Spinal involvement in mucopolysaccharidoses: a review.

BACKGROUND: Mucopolysaccharidoses (MPS) represent a group of inheritable lysosomal storage diseases caused by mutations in the genes coding for enzymes involved in catabolism of different glycosaminoglycans (GAGs). They are clinically heterogeneous multisystemic diseases, often involving the spine. Bony abnormalities of the spine included in the so-called dysostosis multiplex and GAG deposits in the dura mater and supporting ligaments can result in spinal cord compression, which can lead to compressive myelopathy. Spinal involvement is a major cause of morbidity and mortality in some MPS (e.g., MPS IVA, VI, and I), and early radiological diagnosis is critical in preventing or arresting neurological deterioration and loss of function. DISCUSSION: Management of MPS, however, requires a multidisciplinary approach because of the multiorgan nature of the disease. Indeed in order to appreciate the relevance and nuances of each other's specialty, radiologists and clinicians need to have a background of common knowledge, rather than a merely compartmentalized point of view. In the interest of the management of spinal involvement in MPS, this review article aims on one hand to provide radiologists with important clinical knowledge and on the other hand to equip clinicians with relevant radiological semiotics.

Number of steady states of quantum evolutions.

We prove sharp universal upper bounds on the number of linearly independent steady and asymptotic states of discrete- and continuous-time Markovian evolutions of open quantum systems. We show that the bounds depend only on the dimension of the system and not on the details of the dynamics. A comparison with similar bounds deriving from a recent spectral conjecture for Markovian evolutions is also provided.

Diffusion-weighted imaging in evaluating the response to neoadjuvant breast cancer treatment.

The aim of this study was to investigate the role of diffusion imaging in the evaluation of response to neoadjuvant breast cancer treatment by correlating apparent diffusion coefficient (ADC) value changes with pathological response. From June 2007 to June 2009, all consecutive patients with histopathologically confirmed breast cancer undergoing neoadjuvant chemotherapy were enrolled. All patients underwent magnetic resonance imaging (MRI) (including diffusion sequence) before and after neoadjuvant treatment. The ADC values obtained using two different methods of region of interest (ROI) placement before and after treatment were compared with MRI response (assessed using RECIST 1.1 criteria) and pathological response (assessed using Mandard's classification). Fifty-one women (mean age 48.41 years) were included in this study. Morphological MRI (RECIST classification) well evaluated the responder status after chemotherapy (TRG class; area-under-the-curve 0.865). Mean pretreatment ADC values obtained with the two different methods of ROI placement were 1.11 and 1.02 × 10(-3) mm(2) /seconds. Mean post-treatment ADC values were 1.40 and 1.35 × 10(-3) mm(2) /seconds, respectively. A significant inverse correlation between mean ADC increase and Mandard's classifications was observed for both the methods of ADC measurements. Diagnostic performance analysis revealed that the single ROI method has a superior diagnostic accuracy compared with the multiple ROIs method (accuracy: 82% versus 74%). The coupling of the diffusion imaging with the established morphological MRI provides superior evaluation of response to neoadjuvant chemotherapy treatment in breast cancer patients compared with morphological MRI alone. There is a potential in the future to optimize patient therapy on the basis of ADC value changes. Additional works are needed to determine whether these preliminary observed changes in tumor diffusion are a universal response to tumor cell death, and to more fully delineate the ability of ADC value changes in early recognizing responder from nonresponder patients.