaccharidosis II.AJNR. American journal of neuroradiology · 2010Trial
- Atypical Neuroimaging Findings in a Patient With Mucopolysaccharidosis Type VII (Sly Syndrome).Cureus · 2024Article
- Venous Sinus Stenosis with Prominent Emissary Veins: A New Common Cranial MRI Finding of Mucopolysaccharidosis I.AJNR. American journal of neuroradiology · 2023Article
- Magnetic resonance imaging findings of the posterior fossa in 47 patients with mucopolysaccharidoses: A cross-sectional analysis.JIMD reports · 2021Article
- Can serial cerebral MRIs predict the neuronopathic phenotype of MPS II?Journal of inherited metabolic disease · 2021Article
- Neurobehavioral phenotypes of neuronopathic mucopolysaccharidoses.Italian journal of pediatrics · 2018Review
- Mucopolysaccharidoses: overview of neuroimaging manifestations.Pediatric radiology · 2018Review
- Correlation of CSF flow using phase-contrast MRI with ventriculomegaly and CSF opening pressure in mucopolysaccharidoses.Fluids and barriers of the CNS · 2017Article
- Hydrocephalus and mucopolysaccharidoses: what do we know and what do we not know?Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2017Review
- Association between brain structural anomalies, electroencephalogram and history of seizures in Mucopolysaccharidosis type II (Hunter syndrome).Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2017Article
- A longitudinal study of emotional adjustment, quality of life and adaptive function in attenuated MPS II.Molecular genetics and metabolism reports · 2016Article
- In Vivo NMR Studies of the Brain with Hereditary or Acquired Metabolic Disorders.Neurochemical research · 2015Review
- Imaging findings of mucopolysaccharidoses: a pictorial review.Insights into imaging · 2013Article
- Features of brain MRI in dogs with treated and untreated mucopolysaccharidosis type I.Comparative medicine · 2013Article
- Brain magnetic resonance imaging findings in patients with mucopolysaccharidosis VI.Journal of inherited metabolic disease · 2013Article
- Brain and spinal MR imaging findings in mucopolysaccharidoses: a review.AJNR. American journal of neuroradiology · 2013Review
- Closed Meningo(encephalo)cele: a new feature in Hunter syndrome.AJNR. American journal of neuroradiology · 2012Article
- Proton nuclear magnetic resonance spectroscopic detection of oligomannosidic n glycans in alpha-mannosidosis: a method of monitoring treatment.Journal of inherited metabolic disease · 2011Article
- Glycosaminoglycan storage in neuroanatomical regions of mucopolysaccharidosis I dogs following intrathecal recombinant human iduronidase.APMIS : acta pathologica, microbiologica, et immunologica Scandinavica · 2011Article
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Authors and funding
8 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
background and purposeThere 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
methodsNineteen 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.
resultsThe 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.
conclusionsOur study showed that severe WM lesions, brain atrophy, hydrocephalus, and elevated mIns/Cr were more common in patients with MPS II and with CI.
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