Mucopolysaccharidosis (MPS) is an inherited metabolic disease and a member of the group of lysosomal storage disorders. Its hallmark is a deficiency of lysosomal enzymes involved in the degradation of mucopolysaccharides, also known as glycosaminoglycans (GAGs). The products of GAG degradation accumulate within lysosomes and in the extracellular space, thereby interfering with the degradation of other macromolecules. This process leads to chronic degeneration of cells, which in turn affects multiple organs and systems. There are seven distinct types of MPS (I, II, III, IV, VI, VII, and IX), which are divided into subtypes according to the deficient enzyme and the severity of the clinical picture. Although clinical manifestations vary considerably among the different types of MPS, the central nervous system (CNS) is characteristically affected, and magnetic resonance (MR) imaging is the method of choice to evaluate brain and spinal cord abnormalities. Enlarged perivascular spaces, white matter lesions, hydrocephalus, brain atrophy, cervical spinal canal stenosis with or without spinal cord compression and myelopathy, and bone abnormalities in the skull and spine (dysostosis multiplex) are typical imaging findings described in the literature and reviewed in this article. The differential diagnosis of MPS is limited because the constellation of imaging findings is highly suggestive. Thus, radiologists should be aware of its typical neuroimaging findings so they can recognize cases not yet diagnosed, exclude other metabolic diseases, monitor CNS findings over time, and assess treatment response. (©)RSNA, 2016.

The term rhombencephalitis refers to inflammatory diseases affecting the hindbrain (brainstem and cerebellum). Rhombencephalitis has a wide variety of etiologies, including infections, autoimmune diseases, and paraneoplastic syndromes. Infection with bacteria of the genus Listeria is the most common cause of rhombencephalitis. Primary rhombencephalitis caused by infection with Listeria spp. occurs in healthy young adults. It usually has a biphasic time course with a flu-like syndrome, followed by brainstem dysfunction; 75% of patients have cerebrospinal fluid pleocytosis, and nearly 100% have an abnormal brain magnetic resonance imaging scan. However, other possible causes of rhombencephalitis must be borne in mind. In addition to the clinical aspects, the patterns seen in magnetic resonance imaging can be helpful in defining the possible cause. Some of the reported causes of rhombencephalitis are potentially severe and life threatening; therefore, an accurate initial diagnostic approach is important to establishing a proper early treatment regimen. This pictorial essay reviews the various causes of rhombencephalitis and the corresponding magnetic resonance imaging findings, by describing illustrative confirmed cases.

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In some cases Neu-Laxova syndrome (NLS) is linked to serine deficiency due to mutations in the phosphoglycerate dehydrogenase (PHGDH) gene. We describe the prenatal and postnatal findings in a fetus with one of the most severe NLS phenotypes described so far, caused by a homozygous nonsense mutation of PHGDH. Serial ultrasound (US) and pre- and postnatal magnetic resonance imaging (MRI) evaluations were performed. Prenatally, serial US evaluations suggested symmetric growth restriction, microcephaly, hypoplasia of the cerebellar vermis, micrognathia, hydrops, shortened limbs, arthrogryposis, and talipes equinovarus. The prenatal MRI confirmed these findings prompting a diagnosis of NLS. After birth, radiological imaging did not detect any gross bone abnormalities. DNA was extracted from fetal and parental peripheral blood, all coding exons of PHGDH were PCR-amplified and subjected to Sanger sequencing. Sequencing of PHGDH identified a homozygous premature stop codon mutation (c.1297C>T; p.Gln433*) in fetal DNA, both parents (first-cousins) being heterozygotes. Based on previous associations of mutations in this gene with a milder NLS phenotype, as well as cases of serine deficiency, these observations lend further support to a genotype-phenotype correlation between the degree of PHGDH inactivation and disease severity.

SYNOPSISPolystyrene (PS) (l)/Poly (n-butyl acrylate (BA)/amide type functional monomer) (2) structured latex particles were prepared through emulsion polymerization varying the hydrophilicity of the functional monomer employed. The second-stage polymerization kinetics, the size and morphology of latex particles, and the location of the functional groups in the final latexes were studied, in order to relate them to the thermomechanical properties of films cast from these latexes, It has been shown that, as expected, increasing the hydrophobicity leads to a better homogeneity in the copolymer formed during the second-stage polymerization, while the more hydrophilic functional monomer partly homopolymerizes in a separate phase. However, the functionalization by all the monomers used in this work, prevents the PS seed particles to form a continuous skeleton (percolated network). Further heat treatments a t 140°C do not lead to the formation of a continuous PS phase as for pure BA/pure PS two-stage particles. In addition, some thermally induced crosslinking effects are discussed in relation with the functional monomer location within the particles. 0 1995 John Wiley & Sons, Inc. Keywords: core-shell N-methylol acrylamide N-methylol methacrylamide N-isobutoxy methyl acrylamide butyl acrylate polystyrene seed latex emulsion polymerization structured particles particle morphology functional monomers latex films phase arrangement mechanical properties film forming scanning electron microscopy annealing percolation coalescence I NTRO DU CTlO NIn traditional emulsion polymerization, a functional monomer is such that, when used in small proportions (up to 10% based on the total monomer mixture), it provides the base polymer with a chemical group which is capable of modifying the physicochemical properties of the resulting latex (e.g., viscosity, stability), and/or of the polymer which is recovered from that latex (e.g., adhesion, softening

Atom transfer radical polymerization (ATRP) is a powerful technique for the controlled synthesis of polymers, and one of the most important ATRP characteristics is the possibility to produce functionalized polymers. 2,2,2-Tribromoethanol appears as a promising initiator for the ATRP process, because it allows the production of polymers with end hydroxyl groups, making it easy for copolymerization with biomonomers. This article explores, in experimental and computational level, the styrene ATRP using 2,2,2-tribromoethanol to understand how this new initiator behaves, and presents a powerful tool to predict the polymer properties for different operating conditions. Simulations and experimental results showed that polymers with high molecular weight and low PDI can be simultaneously obtained using 2,2,2-tribromoethanol as initiator. For all operational conditions, the reaction was fast and polydispersity values kept lower than 1.4, confirming the "living"/controlled characteristic. The polymers produced contain hydroxyl as functional group and in some operating conditions, PDI values of 1

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Background?Anterior petrosectomy has become an increasingly used approach for petroclival lesions. This study measures the volume and the anatomical variants of the anterior portion of the petrous apex outlined by the Kawase triangle using computed tomography (CT). Methods?This was a transversal retrospective study. We assessed the anterior petrous apex portion outlined by the Kawase triangle in consecutive patients?>?18 years of age from CT scans of temporal bone stored in an archive system. The volumetry was performed on a workstation. Results?A total of 154 petrosal apex were analyzed in 77 patients (36 men). The average volume of the region outlined by the Kawase triangle was 1.89???0.52 cm3. The volume average in men was 2.01???0.58 cm3, and the average in women was 1.79???0.41 cm3. Intra- and interobserver agreement were both excellent, and there was little variance. Nineteen petrous apex demonstrated anatomical variations. In 18 cases it was pneumatized, and in one case a vascular or nerve-like structure was identified, a report we did not find in the literature. Conclusion?The volumetry of the petrous apex anterior portion outlined by the Kawase triangle can be made by CT with excellent intra- and interobserver agreement and reproducibility. There are anatomical variants in this region that are relevant to surgery.